Revistas

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Prevalence of hematological abnormalities in patients with Sheehan?s syndrome: response to replacement of glucocorticoids and thyroxine
Abstract
Anemia and other hematological abnormalities are common in patients with Sheehan?s syndrome. The response of these abnormalities to replacement of thyroxine and glucocorticoids is not clear. The aim of the present study was to document the profile of hematological abnormalities and response to treatment in patients with Sheehan?s syndrome. Forty patients of Sheehan?s syndrome and an equal number of age and parity matched healthy controls were studied for prevalence of hematological abnormalities. Hemoglobin concentration, hematocrit, red cell, white cell and platelet count were significantly decreased in patients with Sheehan?s syndrome compared to controls. Frequency of anemia, leucopenia, thrombocytopenia and pancytopenia was significantly higher in these patients compared to controls. After achieving euthyroid and eucortisol state, there was a complete recovery of these hematological abnormalities. We conclude that anemia and other cytopenias are common in patients with Sheehan?s syndrome and replacement with thyroxine and glucocorticoids results in complete recovery of these abnormalities.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0255-2
- Authors
  - Bashir Ahmad Laway, Department of Endocrinology, Sher-I- Kashmir Institute of Medical Sciences Soura, Srinagar, Jammu and Kashmir India
  - Shahnaz Ahmad Mir, Department of Endocrinology, Sher-I- Kashmir Institute of Medical Sciences Soura, Srinagar, Jammu and Kashmir India
  - Mir Iftikhar Bashir, Department of Endocrinology, Sher-I- Kashmir Institute of Medical Sciences Soura, Srinagar, Jammu and Kashmir India
  - Javid Rasool Bhat, Department of Clinical Hematology, Sher-I- Kashmir Institute of Medical Sciences Soura, Srinagar, Jammu and Kashmir India
  - Jeelani Samoon, Department of Clinical Hematology, Sher-I- Kashmir Institute of Medical Sciences Soura, Srinagar, Jammu and Kashmir India
  - Abdul Hamid Zargar, Department of Endocrinology, Sher-I- Kashmir Institute of Medical Sciences Soura, Srinagar, Jammu and Kashmir India
MGMT immunoexpression in aggressive pituitary adenoma and carcinoma
Abstract
Recent case reports have documented the efficacy of temozolomide therapy in some aggressive pituitary adenomas and pituitary carcinomas resistant to multimodality therapy. Evidence suggests that low O6-methylguanine-DNA methyltransferase (MGMT) immunoexpression correlates with response to temozolomide chemotherapy. Herein, we aimed to study MGMT immunoexpression in a spectrum of pituitary tumors, indolent, aggressive and malignant. A literature review of the use of temozolomide in pituitary tumors was also performed. Immunohistochemistry for MGMT was performed on 60 pituitary tumors identified in the Mayo Clinic Tissue Registry and the consultation files of one of us (BWS). The group included 30 pituitary carcinomas (15 ACTH, 10 PRL, 1 FSH/LH, 1 TSH, 1 silent subtype 3 and 2 null cell). Tissue from recurrences was available in 17 cases. In addition, 30 functionally different pituitary adenomas were studied, including 15 invasive and 15 non-invasive adenomas. Overall, 32 cases of pituitary tumors (54%) demonstrated low MGMT immunoexpression. This included 17 of 30 (57%) carcinomas, 9 of 15 (60%) invasive adenomas, and 6 of 15 cases (40%) of non-invasive pituitary adenomas. There was no significant change in MGMT immunoexpression between primary and recurrent tumors. Prolactin-producing carcinomas had the highest proportion of tumors (80%) with low expression. A significant proportion of pituitary adenomas and carcinomas demonstrate low MGMT immunoexpression. In an effort to anticipate the likelihood of a temozolomide response, all cases of aggressive pituitary tumors should be assessed for MGMT expression.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0249-0
- Authors
  - Queenie Lau, Department of Anatomical Pathology and Cytopathology, Royal Brisbane and Women?s Hospital and Gold Coast Hospital, Queensland, Australia
  - Bernd Scheithauer, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA
  - Kalman Kovacs, Department of Laboratory Medicine, St. Michael?s Hospital, Toronto, Canada
  - Eva Horvath, Department of Laboratory Medicine, St. Michael?s Hospital, Toronto, Canada
  - Luis V. Syro, Department of Neurosurgery, Hospital Pablo Tobon Uribe and Clinica Medellin, Medellin, Colombia
  - Ricardo Lloyd, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA
Cardiac function in growth hormone deficient patients before and after 1 year with replacement therapy: a magnetic resonance imaging study
Abstract
Assessed by conventional echocardiography the influence of growth hormone deficiency (GHD) and effects of replacement therapy on left ventricle (LV) function and mass (LVM) have shown inconsistent results. We aimed to evaluate cardiac function before and during replacement therapy employing the gold standard method cardiac magnetic resonance imaging (CMRI) and measurements of circulating levels of B-type natriuretic peptides. Sixteen patients (8 males and 8 females, mean age 49 years (range 18?75)) with severe GHD and 16 matched control subjects were included. CMRI was performed at baseline and after 1 year of GH replacement therapy. IGF-I, B-type natriuretic peptide (BNP) and N-terminal pro-BNP (NT-proBNP) were measured after 0, 1, 2, 3, 6 and 12 months of treatment. IGF-I Z-score increased from (median (IQR)) ?2.3 (?3.8 to ?1.4) to 0.5 (?0.3 to 1.7). LVM index (LVMI), ejection fraction (range 63?80%), cardiac output index and levels of BNP and NT-proBNP were similar at baseline in patients compared to controls (P-values from 0.09 to 0.37). The patients had significantly smaller LV end-diastolic volume index (P = 0.032) and end-systolic volume index (P = 0.038). No significant change in LV systolic function or LVM occurred during 1 year of GH treatment. BNP levels were unchanged (P = 0.88), whereas NT-proBNP tended to decrease (P = 0.052). Assessed by the highly sensitive and precise CMRI method, untreated GHD was not associated with impaired systolic function or reduced LVMI and 1 year of GH replacement using physiological doses did not influence cardiac mass or function.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0250-7
- Authors
  - Mikkel Andreassen, Department of Endocrinology, Endocrine Research Laboratory 54o4, Herlev Hospital, University of Copenhagen, Herlev Ringvej 75, 2730 Herlev, Denmark
  - Jens Faber, Department of Endocrinology, Endocrine Research Laboratory 54o4, Herlev Hospital, University of Copenhagen, Herlev Ringvej 75, 2730 Herlev, Denmark
  - Andreas Kjær, Department of Clinical Physiology, Nuclear Medicine and PET and Cluster for Molecular Imaging, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
  - Claus Leth Petersen, Department of Clinical Physiology and Nuclear Medicine, Frederiksberg Hospital, University of Copenhagen, Copenhagen, Denmark
  - Lars Østergaard Kristensen, Department of Endocrinology, Endocrine Research Laboratory 54o4, Herlev Hospital, University of Copenhagen, Herlev Ringvej 75, 2730 Herlev, Denmark
Anemia in a cohort of men with macroprolactinomas: increase in hemoglobin levels follows prolactin suppression
Abstract
Men with hypogonadism tend to have low hemoglobin (HGB) levels. We have investigated a cohort of 36 consecutive male patients with macroprolactinomas to evaluate HGB during presentation and following treatment with cabergoline to suppress prolactin (PRL). Patients? mean age at diagnosis was 48 years, the mean adenoma size measured 31 mm. The median PRL at baseline was 1,969 ng/ml; the mean testosterone level was low, 1.5 ng/ml. PRL had been successfully normalized in all but six men by using cabergoline. Mean baseline HGB at diagnosis was 13.1 gr%. Sixteen patients had HGB ? 13 gr%, including 4 men with HGB ? 11.5 gr%. In the subgroup of 15 men with very low testosterone (?1 ng/ml), baseline HGB was 12.6 gr% compared with 13.5 gr% in patients with higher testosterone (P < 0.005). In 30 men in whom follow-up CBC data were available, mean baseline HGB increased from 13.2 to 13.9 gr% following PRL suppression by cabergoline. Baseline HGB levels inversely correlated with tumor size, reaching levels of 13.7 gr% in 10 men with macroprolactinomas of 10?20 mm in size, 13.0 gr% in 18 subjects with macroadenomas of 21?40 mm, and 12.4 gr% in 7 patients with giant prolactinomas (>40 mm). In 22 men with normal follow-up testosterone, current HGB levels measured 14.5 gr%, but only 12.8 gr% in 9 men with current low testosterone (P < 0.0005). In men with macroprolactinomas, anemia is common. It is associated with hypogonadism and tumor size, and improves following treatment that normalizes PRL and increases testosterone.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0251-6
- Authors
  - Ilan Shimon, Institute of Endocrinology, Beilinson Hospital, Rabin Medical Center, Petah-Tiqva, 49100 Israel
  - Carlos Benbassat, Institute of Endocrinology, Beilinson Hospital, Rabin Medical Center, Petah-Tiqva, 49100 Israel
  - Gloria Tzvetov, Institute of Endocrinology, Beilinson Hospital, Rabin Medical Center, Petah-Tiqva, 49100 Israel
  - Simona Grozinsky-Glasberg, Institute of Endocrinology, Beilinson Hospital, Rabin Medical Center, Petah-Tiqva, 49100 Israel
The elusive Minnie G.: revisiting Cushing?s case XLV, and his early attempts at improving quality of life
Abstract
Although researchers have discovered that Minnie G. had nearly 50 years of progression-free survival, the absence of her original surgical records have precluded anything more than speculation as to the etiology of her symptoms or the details of her admission. Following IRB approval, and through the courtesy of the Alan Mason Chesney Archives, the microfilm surgical records from the Johns Hopkins Hospital, 1896?1912 were reviewed. Using the surgical number provided in Cushing?s publications, the record for Minnie G. was recovered for further review. Cushing?s diagnosis relied largely on history and physical findings. Minnie G. presented with stigmata associated with classic Cushings Syndrome: abdominal stria, supraclavicular fat pads, and a rounded face. However, she also presented with unusual physical findings: exophthalmos, and irregular pigmentation of the extremities, face, and eyelids. A note in the chart indicates Minnie G. spoke very little English, implying the history-taking was fraught with opportunities for error. Although there remains no definitive etiology for Minnie G.?s symptoms, this report contributes additional information about her diagnosis and treatment.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0248-1
- Authors
  - Courtney Pendleton, Brain Tumor Stem Cell Laboratory, Department of Neurosurgery and Oncology, Johns Hopkins School of Medicine, 1550 Orleans Street, Cancer Research Building II Room 253, Baltimore, MD 21231, USA
  - Hadie Adams, Brain Tumor Stem Cell Laboratory, Department of Neurosurgery and Oncology, Johns Hopkins School of Medicine, 1550 Orleans Street, Cancer Research Building II Room 253, Baltimore, MD 21231, USA
  - Edward R. Laws, Department of Neurosurgery, Brigham and Women?s Hospital, Harvard Medical School, Boston, MA USA
  - Alfredo Quinones-Hinojosa, Brain Tumor Stem Cell Laboratory, Department of Neurosurgery and Oncology, Johns Hopkins School of Medicine, 1550 Orleans Street, Cancer Research Building II Room 253, Baltimore, MD 21231, USA
ACTH-producing remnants following apoplexy of an ACTH-secreting pituitary macroadenoma
Abstract
Describe a case of apoplexy of an ACTH-producing pituitary adenoma which resulted not only in an empty sella with concurrent hypothyroidism, hypoprolactinemia, and hypogonadism but persistent hypercortisolemia from two distinct extrasellar remnants of the original adenoma. Review the literature to identify other similar cases. The patient?s medical history, physical exam, lab data, imaging exams and histopathological results were analyzed and compiled into a case report, and an extensive review of the literature was performed. Endocrinological data revealed hypercortisolism and an elevated ACTH with an otherwise suppressed pituitary axis. A pituitary MRI showed a macroadenoma in the left cavernous sinus in addition to an empty sella. An octreotide scan revealed lesions in the left sella turcica and the right sphenoid sinus. Tissue samples of both lesions stained positive for ACTH and negative for GH, prolactin, FSH, LH, and TSH. The lesions were surgically removed, and the patient treated with radiation and ketoconazole. This resulted in a significant decrease in ACTH and cortisol as well as a marked improvement in blood glucose control. The review of literature revealed the absence of any similar cases in the past. The patient presented with apoplexy of an ACTH-secreting pituitary macroadenoma with two hormonally active extrasellar remnants. Several cases in the literature describe recurrence of Cushing?s disease following infarction of ACTH-secreting adenomas. This is the first documented case of infarction of an ACTH-producing adenoma resulting in two distinct ACTH-producing remnants without recurrence of the original adenoma.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0247-2
- Authors
  - Caroline Korsten Messer, Department of Endocrinology, Mount Kisco Medical Group, 90 South Bedford Road, Mount Kisco, NY 10549, USA
  - Mary E. Fowkes, Department of Pathology, Mount Sinai School of Medicine, 1190 Fifth Avenue, New York, NY 10128, USA
  - J. Lester Gabrilove, Department of Endocrinology, Mount Sinai School of Medicine, 1 Gustave L. Levy Place, Box 1055, New York, NY 10029, USA
  - Kalmon D. Post, Department of Neurosurgery, Mount Sinai School of Medicine, 1 Gustave L. Levy Place, Annenberg 806, New York, NY 10029, USA
  - Honju Son, Department of Nuclear Medicine, Mount Sinai School of Medicine, 1 Gustave L Levy Place, Box 1141, New York, NY 10029, USA
  - Alice C. Levine, Department of Endocrinology, Mount Sinai School of Medicine, 1 Gustave L. Levy Place, Box 1055, New York, NY 10029, USA
Quantification of day-to-day variability in growth hormone levels in acromegaly
Abstract
Growth hormone (GH) measurements are routinely used for important treatment decisions in patients with acromegaly, yet their reliability is affected by numerous factors including assay precision and variability, sampling intensity, and hormone pulsatility. The day-to-day variation in GH in acromegaly has not been studied. This study quantified the magnitude of day-to-day GH variability in patients with acromegaly by performing an analysis of previously obtained plasma GH profiles. The analysis was performed at the Michigan Clinical Research Unit at the University of Michigan. A total of nine 48 h Q10 min GH profiles obtained in nine patients with active acromegaly were examined. The study was planned after data collection and analysis was conducted using Altman?Bland methods. Day 1 vs. Day 2 values were examined. 95% confidence intervals of the D2 vs. D1 ratios were calculated on all individual subject data as well as on a single 0800 h GH sample and composite mean data for 2-, 5-, 9-, and 24-h sampling protocols. Confidence interval range was 0.66?1.50 for the 0800 h sample and was similar for all sampling protocols except somewhat more narrow for the 24-h sampling (0.75?1.32). Daily variations in GH levels introduce an additional confounding element when using a single GH level or even daily GH curves to assess a patient?s GH milieu. It may have an impact on result interpretation and subsequent treatment decisions especially when GH results are considered borderline.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0245-4
- Authors
  - Andrew Kraftson, Division of Metabolism, Endocrinology and Diabetes, University of Michigan Medical Center, 24 Frank Lloyd Wright Drive, G-1500, Ann Arbor, MI 48109, USA
  - Ariel Barkan, Division of Metabolism, Endocrinology and Diabetes, Department of Neurosurgery, University of Michigan Medical Center, 24 Frank Lloyd Wright Drive, G-1500, Ann Arbor, MI 48109, USA
Cardiac effects of 3 months treatment of acromegaly evaluated by magnetic resonance imaging and B-type natriuretic peptides
Abstract
Long-term treatment of acromegaly prevents aggravation and reverses associated heart disease. A previous study has shown a temporary increase in serum levels of the N-terminal fraction of pro B-type natriuretic peptide (NT-proBNP) suggesting an initial decline in cardiac function when treatment of acromegaly is initiated. This was a three months prospective study investigating short-term cardiac effects of treatment in acromegalic patients. Cardiac function was evaluated by the gold standard method cardiac magnetic resonance imaging (CMRI) and circulating levels of B-type natriuretic peptides (BNP and NT-proBNP). CMRI was performed at baseline and after 3 months of treatment. Levels of IGF-I, BNP and NT-proBNP were measured after 0, 1, 2 and 3 months. Eight patients (5 males and 3 females, mean age 53 ± 12 years (range 30?70)) and 8 matched healthy control subjects were included. Median IGF-I Z-score decreased from 4.5 (range 2.5?6.4) to 2.3 (?0.1 to 3.3). At baseline the patients had increased left ventricle mass index (LVMI) compared to control subjects (?LVMI 35 g/m² (95% CI 8?63 g/m², P = 0.016). After 3 months of treatment there was an increase in end-diastolic volume index EDVI (?EDVI 9 mL/m² (95% CI 3?14), P = 0.007) and an increase in levels of BNP (median (ranges) 7 (0.58?286) vs. 20 (1?489) pg/mL, P = 0.033) and of NT-proBNP (63 (20?1004) vs. 80 (20?3391) pg/mL, P = 0.027). Assessed by the highly sensitive and precise CMRI method, 3 months treatment of acromegaly resulted in an increase in EDVI, and increased levels of BNP and NT-proBNP suggesting an initial decrease in cardiac function.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0240-9
- Authors
  - Mikkel Andreassen, Endocrine Research Laboratory 54o4, Department of Endocrinology, Herlev Hospital, University of Copenhagen, Herlev Ringvej 75, 2730 Herlev, Denmark
  - Jens Faber, Endocrine Research Laboratory 54o4, Department of Endocrinology, Herlev Hospital, University of Copenhagen, Herlev Ringvej 75, 2730 Herlev, Denmark
  - Andreas Kjær, Department of Clinical Physiology, Nuclear Medicine and PET, University of Copenhagen, Copenhagen, Denmark
  - Claus Leth Petersen, Department of Clinical Physiology and Nuclear Medicine, Frederiksberg Hospital, University of Copenhagen, Copenhagen, Denmark
  - Lars Østergaard Kristensen, Endocrine Research Laboratory 54o4, Department of Endocrinology, Herlev Hospital, University of Copenhagen, Herlev Ringvej 75, 2730 Herlev, Denmark
Bone age and factors affecting skeletal maturation at diagnosis of paediatric Cushing?s disease
Abstract
Paediatric Cushing?s disease (CD) is usually associated with growth retardation, but there are only few published data on skeletal maturation at diagnosis. We analysed factors contributing to skeletal maturation and final height in Asian Indian patients with paediatric CD. We conducted retrospective analysis of 48 patients (29 males; 19 females) with mean age: 14.84 years at diagnosis (range 9?19 years). A single observer using the Greulich Pyle method determined the bone age (BA) of each child. BA delay, i.e. the difference between chronological age (CA) and BA, was compared with clinical and biochemical variables. BA delay was present in 35/48 (73%) patients (mean delay 1.6 years, range 0.5?5 years) and correlated negatively with height SDS (r = ?0.594, P < 0.001) and positively with CA at diagnosis (r = 0.247, P < 0.05). There was no correlation with duration of symptoms before diagnosis, basal cortisol, midnight cortisol, ACTH or percentage suppression of low dose dexamethasone suppression cortisol (LDDST). We could not demonstrate any relationship between the duration of history before diagnosis and height SDS at final height. Mean final height SDS in patients was ?1.84. We found that most children with CD had delayed BA and correlated significantly with CA and height SDS at diagnosis. Early diagnosis may reduce delay in skeletal maturation and thus contribute to optimal catch-up growth.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0246-3
- Authors
  - Shrikrishna V. Acharya, Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, 400 012 Maharashtra India
  - Raju A. Gopal, Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, 400 012 Maharashtra India
  - Anurag Lila, Seth G S Medical College and KEM Hospital, Mumbai, 400 012 Maharashtra India
  - Padma S. Menon, Seth G S Medical College and KEM Hospital, Mumbai, 400 012 Maharashtra India
  - Tushar R. Bandgar, Seth G S Medical College and KEM Hospital, Mumbai, 400 012 Maharashtra India
  - Nalini S. Shah, Seth G S Medical College and KEM Hospital, Mumbai, 400 012 Maharashtra India
Effects of cabergoline on pregnancy and embryo-fetal development: retrospective study on 103 pregnancies and a review of the literature
Abstract
The aim of the study is to assess the rate of any potential adverse effects on women who became pregnant under cabergoline (CAB) treatment and to evaluate any effects on the embryo-fetal development and on children who were born from mothers exposed to CAB in early weeks of gestation. Observational, retrospective and multicenter study on 103 pregnancies in 90 women with hyperprolactinemia. All patients were under CAB at conception. Serum prolactin at baseline was between 30 and 1921 ng/ml. Duration of therapy before pregnancy ranged from 1 to 120 months and doses ranged from 0.125 to 5 mg/week. Fetal exposure ranged from 3 to 25 weeks, 96.9% of patients received CAB during the first trimester of pregnancy and the rest until the second one. No significant complications during pregnancy were found. Seven women (7.2%) had spontaneous abortions. Preterm deliveries were recorded in eight (8.8%), only one with low weight for gestational age. Neonatal abnormalities were observed in 3 (3.6%): 1 major (Down syndrome) and 2 minor malformations (umbilical and inguinal hernia). We were able to asses the children?s development in 61. Two had epilepsy and two had Pervasive Developmental Disorder (PDD). No significantly higher frequency of complications was found in pregnancies and/or offspring exposed to CAB than in the normal population. We registered 2 abnormalities in the development of the children: epilepsy and PDD. Larger series of patients are needed to assess the safety of this drug during pregnancy.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0243-6
- Authors
  - Graciela Stalldecker, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - María Susana Mallea-Gil, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Mirtha Guitelman, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Analía Alfieri, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - María Carolina Ballarino, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Laura Boero, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Alberto Chervin, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Karina Danilowicz, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Sabrina Diez, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Patricia Fainstein-Day, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Natalia García-Basavilbaso, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Mariela Glerean, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Viviana Gollan, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Débora Katz, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Mónica Graciela Loto, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Marcos Manavela, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Amelia Susana Rogozinski, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Marisa Servidio, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
  - Nicolás Marcelo Vitale, Sociedad Argentina de Endocrinología y Metabolismo Departamento de Neuroendocrinología (Neuroendocrinology Department) Diaz Velez 3889 1200 Buenos Aires Argentina
Lymphocytic hypophysitis in a patient presenting with sequential episodes of optic neuritis
Abstract
A 41-year-old man presented with left optic neuritis (ON) without evidence of other autoimmune disease or hormonal imbalance. MRI showed enlargement of the left optic nerve but no sellar lesion. The patient recovered after steroid therapy but later developed right ON and required treatment again. Follow-up MRI revealed an ill-defined, enlarging sellar lesion with enhancement extending into the right cavernous sinus, and the patient developed symptoms of fatigue and loss of libido. Hormonal studies revealed hypogonadism and hypocortisolism. All laboratory investigation for autoimmune and infectious diseases remained negative. A transsphenoidal biopsy of the lesion revealed lymphocytic hypophysitis. The concomitant development of lymphocytic hypophysitis and optic neuritis suggests a common and likely autoimmune etiology. Visual loss in patients with LYH can sometimes be due to ON rather than compression of the optic apparatus, with significant implications for treatment strategies.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0244-5
- Authors
  - Garrett K. Zoeller, University of Miami Miller School of Medicine Department of Neurological Surgery Lois Pope LIFE Center, 1095 NW 14th Terrace (D4-6) Miami FL 33136 USA
  - Ronald J. Benveniste, University of Miami Miller School of Medicine Department of Neurological Surgery Lois Pope LIFE Center, 1095 NW 14th Terrace (D4-6) Miami FL 33136 USA
  - F. A. Farhadi, University of Miami Miller School of Medicine Department of Pathology Miami FL USA
  - Jocelyn H. Bruce, University of Miami Miller School of Medicine Department of Pathology Miami FL USA
Acquired resistance to cabergoline: progression from initially responsive micro to macroprolactinoma
Acquired resistance to cabergoline: progression from initially responsive micro to macroprolactinoma
- Content Type Journal Article
- DOI 10.1007/s11102-010-0237-4
- Authors
  - M. Alberiche Ruano, Hospital Universitario Insular de Gran Canaria Endocrinology Deparment Las Palmas de Gran Canaria 35016 Canary Island Spain
  - M. Boronat Cortés, Hospital Universitario Insular de Gran Canaria Endocrinology Deparment Las Palmas de Gran Canaria 35016 Canary Island Spain
  - A. Ojeda Pino, Hospital Universitario Insular de Gran Canaria Endocrinology Deparment Las Palmas de Gran Canaria 35016 Canary Island Spain
  - C. Rodriguez Perez, Hospital Universitario Insular de Gran Canaria Endocrinology Deparment Las Palmas de Gran Canaria 35016 Canary Island Spain
  - M. Gracía Nuñez, Hospital Universitario Insular de Gran Canaria Endocrinology Deparment Las Palmas de Gran Canaria 35016 Canary Island Spain
  - D. Marrero Arencibia, Hospital Universitario Insular de Gran Canaria Endocrinology Deparment Las Palmas de Gran Canaria 35016 Canary Island Spain
  - F. J. Novoa Mogollón, Hospital Universitario Insular de Gran Canaria Endocrinology Deparment Las Palmas de Gran Canaria 35016 Canary Island Spain
The clinical significance of MIB-1 labeling index in pituitary adenomas
Abstract
Pituitary adenomas are unique in several ways?they are rarely malignant and yet can be invasive of several compartments. Recurrences in tumors with bland histological features that have been radically excised are a reason for frustration faced by endocrinologists and neurosurgeons in treatment of pituitary adenomas. Several attempts have therefore been made to determine the growth potential of pituitary adenomas. The aim of the present study was to define the biological significance of the MIB-1 labelling index (MIB-1 LI) in pituitary adenomas. The study included 159 cases of surgically treated pituitary adenoma seen in a single institution. MIB-1 LI was not found to be related to age or gender. The mean MIB-1 LI for clinically functional adenomas was marginally higher than that for clinically non-functional adenomas. There was a significant difference in the MIB-1 LI for tumors with a maximum diameter of more than 4 cm at a MIB-1 LI of ?2%, however this difference was not statistically significant at a higher MIB-1 LI cut off value of >3%. The mean MIB-1 LI was significantly higher in tumors causing hydrocephalus and in those with cavernous sinus invasion and not when invasion was defined as invasion by tumor in any direction. We conclude that large pituitary macroadenomas, tumors filling the third ventricle causing hydrocephalus and tumors with true cavernous sinus invasions are more likely to have a higher proliferation index. Close follow up of tumors showing these imaging features would be recommended.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0242-7
- Authors
  - Geeta Chacko, Christian Medical College Section of Neuropathology, Department of Neurological Sciences & Pathology Vellore 632004 India
  - Ari G. Chacko, Christian Medical College Division of Neurosurgery, Department of Neurological Sciences Vellore India
  - Kalman Kovacs, St. Michaels Hospital Department of Laboratory Medicine Toronto ON Canada
  - Bernd W. Scheithauer, Mayo Clinic Department of Laboratory Medicine Rochester MN USA
  - Sunithi Mani, Christian Medical College Department of Radiology Vellore India
  - J. P. Muliyil, Christian Medical College Department of Community Health Vellore India
  - M. S. Seshadri, Christian Medical College Department of Endocrinology Vellore India
Cost of evaluation of patients with pituitary incidentaloma
Cost of evaluation of patients with pituitary incidentaloma
- Content Type Journal Article
- DOI 10.1007/s11102-010-0241-8
- Authors
  - Benjamin R. Randall, University of Utah Department of Neurosurgery Salt Lake City UT USA
  - Kristin L. Kraus, University of Utah Department of Neurosurgery Salt Lake City UT USA
  - Marie F. Simard, University of Utah Department of Pediatrics, Division of Endocrinology Salt Lake City UT USA
  - William T. Couldwell, University of Utah Department of Neurosurgery Salt Lake City UT USA
The autopsy was conducted ?Under most inauspicious circumstances:? John Turner, Harvey Cushing?s case XXXII, and his unwitting contributions to the early understanding of acromegaly
Abstract
Harvey Cushing?s monograph The Pituitary Body and Its Disorders describes Case XXXII, a 36-year-old man who presented with gigantism in 1910. The detailed post-mortem exam findings are prefaced with a cryptic statement, describing ?inauspicious circumstances? surrounding the autopsy. Although contemporary biographies of Cushing have offered insight into these circumstances, the original surgical file for Case XXXII has not been previously reviewed. The original Johns Hopkins Hospital surgical records were reviewed, and the case of John Turner, who Cushing identified by name in his monograph The Pituitary Body and Its Disorders, was selected for further review. A review of the original surgical file revealed a typewritten note by Dr. Crowe, one of the surgeons who performed the post-mortem exam, with a handwritten addendum by Dr. Cushing. This document provides detail regarding the ?inauspicious circumstances? surrounding the autopsy. Namely, the autopsy was conducted without permission of the family, during the funeral service, following a payment to the undertaker. The new information regarding the autopsy of John Turner offers insight into the previously incompletely described circumstances surrounding the autopsy. Additionally, the case illuminates the obligations and ethical quandaries that physician?scientists face.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0239-2
- Authors
  - Courtney Pendleton, Johns Hopkins School of Medicine Department of Neurosurgery, Oncology and Medicine Baltimore MD USA
  - Gary Wand, Johns Hopkins School of Medicine Department of Neurosurgery, Oncology and Medicine Baltimore MD USA
  - Alfredo Quinones-Hinojosa, Johns Hopkins School of Medicine Department of Neurosurgery, Oncology and Medicine Baltimore MD USA
Effects of ghrelin, GH-releasing peptide-6 (GHRP-6) and GHRH on GH, ACTH and cortisol release in hyperthyroidism before and after treatment
Abstract
In thyrotoxicosis GH responses to stimuli are diminished and the hypothalamic?pituitary?adrenal axis is hyperactive. There are no data on ghrelin or GHRP-6-induced GH, ACTH and cortisol release in treated hyperthyroidism. We, therefore, evaluated these responses in 10 thyrotoxic patients before treatment and in 7 of them after treatment. GHRH-induced GH release was also studied. Peak GH (?g/L; mean ± SE) values after ghrelin (22.6 ± 3.9), GHRP-6 (13.8 ± 2.3) and GHRH (4.9 ± 0.9) were lower in hyperthyroidism before treatment compared to controls (ghrelin: 67.6 ± 19.3; GHRP-6: 25.4 ± 2.7; GHRH: 12.2 ± 2.8) and did not change after 6 months of euthyroidism (ghrelin: 32.7 ± 4.7; GHRP-6: 15.6 ± 3.6; GHRH: 7.4 ± 2.3), although GH responses to all peptides increased in ~50% of the patients. In thyrotoxicosis before treatment ACTH response to ghrelin was two fold higher (107.4 ± 26.3) than those of controls (54.9 ± 10.3), although not significantly. ACTH response to GHRP-6 was similar in both groups (hyperthyroid: 44.7 ± 9.0; controls: 31.3 ± 7.9). There was a trend to a decreased ACTH response to ghrelin after 3 months of euthyroidism (35.6 ± 5.3; P = 0.052), but after 6 months this decrease was non-significant (50.7 ± 14.0). After 3 months ACTH response to GHRP-6 decreased significantly (20.4 ± 4.2), with no further changes. In hyperthyroidism before treatment, peak cortisol (?g/dL) responses to ghrelin (18.2 ± 1.2) and GHRP-6 (15.9 ± 1.4) were comparable to controls (ghrelin: 16.4 ± 1.6; GHRP-6: 13.5 ± 0.9) and no changes were seen after treatment. Our results suggest that the pathways of GH release after ghrelin/GHRP-6 and GHRH are similarly affected by thyroid hormone excess and hypothalamic mechanisms of ACTH release modulated by ghrelin/GHSs may be activated in this situation.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0238-3
- Authors
  - Patricia Molica, Universidade Federal de São Paulo, UNIFESP-EPM Division of Endocrinology Rua Pedro de Toledo, 910 04039-002 São Paulo SP Brazil
  - Sergio Oliva Nascif, Universidade Federal de São Paulo, UNIFESP-EPM Division of Endocrinology Rua Pedro de Toledo, 910 04039-002 São Paulo SP Brazil
  - Silvia Regina Correa-Silva, Universidade Federal de São Paulo, UNIFESP-EPM Division of Endocrinology Rua Pedro de Toledo, 910 04039-002 São Paulo SP Brazil
  - Larissa Bianca Paiva Cunha de Sá, Universidade Federal de São Paulo, UNIFESP-EPM Division of Endocrinology Rua Pedro de Toledo, 910 04039-002 São Paulo SP Brazil
  - José Gilberto Henriques Vieira, Universidade Federal de São Paulo, UNIFESP-EPM Division of Endocrinology Rua Pedro de Toledo, 910 04039-002 São Paulo SP Brazil
  - Ana-Maria Judith Lengyel, Universidade Federal de São Paulo, UNIFESP-EPM Division of Endocrinology Rua Pedro de Toledo, 910 04039-002 São Paulo SP Brazil
Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adults
Abstract
Langerhans cell histiocytosis (LCH) is a rare granulomatous disease of unknown etiology. We retrospectively reviewed data from four patients (3 males and 1 female), mean age 33.5 years old (range: 21?40), with histopathological diagnosis of LCH. All of them presented with symptoms suggestive of endocrine involvement. The main complaint was goiter in two patients and polyuria and polydipsia in three. Before the LCH diagnosis, two patients had unevaluated symptoms of diabetes insipidus (DI) and hypogonadism. The mean time from symptoms onset to diagnosis was 6.25 years (range: 2?13). Histopathological diagnosis was established by total thyroidectomy (TT) biopsy in two patients, skin lesion biopsy in one, and pituitary stalk biopsy in the other. In the two-first patients, surgery was indicated after the fine-needle aspiration biopsy (FNAB) showed a false positive result of differentiated thyroid carcinoma and immunohistochemistry was used for diagnosis confirmation. Three cases were treated with chemotherapy; one of them had already received radiation therapy on the hypothalamic-pituitary region, developing post-radiation hypopituitarism.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0233-8
- Authors
  - M. S. García Gallo, Hospital Carlos G. Durand Endocrinology Division Av. Díaz Vélez 5040 Buenos Aires C1405DCR Argentina
  - M. P. Martínez, Hospital Carlos G. Durand Endocrinology Division Av. Díaz Vélez 5040 Buenos Aires C1405DCR Argentina
  - M. S. Abalovich, Hospital Carlos G. Durand Endocrinology Division Av. Díaz Vélez 5040 Buenos Aires C1405DCR Argentina
  - S. Gutiérrez, Hospital Carlos G. Durand Endocrinology Division Av. Díaz Vélez 5040 Buenos Aires C1405DCR Argentina
  - M. A. Guitelman, Hospital Carlos G. Durand Endocrinology Division Av. Díaz Vélez 5040 Buenos Aires C1405DCR Argentina
Oral estroprogestin: an alternative low cost therapy for women with postoperative persistent acromegaly?
Abstract
Oral estrogens reduce GH-induced IGF-1 production and preliminary studies have shown that adjuvant estroprogestin (EP) therapy with octreotide LAR may control disease activity in some female patients who are partially responsive to octreotide LAR. Our aim was to verify if EP alone or in combination with octreotide LAR can achieve remission of acromegaly in selected cases of patients uncontrolled by surgery. Eleven women with persistent active acromegaly following surgery participated in this unblinded open label pilot study. Their mean age was 49.8 ± 4.3 years. Two patients were drug naïve, two patients had stopped octreotide LAR because of intolerance and seven were treated with octreotide LAR. The patients received either EP (EP pill, 20 ?g ethinylestradiol, 100 ?g levonorgestrel) alone (4 patients) or added to octreotide LAR (7 patients). Fasting GH, IGF-1, glucose, HDL- and LDL-cholesterol, and triglycerides were measured at baseline and at last visit. MRI was controlled at baseline and at last visit. Duration of estrogen treatment was 3.1 ± 0.5 years. Serum IGF-1 levels were normalized in 8/11 patients (73%). Serum GH concentrations did not change significantly during treatment (11.6 ± 5.6 ?g/L prior to EP vs 5.5 ± 1.2 ?g/L following EP). In patients treated with EP alone, remission was achieved in 2/4 patients (IGF-1 percentages of the upper limit of normal age-matched range (%ULN): 211 ± 40% before EP compared to 95 ± 15% after EP, P = 0.028). In the seven patients treated by EP added to octreotide LAR, remission was achieved in 6 patients (IGF-1%ULN: 158 ± 9% before EP compared to 86 ± 4% after EP, P = 0.0003). Glucose and cholesterol levels were unchanged by EP treatment (data not shown). MRI did not show any evidence of tumour progression with EP in patients who had a tumour remnant. In conclusion, oral estrogen treatment appears to normalize serum IGF-1 concentrations in over 70% of women with acromegaly uncured by surgery irrespective of their sensitivity to octreotide LAR. We suggest that estrogens may be a temporary cost-effective and safe treatment for women with postoperative persistent acromegaly.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0236-5
- Authors
  - Sophie Vallette, Université de Montréal Department of Endocrinology, Centre Hospitalier de l?Université de Montréal, Notre-Dame Hospital 1560 Sherbrooke East Montreal QC Canada
  - Omar Serri, Université de Montréal Department of Endocrinology, Centre Hospitalier de l?Université de Montréal, Notre-Dame Hospital 1560 Sherbrooke East Montreal QC Canada
Hypopituitarism following brain injury: when does it occur and how best to test?
Abstract
Aim of this review is to highlight how and when Traumatic Brain Injury (TBI) as well as Subarachnoid Haemorrhage (SAH) and primary Brain Tumours (pBT) of the Central Nervous System (CNS) can induce hypopituitarism, an under-diagnosed clinical problem. Moreover, this review aims to clarify, on the basis of the recent evidences, how these patients have to be tested for pituitary-function. Both retrospective and prospective studies recommended that patients with more severe form of Brain Injuries (BI) and in particular, those with fractures of the base of the skull or early diabetes insipidus, have to be closely monitored for signs and symptoms of endocrine dysfunction. Further studies will be crucial to raise awareness and remind physicians on the prevalence of hypopituitarism in patients with BI and to elucidate any incremental benefits these patients may receive from hormone replacement.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0235-6
- Authors
  - Valentina Gasco, University of Turin Division of Endocrinology and Metabolism, Department of Internal Medicine c.so Dogliotti 14 10126 Turin Italy
  - Flavia Prodam, University of Eastern Piedmont ?Amedeo Avogadro? Division of Endocrinology, Department of Clinical and Experimental Medicine Novara Italy
  - Loredana Pagano, University of Eastern Piedmont ?Amedeo Avogadro? Division of Endocrinology, Department of Clinical and Experimental Medicine Novara Italy
  - Silvia Grottoli, University of Turin Division of Endocrinology and Metabolism, Department of Internal Medicine c.so Dogliotti 14 10126 Turin Italy
  - Sara Belcastro, University of Eastern Piedmont ?Amedeo Avogadro? Division of Endocrinology, Department of Clinical and Experimental Medicine Novara Italy
  - Paolo Marzullo, University of Eastern Piedmont ?Amedeo Avogadro? Division of Endocrinology, Department of Clinical and Experimental Medicine Novara Italy
  - Guglielmo Beccuti, University of Turin Division of Endocrinology and Metabolism, Department of Internal Medicine c.so Dogliotti 14 10126 Turin Italy
  - Ezio Ghigo, University of Turin Division of Endocrinology and Metabolism, Department of Internal Medicine c.so Dogliotti 14 10126 Turin Italy
  - Gianluca Aimaretti, University of Eastern Piedmont ?Amedeo Avogadro? Division of Endocrinology, Department of Clinical and Experimental Medicine Novara Italy
Hypothalamic pituitary abnormalities in tubercular meningitis at the time of diagnosis
Abstract
Tubercular meningitis (TBM) is the most dreaded form of extra pulmonary tuberculosis associated with high morbidity and mortality. Various hypothalamic pituitary hormonal abnormalities have been reported to occur years after recovery from disease but there are no systematic studies in the literature to evaluate the pituitary hypothalamic dysfunction in patients with TBM at the time of presentation. Therefore, the present study was designed to evaluate hypothalamic pituitary abnormalities in newly diagnosed patients with TBM. Patient case series. This prospective study included 75 untreated adult patients with TBM diagnosed as ?definite?, ?highly probable? and ?probable? TBM by Ahuja?s criteria and in clinical stage 1, 2 or 3 at the time of presentation to hospital. Basal hormonal profile was measured by electrochemilumniscence technique for serum cortisol, luetinizing hormone (LH), follicular stimulating hormone (FSH), prolactin (PRL), thyrotropin (TSH), free tri-iodothyronine (fT3), and free thyroxine (fT4). All patients were subjected to MRI to image brain and hypothalamic pituitary axis and CT for adrenal glands. Thirty-two (42.7%) cases showed relative or absolute cortisol insufficiency. Twenty-three (30.7%) cases showed central hypothyroidism and 37 (49.3%) cases had hyperprolactinemia. No patient had evidence of diabetes insipidus. Multiple hormone deficiency was seen in 22 (29.3%) cases. MRI of hypothalamic pituitary axis using dynamic scanning and thin cuts revealed abnormalities in 10 (13.3%) of the cases. CT adrenal gland was normal in all the patients. Tubercular meningitis is associated with both hormonal and structural abnormalities in the hypothalamic pituitary axis at the time of diagnosis.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0234-7
- Authors
  - Dinesh Kumar Dhanwal, Maulana Azad Medical College and Associated Hospitals Department of Medicine New Delhi 110002 India
  - Anirudh Vyas, Maulana Azad Medical College and Associated Hospitals Department of Medicine New Delhi 110002 India
  - Ashok Sharma, Maulana Azad Medical College and Associated Hospitals Department of Radiology, GB Pant Hospital New Delhi 110002 India
  - Alpana Saxena, Maulana Azad Medical College and Associated Hospitals Deaprtment of Biochemistry New Delhi 110002 India
Radiotherapy in paediatric Cushing?s disease: efficacy and long term follow up of pituitary function
Abstract
Pituitary radiotherapy (RT) is an effective second-line treatment for paediatric Cushing?s disease (CD). We report long-term efficacy and anterior pituitary function in a cohort of paediatric CD patients treated with RT. Between 1988 and 2008, from our cohort of 48 paediatric CD patients, eight paediatric CD patients (5 males and 3 females) underwent second-line pituitary RT (45 Gy in 25 fractions), following unsuccessful transsphenoidal surgery. Out of eight whose long term follow up is available, four patients were cured by RT. Minimum follow up of 2 years is available for all patients. Four patients achieved cure after RT. Two patients were not cured even after follow up of 60 and 132 months, respectively. Out of four uncured patients, two of them had suppressible low dose dexamethasone cortisol with altered circadian rhythm suggesting possibility of response in near future with follow up of just 26 months. Five patients were hypogonadal and one patient was hypothyroid. All patients were below their target height at the time of last follow up. None of the patients had posterior pituitary dysfunction. This series of patients illustrates the efficacy and long-term follow up of pituitary function in children with CD treated with RT. This study also emphasizes the need of growth hormone statues assessment and timely intervention.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0231-x
- Authors
  - Shrikrishna V. Acharya, Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, 12 Maharashtra India
  - Raju A. Gopal, Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, 12 Maharashtra India
  - Joe Goerge, Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, 12 Maharashtra India
  - Padma S. Menon, Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, 12 Maharashtra India
  - Tushar R. Bandgar, Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, 12 Maharashtra India
  - Nalini S. Shah, Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, 12 Maharashtra India
Failure of temozolomide and conventional doses of pegvisomant to attain biochemical control in a severe case of acromegaly
Abstract
It has been suggested that treatment with adequate dose titration of pegvisomant, a GH antagonist, up to a maximum of 40 mg daily, can achieve IGF-1 normalisation in virtually all patients with acromegaly. On the other hand, temozolomide (TMZ), an alkylating cytostatic agent, has been reported to reduce pituitary tumour size and hormone hypersecretion in a small number of aggressive pituitary macroadenomas. In this paper we report the case of a patient resistant to very high doses of pegvisomant used in combination with somatostatin analogs (SSA) and to TMZ therapy. The patient, initially a 22 year-old man with an invasive GH-secreting pituitary macroadenoma (IGF-1, 371% upper limit of normal), had active acromegaly despite a repeat transsphenoidal surgery followed by radiotherapy and SSA (octreotide 800 ?g sc daily) (IGF-1, 262% ULN). In combination with SSA, pegvisomant was started at 20 mg daily and doses were titrated up to 60 mg daily. IGF-1 was moderately reduced and stabilized at 200% ULN after 1 year of treatment. Serum pegvisomant level was 30,500 ng/l, the denaturalized GHBP concentration 1,120 pM and the endogenous GH level was 220 ?g/l. Pegvisomant was stopped and TMZ therapy was given for 5 cycles. However, the patient reported an increase of acromegaly symptoms and the serum IGF-1 was raised to the same level prior to pegvisomant therapy. Consequently, pegvisomant was tried again with doses up to 100 mg daily finally resulting in normalisation of serum IGF-1 level and improvement of acromegaly symptoms and patient well-being. We conclude that in some patients with severe acromegaly refractory to multimodal therapy, biochemical control may be difficult to attain with conventional doses of pegvisomant or TMZ therapy.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0232-9
- Authors
  - Emilie Morin, Notre-Dame Hospital Department of Endocrinolgy, CHUM Research Centre 1560, rue Sherbrooke East Montreal QC H2L 4M1 Canada
  - France Berthelet, Notre-Dame Hospital Department of Pathology, CHUM Research Centre Montreal QC Canada
  - John Weisnagel, CHUL Service of Endocrinology Quebec City QC Canada
  - Martin Bidlingmaier, University of Munich Endocrine Laboratories Munich Germany
  - Omar Serri, Notre-Dame Hospital Department of Endocrinolgy, CHUM Research Centre 1560, rue Sherbrooke East Montreal QC H2L 4M1 Canada
Polyuria and polydipsia in a young child: diagnostic considerations and identification of novel mutation causing familial neurohypophyseal diabetes insipidus
Abstract
A 3-year 5-month-old boy was seen for second opinion regarding polydipsia and polyuria. Previously, a diagnosis of primary polydipsia was made after normal urine concentration after overnight water deprivation testing. The boy?s father, paternal grandfather, and paternal aunt had diabetes insipidus treated with desmopressin acetate. Based on this young boy?s symptoms, ability to concentrate urine after informal overnight water deprivation, and family history of diabetes insipidus, we performed AVP gene mutation testing. Analysis of the AVP gene revealed a novel mutation G54E that changes a normal glycine to glutamic acid, caused by a guanine to adenine change at nucleotide g.1537 (exon 2) of the AVP gene. Commonly, patients with familial neurohypophyseal diabetes insipidus (FNHDI) present within the first 6 years of life with progressively worsening polyuria and compensatory polydipsia. Since these patients have progressive loss of arginine vasopressin (AVP), they may initially respond normally to water deprivation testing and have normal pituitary findings on brain MRI. Genetic testing may be helpful in these patients, as well as preemptively diagnosing those with a mutation, thereby avoiding unnecessary surveillance of those unaffected.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0230-y
- Authors
  - Matthew D. Stephen, The University of Texas Health Science Center, Houston Department of Pediatrics 6431 Fannin, MSB 3.122 Houston TX 77030 USA
  - Raymond G. Fenwick, Quest Diagnostics Nichols Institute Molecular Endocrinology San Juan Capistrano CA USA
  - Patrick G. Brosnan, The University of Texas Health Science Center, Houston Department of Pediatrics 6431 Fannin, MSB 3.122 Houston TX 77030 USA
Ectopic growth hormone-releasing hormone secretion by a bronchial carcinoid tumor: clinical experience following tumor resection and long-acting octreotide therapy
Abstract
Acromegaly resulting from the ectopic secretion of growth hormone-releasing hormone (GHRH) is rare. We present a case of acromegaly secondary to proven GHRH-secretion by a bronchial carcinoid tumor in a type 1 diabetic subject and document the clinical course pre- and post-resection of the tumor and of subsequent octreotide therapy. A 54-year-old Caucasian man was referred for evaluation of acromegalic symptoms and significantly increased insulin requirements. He had a history of left lung surgery 20 years prior for hemoptysis. Initial laboratory results indicated acromegaly. Fasting serum growth hormone (GH): 26.1 ng/mL (0?5 ng/mL), insulin-like growth factor 1 (IGF-1): 635 ng/mL (87?283 ng/mL), GH at 60 min post-ingestion of 75 grams of oral glucose during a glucose tolerance test: 8.3 ng/mL (normal <1 ng/mL). Pituitary magnetic resonance imaging (MRI) revealed diffuse pituitary enlargement without adenoma. A 4.4 cm left hilar mass was noted on chest computed tomography (CT) scan. Further evaluation for a suspected GHRH-secreting neuroendocrine tumor was pursued. Plasma GHRH level was elevated: 198 pg/mL (<50 pg/mL). Octreoscan showed radiolabelled-octreotide uptake in the left lung mass and pituitary gland. Surgical resection of the lung mass was performed. Immunohistochemical study of the tumor tissue indicated a neuroendocrine tumor secreting GHRH. Postoperatively, serum GHRH, GH and IGF-1 levels fell precipitously. At 10 months, IGF-1 levels were mildly elevated and 7 months of 10 mg long-acting octreotide therapy (Sandostatin^® LAR^®) was trialed. At 20 months, off octreotide, serum IGF-1 levels had normalized, acromegalic features were receding, and the patient?s daily insulin requirements had decreased by 57%.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0226-7
- Authors
  - Peter W. Butler, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) Clinical Endocrinology Branch Bethesda MD USA
  - Craig S. Cochran, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) Clinical Endocrinology Branch Bethesda MD USA
  - Maria J. Merino, National Institutes of Health National Cancer Institute (NCI) Bethesda MD USA
  - Dao M. Nguyen, National Institutes of Health National Cancer Institute (NCI) Bethesda MD USA
  - David S. Schrump, National Institutes of Health National Cancer Institute (NCI) Bethesda MD USA
  - Phillip Gorden, National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) Clinical Endocrinology Branch Bethesda MD USA
Primary pituitary lymphoma in immunocompetent patient: diagnostic problems and prolonged follow-up
Abstract Primary pituitary lymphoma in immunocompetent patients is a rare disease and has been described in less than 20 cases. Moreover, low-grade lymphomas constitute only 3% of all primary central nervous system lymphoma. The objective of this report is to report a low-grade primary pituitary lymphoma, diagnostic problems and to give more evidence about the evolution of this rare disease. A 49 y.o. woman was referred to our clinic with symptoms of hypopituitarism. A diagnostic work-up showed mild anemia, an erythrocyte sedimentation rate of 122 mm/h and a negative Elisa test for HIV. Panhypopituitarism was confirmed and the MRI showed a 20 mm sellar and suprasellar enhancing mass with a thickening of the pituitary stalk, chiasmal compression and bitemporal hemianopsia. She underwent transsphenoidal resection only 10 months later for non medical reasons. During this period she was clinically asymptomatic on hormonal replacement therapy. A new MRI showed regression of the suprasellar extension and invasion to the left cavernous sinus. A firm and infiltrative mass was found during transsphenoidal surgery, and only partial resection was performed. Biopsy showed a low-grade lymphoplasmacytic lymphoma. Staging was negative for other localizations. She was given chemotherapy and localized radiotherapy. Four years after surgery, the sellar MRI showed a 10 mm residual sellar mass with the persistence of a cavernous sinus invasion and she is considered to be in remission. The neurosurgeon and clinician should consider primary pituitary lymphoma as a potential cause of a sellar mass, especially in the presence of diabetes insipidus and an enhancing invasive mass. Neurosurgical biopsy is crucial for a correct diagnosis and prognosis could be better than classic CNS primary lymphomas.
- Content Type Journal Article
- DOI 10.1007/s11102-010-0219-6
- Authors
  - Carmen A. Carrasco, Pontificia Universidad Católica de Chile Department of Endocrinology, Faculty of Medicine Lira 85, 5º Piso Santiago Chile
  - David Rojas-Z, Universidad de Chile Department of Neurological Sciences, Institute of Neurosurgery Asenjo José Manuel Infante 553, Providencia Santiago Chile
  - Renato Chiorino, Universidad de Chile Department of Neuropathology, Institute of Neurosurgery Asenjo José Manuel Infante 553, Providencia Santiago Chile
  - Gilberto González, Pontificia Universidad Católica de Chile Department of Endocrinology, Faculty of Medicine Lira 85, 5º Piso Santiago Chile
Temozolomide (Temodar®) and capecitabine (Xeloda®) treatment of an aggressive corticotroph pituitary tumor
Abstract Only rarely do corticotroph pituitary tumors become invasive leading to symptoms caused by compression of cranial nerves and other local structures. When aggressive pituitary neuroendocrine tumors do develop, conventional treatment options are of limited success. A 50-year-old man developed a giant invasive corticotroph pituitary tumor 2 years after initial presentation. His tumor and symptoms failed to respond to maximal surgical, radio-surgical, radiation and medical therapy and a bilateral adrenalectomy was done. He subsequently developed rapid growth of his tumor leading to multiple cranial nerve deficits. He was administered salvage chemotherapy with capecitabine and temozolomide (CAPTEM), a novel oral chemotherapy regimen developed at our institution for treatment of neuroendocrine tumors. After two cycles of CAPTEM, his tumor markedly decreased in size and ACTH levels fell by almost 90%. Despite further decreases in ACTH levels, his tumor recurred after 5 months with increased avidity on PET scan suggesting a transformation to a more aggressive phenotype. Temozolomide had been reported to be effective against other pituitary tumors and this case adds to this literature demonstrating its use along with capecitabine (CAPTEM) against a corticotroph tumor. Further evaluation of the CAPTEM regimen in patients with pituitary neuroendocrine tumors which fail to respond to classic treatments is warranted.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0211-1
- Authors
  - Marie S. Thearle, Columbia University College of Physicians and Surgeons Department of Medicine, Division of Medical Oncology 650 West 168th St. BB 20-05 New York NY 10032 USA
  - Pamela U. Freda, Columbia University College of Physicians and Surgeons Department of Medicine, Division of Medical Oncology 650 West 168th St. BB 20-05 New York NY 10032 USA
  - Jeffrey N. Bruce, Columbia University College of Physicians and Surgeons Department of Neurosurgery, Division of Medical Oncology New York NY 10032 USA
  - Steven R. Isaacson, Columbia University College of Physicians and Surgeons Department of Radiation Oncology, Division of Medical Oncology New York NY 10032 USA
  - Yoomi Lee, Columbia University College of Physicians and Surgeons Experimental Therapeutics Program, Division of Medical Oncology New York NY 10032 USA
  - Robert L. Fine, Columbia University College of Physicians and Surgeons Department of Medicine, Division of Medical Oncology 650 West 168th St. BB 20-05 New York NY 10032 USA
Acromegaly with negative pituitary MRI and no evidence of ectopic source: the role of transphenoidal pituitary exploration?
Abstract Growth hormone (GH) producing adenomas of the pituitary gland are usually macroadenomas (>10 mm in size). Often these adenomas are locally invasive by the time of diagnosis. Acromegaly secondary to a very small pituitary microadenoma not visualized on pituitary magnetic resonance (MR) imaging is rare. We report a patient with acromegaly and an unremarkable pituitary MR imaging who had negative work up for ectopic growth hormone-releasing hormone (GHRH) or GH secreting tumors. Transsphenoidal pituitary exploration revealed a pituitary adenoma located on the left side of the sella against the medial wall of the cavernous sinus extending posteriorly along the floor of the sella all the way to the right side. The acromegaly was treated with resection of the pituitary adenoma and normalization of serum insulin-like growth factor 1 (IGF-1) and GH levels. In a patient with acromegaly and unremarkable pituitary MR imaging, with no evidence of ectopic GH and GHRH production, transsphenoidal pituitary exploration is a reasonable approach and may result in clinical improvement and biochemical cure in the hand of experienced surgeon. This approach may avoid long term medical treatment with its associated cost.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0205-z
- Authors
  - Sameera Daud, 452 Old Hook Road Emerson NJ 07630 USA
  - Amir H. Hamrahian, The Endocrinology and Metabolism Institute, Cleveland Clinic The Department of Endocrinology 9500 Euclid Ave, Desk A53 Cleveland OH 44195 USA
  - Robert J. Weil, The Neurological Institute, Cleveland Clinic The Brain Tumor and Neuro-Oncology Center, Department of Neurosurgery Cleveland OH USA
  - Marwan Hamaty, The Endocrinology and Metabolism Institute, Cleveland Clinic The Department of Endocrinology 9500 Euclid Ave, Desk A53 Cleveland OH 44195 USA
  - Richard A. Prayson, Cleveland Clinic Department of Pathology and Laboratory Medicine Cleveland OH USA
  - Leann Olansky, The Endocrinology and Metabolism Institute, Cleveland Clinic The Department of Endocrinology 9500 Euclid Ave, Desk A53 Cleveland OH 44195 USA
Cushing disease as possible cause of persistent growth failure despite growth hormone therapy in a small for gestational age male
Abstract Growth hormone (GH) therapy in children with small for gestational age (SGA) has been shown to be of significant therapeutic benefit. We report the case of an 11-year-old Caucasian male who developed early adrenarche, hypertension and insulin resistance on GH therapy for SGA and profound short stature (ht ?5 SD). This patient demonstrated a poor response to GH therapy and developed physical and biochemical findings of insulin resistance responsive to metformin therapy. He remained hypertensive, however, and continued to have elevated serum dehydroepiandrosterone sulfate levels. Urinary free cortisol excretion was subsequently found to be elevated. The diagnosis of Cushing?s disease was confirmed with inferior petrosal sinus sampling and pituitary MRI. The patient underwent partial adenohypophysectomy with resulting normalization of plasma cortisol levels and associated symptoms. Our patient?s diagnosis of Cushing?s disease was complicated by his past history of poor growth since birth and history of SGA. The signs of Cushing?s disease did not overtly appear until GH therapy was initiated to help treat severe short stature. It is possible that the metabolic effects of GH therapy unmasked the presence of underlying Cushing?s disease.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0201-3
- Authors
  - MaryKathleen Heneghan, Medical College of Wisconsin Section of Pediatric Endocrinology and Diabetes, Department of Pediatrics 8701 Watertown Plank Road Milwaukee WI 53226-0509 USA
  - Ramin Alemzadeh, Medical College of Wisconsin Section of Pediatric Endocrinology and Diabetes, Department of Pediatrics 8701 Watertown Plank Road Milwaukee WI 53226-0509 USA
Sellar collision tumor involving pituitary gonadotroph adenoma and chondroma: a potential clinical diagnosis
Abstract We report on a 74-year-old male patient who presented with progressive neuroophthalmologic symptoms soon after the administration of a long-acting gonadotropin-releasing hormone agonist for treatment of a prostate cancer. Imaging revealed a destructively growing and extensively calcified sellar mass inconsistent with a pituitary adenoma. A transseptal transsphenoidal tumor mass reduction yielded a histological diagnosis of a collision tumor comprised of a gonadotroph adenoma intermingled with osteochondroma. We discuss a potential causal relationship between the administration of the long-acting gonadotropin-releasing hormone agonist and the sudden appearance of the previously unsuspected sellar lesion. Although the association of these two tumors is very likely coincidental, the possibility of causal relationship is addressed.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0199-6
- Authors
  - Rahel Sahli, Department of Endocrinology, Diabetes and Clinical Nutrition, Bern University Hospital and University of Bern, 3010 Bern, Switzerland
  - Emanuel Christ, Department of Endocrinology, Diabetes and Clinical Nutrition, Bern University Hospital and University of Bern, 3010 Bern, Switzerland
  - Dominique Kuhlen, Department of Neurosurgery, Bern University Hospital and University of Bern, 3010 Bern, Switzerland
  - Olivier Giger, Institute of Pathology, University of Bern, 3010 Bern, Switzerland
  - Istvan Vajtai, Institute of Pathology, University of Bern, 3010 Bern, Switzerland
Possible role of a radiation-induced p53 mutation in a Nelson?s syndrome patient with a fatal outcome
Abstract Nelson?s syndrome (NS) is characterized by the appearance and/or progression of ACTH-secreting pituitary macroadenomas in patients who had previously undergone bilateral adrenalectomy for the treatment of Cushing?s disease. Such corticotroph macroadenomas respond poorly to currently available therapeutic options which include surgery, radiotherapy and chemotherapy. P53 protein accumulation may be detected by immunohistochemistry in pituitary corticotroph adenomas and it has been suggested that it might be causally related to tumor development. Wild type P53 protein plays an important role in the cellular response to ionizing radiation and other DNA damaging agents and is mutated in many human tumors. In this study we report an adult male patient with NS who underwent both transsphenoidal and transcranial pituitary surgeries, conventional and stereotaxic radiotherapy and brachytherapy. Despite of the efforts to control tumor mass and growth, this macroadenoma displayed relentless growth and aggressive behavior. DNA extracted from the first two surgical samples, as well as DNA from peripheral blood leukocytes disclosed normal p53 sequence. DNA extracted from tumor samples obtained at surgeries performed after pituitary irradiation carried a somatic heterozygous mutation, consisting of a deletion of four cytosines between nucleotides 12,144?12,149 in exon 4 of the p53 gene. This frameshift mutation creates a stop codon in exon 4 excluding the expression of a functional protein from the defective allele. These data demonstrate a possible association between the P53 protein loss of function induced by radiotherapy and the aggressive course of the disease in this patient.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0194-y
- Authors
  - Emilia Modolo Pinto, Faculdade de Medicina da Universidade de São Paulo Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular LIM/42, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas São Paulo Brazil
  - Sheila A. C. Siqueira, Faculdade de Medicina da Universidade de São Paulo Divisão de Anatomia Patológica, Hospital das Clínicas São Paulo Brazil
  - Priscilla Cukier, Faculdade de Medicina da Universidade de São Paulo Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular LIM/42, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas São Paulo Brazil
  - Maria C. B. V. Fragoso, Faculdade de Medicina da Universidade de São Paulo Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular LIM/42, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas São Paulo Brazil
  - Chin Jia Lin, Faculdade de Medicina da Universidade de São Paulo Laboratório de Patologia Molecular, LIM22, Departamento de Patologia Av. Dr. Arnaldo, 455, sala 1144 São Paulo 01246-903 Brazil
  - Berenice Bilharinho de Mendonca, Faculdade de Medicina da Universidade de São Paulo Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular LIM/42, Disciplina de Endocrinologia e Metabologia, Hospital das Clínicas São Paulo Brazil
Rathke?s cleft cyst presenting as bilateral abducens nerve palsy
Abstract We present a patient with a Rathke?s cleft cyst who presented with rapidly progressive bilateral 6th nerve palsy. A 20-year-old woman with a history of cleft palate, hypertension, and hydronephrotic kidneys presented with a one month history of headache, associated with dizziness and diplopia on horizontal gaze. Examination was significant for profound bilateral 6th nerve palsies. Magnetic resonance imaging showed a hypodense mass that filled the sella and compressed the right cavernous sinus without contacting the optic chiasm. Pituitary function was normal. An endoscopic, transnasal transsphenoidal resection of the lesion was performed; microscopic examination revealed a Rathke?s cleft cyst. Surgical excision resulted in near complete resolution of the bilateral 6th nerve palsy. Rathke?s cleft cysts are an unusual cause of bilateral sixth nerve palsy and represent a potential cause of cranial neuropathy.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0192-0
- Authors
  - Vinni Grover, Cleveland Clinic Department of Endocrinology, Diabetes and Metabolism, Endocrinology and Metabolism Institute 9500 Euclid Avenue Cleveland OH 44195 USA
  - Amir H. Hamrahian, Cleveland Clinic Department of Endocrinology, Diabetes and Metabolism, Endocrinology and Metabolism Institute 9500 Euclid Avenue Cleveland OH 44195 USA
  - Richard A. Prayson, Cleveland Clinic The Department of Pathology and Laboratory Medicine Cleveland OH USA
  - Robert J. Weil, Cleveland Clinic The Brain Tumor and Neuro-Oncology Center, Department of Neurosurgery, The Neurological Institute 9500 Euclid Avenue Cleveland OH 44195 USA
Pituitary and systemic autoimmunity in a case of intrasellar germinoma
Abstract Germinomas arising in the sella turcica are difficult to differentiate from autoimmune hypophysitis because of similar clinical and pathological features. This differentiation, nevertheless, is critical for patient care due to different treatments of the two diseases. We report the case of an 11-year-old girl who presented with diabetes insipidus and growth retardation, and was found to have an intra- and supra-sellar mass. Initial examination of the pituitary biopsy showed diffuse lymphocytic infiltration of the adenohypophysis and absent placental alkaline phosphatase expression, leading to a diagnosis of hypophysitis and glucocorticoid treatment. Because of the lack of clinical and radiological response, the pituitary specimen was re-examined, revealing this time the presence of scattered c-kit and Oct4 positive germinoma cells. The revised diagnosis prompted the initiation of radiotherapy, which induced disappearance of the pituitary mass. Immunological studies showed that the patient?s serum recognized antigens expressed by the patient?s own germinoma cells, as well as pituitary antigens like growth hormone and systemic antigens like the Sjögren syndrome antigen B and alpha-enolase. The study first reports the presence of pituitary and systemic antibodies in a patient with intrasellar germinoma, and reminds us that diffuse lymphocytic infiltration of the pituitary gland and pituitary antibodies does not always indicate a diagnosis of autoimmune hypophysitis.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0187-x
- Authors
  - Angelika Gutenberg, Georg August University Göttingen Department for Neurosurgery Göttingen Germany
  - Jennifer J. Bell, Texas Children?s Hospital Department for Pediatrics Houston TX USA
  - Isabella Lupi, University of Pisa Department of Endocrinology Pisa Italy
  - Shey-Cherng Tzou, Johns Hopkins University Department for Pathology Baltimore MD USA
  - Melissa A. Landek-Salgado, Johns Hopkins University Department for Pathology Baltimore MD USA
  - Hiroaki Kimura, Johns Hopkins University Department for Pathology Baltimore MD USA
  - Jack Su, Texas Children?s Hospital Department for Pediatrics Houston TX USA
  - Lefkothea P. Karaviti, Texas Children?s Hospital Department for Pediatrics Houston TX USA
  - Roberto Salvatori, Johns Hopkins University Division of Endocrinology, Department of Medicine Baltimore MD USA
  - Patrizio Caturegli, Johns Hopkins University Department for Pathology Baltimore MD USA
Brain and optic chiasmal herniations into sella after cabergoline therapy of giant prolactinoma
Abstract Optic chiasmal herniation following dopamine agonist therapy is a rare complication in patients with giant prolactinomas. But there are a few case reports of brain and chiasmal herniation following medical therapy in such cases. We report a young man who developed secondary visual loss and seizures after 6 months of medical treatment with cabergoline for giant prolactinoma. Magnetic resonance imaging of hypothalamic pituitary region revealed optic chiasmal and frontal lobe herniation into sella. There was marginal improvement in his vision after cabergoline dose reduction. The present case report highlights frontal lobe herniation in conjunction with optic chiasmal herniation as a very rare complication of medical therapy of giant prolactinoma. Different treatment options of this condition are being discussed.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0179-x
- Authors
  - Dinesh Kumar Dhanwal, Maulana Azad Medical College Department of Medicine and Division of Endocrinology 115, B.L. Taneja Block, Bahadur Shah Zafar Marg New Delhi 110002 India
  - Ashok Kumar Sharma, Maulana Azad Medical College Department of Radiology, G.B. Pant Hospital New Delhi India
Multiple head and neck tumors following treatment for craniopharyngioma
Abstract To report a unique case of a patient with multiple head and neck tumors following treatment for craniopharyngioma. Retrospective review of the medical record. There is an association between radiation treatment for primary intracranial malignancies and the subsequent development of other intracranial tumors. At the same time, reports regarding the association between thyroid cancer and radiation exposure are abundant within the literature. We present a unique case of a young female who underwent resection and radiation treatment for a craniopharyngioma in 1988. With the exception of bitemporal visual loss and panhypopituitarism requiring hormone replacement therapy, the patient had a relatively uneventful course until 2005. At that time, she developed hearing loss and incapacitating vertigo, which was ultimately attributed to a temporal bone Langerhans histiocytosis. A year and a half later, she was noted to have a thyroid nodule, the pathology of which revealed papillary carcinoma. Seven months after that, she developed new auditory and vestibular symptoms attributable to an acoustic neuroma. This case is remarkable because neither Langerhans histiocytosis following radiation of a craniopharyngioma nor this distinctive combination of head and neck tumors in general have ever been previously reported.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0177-z
- Authors
  - Alyson Dobracki, Department of Internal Medicine, Crozer Chester Medical Center, 3 East, One Medical Center Blvd., Upland, PA 19013, USA
  - Paul Woolf, Department of Internal Medicine, Crozer Chester Medical Center, 3 East, One Medical Center Blvd., Upland, PA 19013, USA
Hematologic neoplasias and acromegaly
Abstract We report a 59-year-old acromegalic woman, who presented with generalized bone pain, weakness, fatigue and foamy urine, who was found to have multiple myeloma (MM); and a 60-year-old acromegalic woman with dizziness, vomiting and abdominal pain, high blood pressure and splenomegaly that was posteriorly diagnosed as having Waldenstrom?s macroglobulinemia (WM). Acromegaly is an uncommon disease and epidemiological studies have provided increasingly debated evidence that elevated IGF-I levels might enhance the neoplastic risk, and that cancers constitute the third leading cause of mortality in acromegaly. It is known that GH and IGF-I can activate B cell lymphocytes, and that IGF-I receptor is universally expressed in MM cells. Although the complication of acromegaly with WM or MM in patients has rarely been reported until now, we described two case reports of acromegalic patients with those hematological neoplasias, which allow a discussion about this controversial issue.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0176-0
- Authors
  - Flavia Regina P. Barbosa, Hospital Universitário Clementino Fraga Filho/Universidade Federal do Rio de Janeiro Division of Endocrinology, Department of Internal Medicine Rua Nascimento Silva, 555/101 Ipanema, Rio de Janeiro 22421-020 Brazil
  - Leonardo Vieira Neto, Hospital Universitário Clementino Fraga Filho/Universidade Federal do Rio de Janeiro Division of Endocrinology, Department of Internal Medicine Rua Nascimento Silva, 555/101 Ipanema, Rio de Janeiro 22421-020 Brazil
  - Giovanna Aparecida B. Lima, Hospital Universitário Clementino Fraga Filho/Universidade Federal do Rio de Janeiro Division of Endocrinology, Department of Internal Medicine Rua Nascimento Silva, 555/101 Ipanema, Rio de Janeiro 22421-020 Brazil
  - Luiz Eduardo Wildemberg, Hospital Universitário Clementino Fraga Filho/Universidade Federal do Rio de Janeiro Division of Endocrinology, Department of Internal Medicine Rua Nascimento Silva, 555/101 Ipanema, Rio de Janeiro 22421-020 Brazil
  - Rodrigo Portugal, Hospital Universitário Clementino Fraga Filho/Universidade Federal do Rio de Janeiro Division of Hematology, Department of Internal Medicine Rio de Janeiro Brazil
  - Monica R. Gadelha, Hospital Universitário Clementino Fraga Filho/Universidade Federal do Rio de Janeiro Division of Endocrinology, Department of Internal Medicine Rua Nascimento Silva, 555/101 Ipanema, Rio de Janeiro 22421-020 Brazil
Self-limited acute hepatotoxicity caused by pegvisomant
Abstract We present a case of acute severe hepatitis in a patient with acromegaly receiving combination therapy with somatostatin analogs and pegvisomant. Hepatitis resolved completely 18 weeks after diagnosis of hypertransaminasemia without discontinuation of therapy and with a close clinical and biochemical follow-up. In this case, despite the severity of the hepatitis, therapy could be continued as hypertransaminasemia was gradually decreasing after the maximum peak. We also review the literature on toxic hepatitis associated to pegvisomant therapy analyzing the etiology, clinical predisposing factors and natural evolution.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0173-3
- Authors
  - A. Soto Moreno, Virgen del Rocio University Hospital Department of Endocrinology and Nutrition, Instituto de Biomedicina de Sevilla (IBIS) Manuel Siurot S/N Avenue 41013 Seville Spain
  - R. Guerrero Vázquez, Virgen del Rocio University Hospital Department of Endocrinology and Nutrition, Instituto de Biomedicina de Sevilla (IBIS) Manuel Siurot S/N Avenue 41013 Seville Spain
  - E. Venegas Moreno, Virgen del Rocio University Hospital Department of Endocrinology and Nutrition, Instituto de Biomedicina de Sevilla (IBIS) Manuel Siurot S/N Avenue 41013 Seville Spain
  - S. Palma Milla, Virgen del Rocio University Hospital Department of Endocrinology and Nutrition, Instituto de Biomedicina de Sevilla (IBIS) Manuel Siurot S/N Avenue 41013 Seville Spain
  - J. P. Castaño, University of Córdoba Department of Cell Biology, Physiology and Immunology Córdoba Spain
  - A. Leal Cerro, Virgen del Rocio University Hospital Department of Endocrinology and Nutrition, Instituto de Biomedicina de Sevilla (IBIS) Manuel Siurot S/N Avenue 41013 Seville Spain
Recurrent spindle cell oncocytoma of the pituitary, a case report and review of literature
Abstract Spindle cell oncocytoma (SCO) is a rare non-functioning tumour of the pituitary which has just been formally recognized as a distinct entity by the 2007 WHO classification of brain tumours. We report a case of SCO who presented with symptoms of visual blurring, weight loss, intermittent vomiting and excessive tiredness of several months duration. Investigations revealed a bitemporal visual field defect, a panhypopituitary hormonal profile and a large pituitary tumour with suprasellar extension. He underwent a successful trans-sphenoidal resection of the pituitary tumour but it subsequently recurred twice at 9 months interval which required further two debulking procedures. A diagnosis of SCO was made based on its unique histologic and staining properties. To date there are only ten reported cases of SCO in total with only two of these cases being recurrent. Our case displayed the most aggressive clinical course despite having a low Ki-67 index contrary to the previously reported cases of recurrent SCO.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0170-6
- Authors
  - Yared N. Demssie, Lancashire Teaching Hospitals NHS Foundation Trust Department of Endocrinology Sharoe Green Lane, Fullwood Preston PR2 9HT UK
  - Jacob Joseph, Lancashire Teaching Hospitals NHS Foundation Trust Department of Neuropathology Sharoe Green Lane, Fullwood Preston PR2 9HT UK
  - Timothy Dawson, Lancashire Teaching Hospitals NHS Foundation Trust Department of Neuropathology Sharoe Green Lane, Fullwood Preston PR2 9HT UK
  - Gareth Roberts, Lancashire Teaching Hospitals NHS Foundation Trust Department of Neurosurgery Sharoe Green Lane, Fullwood Preston PR2 9HT UK
  - John de Carpentier, Lancashire Teaching Hospitals NHS Foundation Trust Department of Neurosurgery Sharoe Green Lane, Fullwood Preston PR2 9HT UK
  - Simon Howell, Lancashire Teaching Hospitals NHS Foundation Trust Department of Endocrinology Sharoe Green Lane, Fullwood Preston PR2 9HT UK
Secondary resistance to cabergoline therapy in a macroprolactinoma: a case report and literature review
Abstract Primary resistance to dopamine agonists occurs in 10?15% of prolactinomas but secondary resistance following initial biochemical and anti-proliferative response is very rare and has only been hitherto described in four previous cases, two with bromocriptine and two with cabergoline. We describe a case of a 57-year-old woman who presented with a large macroprolactinoma with suprasellar extension. She was initially treated with bromocriptine therapy with a resolution of symptoms, marked reduction in prolactin concentration and complete tumour shrinkage; a response which was subsequently maintained on cabergoline. After 8 years of dopamine agonist therapy, her prolactin concentration began to rise and there was symptomatic recurrence of her tumour despite escalating doses of cabergoline up to 6 mg weekly. Non-compliance was outruled by observed inpatient drug administration. The patient underwent surgical debulking followed by radiotherapy with good response. This case adds to the previous two cases of secondary resistance to cabergoline therapy in prolactinomas a marked initial response. While the mechanism of secondary resistance remains unknown and not possible to predict, close observation of prolactinoma patients on treatment is necessary.
- Content Type Journal Article
- DOI 10.1007/s11102-009-0168-0
- Authors
  - L. A. Behan, Beaumont Hospital and RCSI Medical School Department of Academic Endocrinology and Diabetes Dublin 9 Ireland
  - M. S. Draman, Beaumont Hospital and RCSI Medical School Department of Academic Endocrinology and Diabetes Dublin 9 Ireland
  - C. Moran, Beaumont Hospital and RCSI Medical School Department of Academic Endocrinology and Diabetes Dublin 9 Ireland
  - T. King, Beaumont Hospital and RCSI Medical School Department of Academic Endocrinology and Diabetes Dublin 9 Ireland
  - R. K. Crowley, Beaumont Hospital and RCSI Medical School Department of Academic Endocrinology and Diabetes Dublin 9 Ireland
  - E. P. O?Sullivan, Beaumont Hospital and RCSI Medical School Department of Academic Endocrinology and Diabetes Dublin 9 Ireland
  - D. Smith, Beaumont Hospital and RCSI Medical School Department of Academic Endocrinology and Diabetes Dublin 9 Ireland
  - C. J. Thompson, Beaumont Hospital and RCSI Medical School Department of Academic Endocrinology and Diabetes Dublin 9 Ireland
  - A. Agha, Beaumont Hospital and RCSI Medical School Department of Academic Endocrinology and Diabetes Dublin 9 Ireland
Efficacy of the combined cabergoline and octreotide treatment in a case of a dopamine-agonist resistant macroprolactinoma
Abstract Prolactinomas in males can be voluminous macroadenomas invading the surrounding structures. Medical therapy with dopamine agonists (the treatment of choice for these tumours) may be ineffective in the case of pharmacological resistance. In such cases, even surgical and/or radiation therapy cannot be curative due to the invasive potential of the adenoma. Hence, the appropriate therapeutic approach for these tumours is still a relevant clinical problem for endocrinologists. We report the history of an adolescent male who was diagnosed with a large invasive macroprolactinoma in 2002. He had severe bitemporal hemianopsia and hypopituitarism; prolactin levels at diagnosis were higher than 8,000 ng/ml. Medical therapy with cabergoline was initiated and resulted in decreased prolactin levels but not complete normalisation (maximal tolerated dose 3 mg/day). However, due to the worsening of the visual defect, the patient was operated in July 2004 through the trans-nasal approach and 2 years later through both the transcranial and the transphenoidal approaches. After the second surgery, a significant reduction of tumour mass was obtained. Immunohistochemistry for somatostatin receptors (sstr) subtypes showed a positive staining with the anti-sstr5 antibody. A scintigraphy with 111In-pentetreotide (Octreoscan) revealed a very intense tracer uptake in the sellar region. The administration of long-acting octreotide was initiated. After 12 months of therapy, prolactin levels normalised for the first time. Pituitary MRI did not reveal any tumor progression during a 2-year follow-up. This is a case of an invasive dopamine-resistant macroprolactinoma that was successfully controlled by extensive surgery and combined treatment with cabergoline and octreotide. The expression and functionality of sstr should be investigated in these tumours since a combined therapy with cabergoline and octreotide may be a good therapeutic course of action for select cases.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0162-y
- Authors
  - Alessandra Fusco, Università Cattolica del Sacro Cuore Section of Endocrinology, Department of Internal Medicine Via Cassia 901 00168 Rome Italy
  - Francesca Lugli, Università Cattolica del Sacro Cuore Section of Endocrinology, Department of Internal Medicine Via Cassia 901 00168 Rome Italy
  - Eugenia Sacco, Università Cattolica del Sacro Cuore Section of Endocrinology, Department of Internal Medicine Via Cassia 901 00168 Rome Italy
  - Laura Tilaro, Università Cattolica del Sacro Cuore Section of Endocrinology, Department of Internal Medicine Via Cassia 901 00168 Rome Italy
  - Antonio Bianchi, Università Cattolica del Sacro Cuore Section of Endocrinology, Department of Internal Medicine Via Cassia 901 00168 Rome Italy
  - Flavia Angelini, Università Cattolica del Sacro Cuore Laboratory of Vascular Biology and Genetics, Department of Internal Medicine Rome Italy
  - Anna Tofani, Sant?Andrea Hospital Nuclear Medicine Rome Italy
  - Angela Barini, Università Cattolica del Sacro Cuore Department of Clinical Biochemistry Rome Italy
  - Libero Lauriola, Università Cattolica del Sacro Cuore Institute of Pathology Rome Italy
  - Giulio Maira, Università Cattolica del Sacro Cuore Institute of Neurosurgery Rome Italy
  - Alfredo Pontecorvi, Università Cattolica del Sacro Cuore Section of Endocrinology, Department of Internal Medicine Via Cassia 901 00168 Rome Italy
  - Laura de Marinis, Università Cattolica del Sacro Cuore Section of Endocrinology, Department of Internal Medicine Via Cassia 901 00168 Rome Italy
Primary CNS lymphoma with bilateral symmetric hypothalamic lesions presenting with panhypopituitarism and diabetes insipidus
Abstract We present an unusual case of primary central nervous system (CNS) lymphoma presenting with bilateral symmetric hypothalamic lesions causing diabetes insipidus and hypopituitarism. A 50-year-old male presented initially with mental status changes, polyuria and polydipsia. The patient was determined to have diabetes insipidus (DI) and significant anterior pituitary deficiencies resulting in symptomatic pleural and pericardial effusions. Brain MRI with contrast demonstrated bilateral enhancement of his hypothalamus extending to the optic tract. The extensive diagnostic workup that ensued on his initial presentation was non-diagnostic as he had no obvious site of involvement that was easily accessible to biopsy. With close follow-up, the patient had rapid radiographic progression of his disease to his cerebral hemispheres. He therefore underwent brain biopsy and was diagnosed with primary CNS large B cell lymphoma. Chemotherapy has resulted in disease remission with resolution of MRI findings, but the patient has not had resolution of the hypopituitarism or DI. This case highlights the unique diagnostic challenge of patients with isolated hypothalamic lesions.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0166-7
- Authors
  - Brian Thomas Layden, Northwestern University Feinberg School of Medicine Department of Medicine, Division of Endocrinology, Metabolism and Molecular Medicine 645 N. Michigan Ave., Suite 530 Chicago IL 60611 USA
  - Steve Dubner, Northwestern University Feinberg School of Medicine Department of Pathology Chicago IL USA
  - Daniel J. Toft, Northwestern University Feinberg School of Medicine Department of Medicine, Division of Endocrinology, Metabolism and Molecular Medicine 645 N. Michigan Ave., Suite 530 Chicago IL 60611 USA
  - Peter Kopp, Northwestern University Feinberg School of Medicine Department of Medicine, Division of Endocrinology, Metabolism and Molecular Medicine 303 E. Chicago Ave., Tarry 15-755 Chicago IL 60611 USA
  - Sean Grimm, Northwestern University Feinberg School of Medicine Department of Neurology Chicago IL USA
  - Mark E. Molitch, Northwestern University Feinberg School of Medicine Department of Medicine, Division of Endocrinology, Metabolism and Molecular Medicine 645 N. Michigan Ave., Suite 530 Chicago IL 60611 USA
Sellar and suprasellar mixed germ cell tumor mimicking a pituitary adenoma
Abstract Germ cell tumors (GCT) are a heterogeneous group of lesions whose origin is not well established. Several cases of primary intrasellar germinomas have been reported, however non-germinomatous GCT have rarely been described. We report the case of a young adult male patient with a mixed GCT that presented with a sellar tumor with suprasellar extension. The patient seeked medical attention because of seizures and magnetic resonance imaging evidenced a tumor of the sellar region. Hyperprolactinemia was also present and dopamine agonist therapy was started. As there was a rapid tumor growth and the patient had concomitant central diabetes insipidus and elevated testosterone levels, a GCT was suspected and confirmed by elevated serum concentration of ?-human chorionic gonadotrophin. Patient underwent surgical resection of the tumor and histopathological examination confirmed the diagnosis of a mixed GCT. Chemotherapy was initiated, followed by conventional radiotherapy. In conclusion, although pituitary adenomas respond for the vast majority of sellar tumors, concomitant symptoms such as central diabetes insipidus and rapid tumor growth should raise the suspicion of a diverse diagnosis. The present report intend not only to show a rare case of sellar and suprasellar mixed GCT but also to remind clinicians that if laboratory findings do not fit into patient?s diagnosis (such as high testosterone levels in our patient), then the diagnosis should be reviewed.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0161-z
- Authors
  - Luiz Eduardo Armondi Wildemberg, Universidade Federal do Rio de Janeiro (UFRJ) Endocrinology Unit, Hospital Universitário Clementino Fraga Filho Rio de Janeiro Brazil
  - Leonardo Vieira Neto, Universidade Federal do Rio de Janeiro (UFRJ) Endocrinology Unit, Hospital Universitário Clementino Fraga Filho Rio de Janeiro Brazil
  - Giselle Fernandes Taboada, Universidade Federal do Rio de Janeiro (UFRJ) Endocrinology Unit, Hospital Universitário Clementino Fraga Filho Rio de Janeiro Brazil
  - Aline Barbosa Moraes, Universidade Federal do Rio de Janeiro (UFRJ) Endocrinology Unit, Hospital Universitário Clementino Fraga Filho Rio de Janeiro Brazil
  - Jorge Marcondes, Universidade Federal do Rio de Janeiro (UFRJ) Neurosurgery Unit, Hospital Universitário Clementino Fraga Filho Rio de Janeiro Brazil
  - Flávia Lúcia Conceição, Universidade Federal do Rio de Janeiro (UFRJ) Endocrinology Unit, Hospital Universitário Clementino Fraga Filho Rio de Janeiro Brazil
  - Leila Chimelli, Universidade Federal do Rio de Janeiro (UFRJ) Pathology Unit, Hospital Universitário Clementino Fraga Filho Rio de Janeiro Brazil
  - Mônica Roberto Gadelha, Universidade Federal do Rio de Janeiro (UFRJ) Endocrinology Unit, Hospital Universitário Clementino Fraga Filho Rio de Janeiro Brazil
Isolated acquired ACTH deficiency and primary hypothyroidism: a short series and review
Abstract Idiopathic isolated ACTH deficiency, congenital or acquired, is rare. It may be found in association with primary hypothyroidism. Here we describe four cases of acquired idiopathic isolated ACTH deficiency illustrating its importance and variable presentation. All cases had a structurally normal pituitary gland and persistently normal residual pituitary function. Three cases had co-existing primary hypothyroidism. We discuss the protean presentation of this rare but important condition, its treatment, associations, and possible aetiologies.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0164-9
- Authors
  - M. J. Hannon, Cork University Hospital Department of Endocrinology and Metabolism Wilton, Cork Ireland
  - D. J. O?Halloran, Cork University Hospital Department of Endocrinology and Metabolism Wilton, Cork Ireland
Unusual clinical presentations of giant prolactinomas
Abstract Giant prolactinomas are rare pituitary tumors which have scarcely been reported in the literature. We describe three men with unusual presenting features of prolactin-secreting giant pituitary adenomas: prolonged and increasingly disturbing intolerance to light and noise; strange behavior and mood disturbances; and rhinorrhea followed by a finding of cerebrospinal fluid leakage. Treatment with dopamine agonist alleviated all symptoms, with concomitant suppression of plasma prolactin levels and a significant reduction in tumor mass. These cases emphasize the importance of considering unusual symptoms in the differential diagnosis of giant prolactinomas and the effectiveness of medical treatment.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0160-0
- Authors
  - Simona Grozinsky-Glasberg, Institute of Endocrinology & Metabolism, Rabin Medical Center, Beilinson Hospital Petah Tiqwa 49100 Israel
  - Ilan Shimon, Institute of Endocrinology & Metabolism, Rabin Medical Center, Beilinson Hospital Petah Tiqwa 49100 Israel
Successful treatment of Cushing?s disease caused by ectopic intracavernous microadenoma
Abstract Adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas are sometimes difficult to visualize, even with high-quality magnetic resonance imaging, due to their small size and variable location. Sampling the cavernous or inferior petrosal sinus is helpful for confirming the central origin of a tumor, but ectopic corticotroph adenomas in the paraseller region also typically exhibit a high central/peripheral plasma ACTH ratio. We experienced an extremely rare case of Cushing?s disease caused by an ACTH-secreting microadenoma located entirely inside the left cavernous sinus attached to the medial wall (ectopic pituitary adenoma) that was not visible by preoperative MRI. In this case, the microadenoma was completely removed and an endocrinologic cure was achieved. This case reveals that in addition to meticulous sectioning of the pituitary gland, bilateral periglandular inspection with visualization of the medial wall of the cavernous sinus and of the diaphragm should always be performed to detect ectopic parasellar microadenomas when no adenoma is visible by preoperative MRI.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0156-9
- Authors
  - Mitsuteru Koizumi, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
  - Takeshi Usui, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
  - Shozo Yamada, Toranomon Hospital Department of Hypothalamic & Pituitary Surgery Tokyo Japan
  - Ichiro Fujisawa, Kishiwada City Hospital Department of Radiology Kishiwada Japan
  - Tsunehisa Tsuru, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
  - Kazutaka Nanba, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
  - Hanae Hagiwara, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
  - Takashi Kimura, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
  - Tamiko Tamanaha, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
  - Tetsuya Tagami, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
  - Mitsuhide Naruse, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
  - Akira Shimatsu, Department of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center Mukaihata-cho Fukakusa Fushimi-ku Kyoto 612-8555 Japan
Primary pituitary abscess followed by empty sella syndrome in an adolescent girl
Abstract Primary pituitary abscess is a rare pituitary pathology, particularly at a young age and is characterized by atypical clinical features making the diagnosis difficult. Correct diagnosis and therapy are mandatory due to the potentially lethal outcome of pituitary infection. We report the case of an adolescent girl presenting with headache, diabetes insipidus and central thyro-gonadic insufficiency with no history of infection, in whom the intra-operative diagnosis of primary pituitary abscess was made. Bacterial cultures indicated infection with Streptococcus spp. One year after neurosurgery and antibiotic therapy, recovery of diabetes insipidus and pituitary insufficiency was documented except for persistence of subnormal growth hormone secretion. Post-surgery, pituitary magnetic resonance imaging revealed an empty sella syndrome.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0150-2
- Authors
  - Carmen Emanuela Pepene, University of Medicine and Pharmacy Department of Endocrinology Cluj-Napoca Romania
  - Ioana Ilie, University of Medicine and Pharmacy Department of Endocrinology Cluj-Napoca Romania
  - Dan Mihu, University of Medicine and Pharmacy Department of Obstetrics and Gynecology Cluj-Napoca Romania
  - Hora?iu Stan, University of Medicine and Pharmacy Department of Neurosurgery Cluj-Napoca Romania
  - Silviu Albu, University of Medicine and Pharmacy Department of Otorhinolaringology Cluj-Napoca Romania
  - Ileana Duncea, University of Medicine and Pharmacy Department of Endocrinology Cluj-Napoca Romania
Atypical pituitary adenoma with malignant features
Abstract Pituitary carcinoma is characterized by the presence of systemic or central nervous system metastases rather than malignant histological features, making it an anomaly amongst carcinomas. In contrast, aggressive or atypical pituitary adenomas often have a relatively bland histological appearance despite their malignant growth patterns. We now report a case of a 67-years-old male with a giant pituitary tumor with overt pathologic and phenotypic features of malignancy, but the absence of metastases, that evolved from a benign non-functioning gonadotroph macroadenoma treated 10 years earlier. This case represents the first report of a pituitary adenoma with overt malignant histology that does not meet criteria for classification as pituitary carcinoma, helping to complete the pathological spectrum of disease observed with pituitary tumors.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0151-1
- Authors
  - Adam N. Mamelak, Cedars-Sinai Medical Center Department of Neurosurgery, Pituitary Center 8631 W. 3rd Street Los Angeles CA 90048 USA
  - John D. Carmichael, Cedars-Sinai Medical Center Department of Endocrinology Los Angeles CA USA
  - Patricia Park, Cedars-Sinai Medical Center Department of Endocrinology Los Angeles CA USA
  - Sergei Bannykh, Cedars-Sinai Medical Center Department of Pathology Los Angeles CA USA
  - Xuemo Fan, Cedars-Sinai Medical Center Department of Pathology Los Angeles CA USA
  - H. Vivien Bonert, Cedars-Sinai Medical Center Department of Endocrinology Los Angeles CA USA
What are critical outcome measures for patients receiving pituitary replacement following brain injury?
Abstract There are scant prospective studies defining improvements in critical outcome measures with hormone replacement in hypopituitarism secondary to brain injury. We review the tests of cognition and physical function and summarize their use for subjects that are deficient in anterior hormone production during anterior pituitary hormone replacement in brain injury and propose these as the minimal tests that are feasible for a physician to perform in a clinical setting. We summarize the studies conducted to assess outcome measures after brain injury and also report preliminary findings for improvements in cognition and physical function in subjects with brain injury and GH deficiency.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0133-3
- Authors
  - Sorin G. Beca, University of Texas Medical Branch Department of Internal Medicine, Endocrinology Division 301 University Blvd. Galveston TX 77555-1188 USA
  - Walter M. High, University of Kentucky College of Medicine Departments of Physical Medicine and Rehabilitation, Neurosurgery and Psychology, Cardinal Hill Rehabilitation Hospital 2050 Versailles Rd. Lexington KY 40504 USA
  - Brent E. Masel, Transitional Learning Center at Galveston 1528 Post Office Street Galveston TX 77550 USA
  - Kurt A. Mossberg, University of Texas Medical Branch Department of Physical Therapy 301 University Blvd. Galveston TX 77555-1144 USA
  - Randall J. Urban, University of Texas Medical Branch Department of Internal Medicine 301 University Blvd. Galveston TX 77555-0569 USA
Chapter 1: pathophysiology of hypopituitarism in the setting of brain injury
Abstract The complex pathophysiology of traumatic brain injury (TBI) involves not only the primary mechanical event but also secondary insults such as hypotension, hypoxia, raised intracranial pressure and changes in cerebral blood flow and metabolism. It is increasingly evident that these initial insults as well as transient events and treatments during the early injury phase can impact hypothalamic-pituitary function both acutely and chronically after injury. In turn, untreated pituitary hormonal dysfunction itself can further hinder recovery from brain injury. Secondary adrenal insufficiency, although typically reversible, occurs in up to 50% of intubated TBI victims and is associated with lower systemic blood pressure. Chronic anterior hypopituitarism, although reversible in some patients, persists in 25?40% of moderate and severe TBI survivors and likely contributes to long-term neurobehavioral and quality of life impairment. While the rates and risk factors of acute and chronic pituitary dysfunction have been documented for moderate and severe TBI victims in numerous recent studies, the pathophysiology remains ill-defined. Herein we discuss the hypotheses and available data concerning hypothalamic-pituitary vulnerability in the setting of head injury. Four possible pathophysiological mechanisms are considered: (1) the primary brain injury event, (2) secondary brain insults, (3) the stress of critical illness and (4) medication effects. Although each of these factors appears to be important in determining which hormonal axes are affected, the severity of dysfunction, their time course and possible reversibility, this process remains incompletely understood.
- Content Type Journal Article
- DOI 10.1007/s11102-008-0130-6
- Authors
  - Joshua R. Dusick, UCLA David Geffen School of Medicine Division of Neurosurgery Los Angeles CA USA
  - Christina Wang, Harbor-UCLA Medical Center Division of Endocrinology, Department of Medicine Torrance CA USA
  - Pejman Cohan, UCLA David Geffen School of Medicine Division of Endocrinology Los Angeles CA USA
  - Ronald Swerdloff, Harbor-UCLA Medical Center Division of Endocrinology, Department of Medicine Torrance CA USA
  - Daniel F. Kelly, Saint John?s Health Center Neuro-Endocrine Tumor Center, John Wayne Cancer Institute 2200 Santa Monica Blvd. Santa Monica CA 90404 USA
Glucose Metabolism and Visceral Fat in GH Deficient Adults: Two Years of GH-Replacement
The aim of this study was to evaluate the effect of 24 months of growth hormone (GH) replacement on glucose metabolism and visceral fat in 17 adults with GH deficiency: 9 men and 8 women; age 40 ± 1.8 yr. [range 20?61] and body mass index 25 ± 0.8 Kg/m².
- Content Type Journal Article
- DOI 10.1007/s11102-005-0009-8
- Authors
  - Luciana Diniz Carneiro Spina, Serviço de Endocrinologia do Hospital Universitário Clementino Fraga Filho, Faculdade de Medicina Universidade Federal do Rio de Janeiro (UFRJ) Rio de Janeiro RJ Brasil
  - Débora Vieira Soares, Serviço de Endocrinologia do Hospital Universitário Clementino Fraga Filho, Faculdade de Medicina Universidade Federal do Rio de Janeiro (UFRJ) Rio de Janeiro RJ Brasil
  - Rosane Resende de Lima Oliveira Brasil, Serviço de Endocrinologia do Hospital Universitário Clementino Fraga Filho, Faculdade de Medicina Universidade Federal do Rio de Janeiro (UFRJ) Rio de Janeiro RJ Brasil
  - Priscila Marise Lobo, Serviço de Endocrinologia do Hospital Universitário Clementino Fraga Filho, Faculdade de Medicina Universidade Federal do Rio de Janeiro (UFRJ) Rio de Janeiro RJ Brasil
  - Flávia Lúcia Conceição, Serviço de Endocrinologia do Hospital Universitário Clementino Fraga Filho, Faculdade de Medicina Universidade Federal do Rio de Janeiro (UFRJ) Rio de Janeiro RJ Brasil
  - Mário Vaisman, Rua José Linhares 244/ 05, Leblon Cep 22430-220 Rio de Janeiro RJ Brasil
Recurrence of Cushing?s Disease Preceded by the Reappearance of ACTH and Cortisol Responses to Desmopressin Test
At present no single test is considered of absolute value in identifying patients successfully operated for Cushing?s disease who are at risk for recurrence. The present report describes the first two patients in whom ACTH/cortisol abnormal responses to desmopressin disappeared after cure and then clearly reappeared during long-term follow-up several months before the clinical and hormonal features of hypercortisolism became manifest.
- Content Type Journal Article
- DOI 10.1007/s11102-005-0008-9
- Authors
  - Chiara Dall?Asta, Endocrinology Unit, Department of Medical and Surgical Sciences, Istituto Policlinico San Donato University of Milan Via Morandi 30 20097 San Doanto Milanese (MI) Italy
  - Laura Barbetta, Endocrinology Unit, Department of Medical and Surgical Sciences, Istituto Policlinico San Donato University of Milan Via Morandi 30 20097 San Doanto Milanese (MI) Italy
  - Luigi Bonavina, Surgery Unit, Department of Medical and Surgical Sciences, Istituto Policlinico San Donato University of Milan Via Morandi 30 20097 San Doanto Milanese (MI) Italy
  - Paolo Beck-Peccoz, Institute of Endocrine Sciences University of Milan, Ospedale Maggiore IRCCS Via F. Sforza 35 20122 Milan Italy
  - Bruno Ambrosi, Endocrinology Unit, Department of Medical and Surgical Sciences, Istituto Policlinico San Donato University of Milan Via Morandi 30 20097 San Doanto Milanese (MI) Italy