Van Wyk-Grumbach syndrome (VWGS) is a rare complication of prolonged untreated juvenile hypothyroidism characterized by precocious puberty and enlarged multicystic ovaries. A 13-year-old girl visited our outpatient clinic due to menstrual irregularities. She had precocious puberty, pituitary hyperplasia and multiple cystic ovaries in addition to clinical signs of severe congenital hypothyroidism. After the initiation of L-thyroxine therapy, the symptoms were alleviated in a short time. This rare syndrome is easy to be misdiagnosed as pituitary and ovarian tumor. High degree of suspicion and timely diagnosis can prevent unnecessary surgical procedures because the symptoms can be reversed with thyroid hormone supplementation.
Adolescent ; Congenital Hypothyroidism ; complications ; diagnosis ; etiology ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Humans ; Hyperpituitarism ; Hyperplasia ; Menstruation Disturbances ; etiology ; Ovarian Cysts ; diagnosis ; etiology ; Ovary ; pathology ; Pituitary Gland ; pathology ; Puberty, Precocious ; diagnosis ; etiology ; Syndrome ; Thyroxine ; therapeutic use

No abstract available.
Acromegaly*

Management of pituitary tumors is multidisciplinary, with medical therapy playing an increasingly important role. With the exception of prolactin-secreting tumors, surgery is still considered the first-line treatment for the majority of pituitary adenomas. However, medical/pharmacological therapy plays an important role in controlling hormone-producing pituitary adenomas, especially for patients with acromegaly and Cushing disease (CD). In the case of non-functioning pituitary adenomas (NFAs), pharmacological therapy plays a minor role, the main objective of which is to reduce tumor growth, but this role requires further studies. For pituitary carcinomas and atypical adenomas, medical therapy, including chemotherapy, acts as an adjuvant to surgery and radiation therapy, which is often required to control these aggressive tumors. In the last decade, knowledge about the pathophysiological mechanisms of various pituitary adenomas has increased, thus novel medical therapies that target specific pathways implicated in tumor synthesis and hormonal over secretion are now available. Advancement in patient selection and determination of prognostic factors has also helped to individualize therapy for patients with pituitary tumors. Improvements in biochemical and “tumor mass” disease control can positively affect patient quality of life, comorbidities and overall survival. In this review, the medical armamentarium for treating CD, acromegaly, prolactinomas, NFA, and carcinomas/aggressive atypical adenomas will be presented. Pharmacological therapies, including doses, mode of administration, efficacy, adverse effects, and use in special circumstances are provided. Medical therapies currently under clinical investigation are also briefly discussed.
Acromegaly ; Adenoma ; Comorbidity ; Drug Therapy ; Humans ; Patient Selection ; Pituitary ACTH Hypersecretion ; Pituitary Neoplasms* ; Prolactinoma ; Quality of Life

The magnetic resonance images (MRI) of 30 patients with surgically or biochemically confirmed pituitary adenomas (20 macroadenomas, 10 microadenomas) were retrospectively evaluated. Ten patients had hyperprolactinemia, another eight had acromegaly, another eight had nonfunctioning adenoma and four had Cashing disease. The examination were performed at a 1.5-T superconducting MR system using a multisection spin-echo technique with 3mm-thick sections and a 256×224 matrix. TI weighted sagittal and coronal images were obtained before and within 30 minutes after the administration of Gd-DTPA (0.1mmol/kg). Analysis of the MRI was focused on the signal intensity and enhamcement patterns of the pituitary adenoma before and after Gd-DTPA administration. Compared with endocrinologic diagnosis, macroadenoma showed heterogeneous enhacement in 55%, rim enhancement in 35% and homogeneous enhancement in 10%, Conclusively, the enhancement patterns of the pituitary adenoma did not correlate with the subtypes made according to hormone production.
Acromegaly ; Adenoma ; Diagnosis ; Gadolinium DTPA ; Humans ; Hyperprolactinemia ; Magnetic Resonance Imaging* ; Pituitary Neoplasms* ; Retrospective Studies

A macroinvasive pituitary adenoma with plurihormonality usually causes acromegaly and hyperprolactinemia, and also accompanies with neurologic symptoms such as visual disturbances. However, its concurrent presentation with a rectal carcinoid tumor is rarely observed. This study reports the history, biochemical, colonoscopic and immunohistochemical results of a 48-year-old female with acromegaly and hyperprolactinemia. Despite the large size and invasive nature of the pituitary adenoma to adjacent anatomical structures, she did not complain of any neurologic symptoms such as visual disturbance or headache. Immunohistochemical staining of the surgical specimen from the pituitary adenoma revealed that the tumor cells were positive for growth hormone (GH), prolactin (PRL), and thyroid stimulating hormone (TSH). Staining for pituitary-specific transcription factor-1 (Pit-1) was shown to be strongly positive, which could have been possibly contributing to the plurihormonality of this adenoma. Colonoscopy found a rectal polyp that was identified to be a carcinoid tumor using immunohistochemical staining. A macroinvasive pituitary adenoma with concomitant rectal carcinoid tumor was secreting GH, PRL, and TSH, which were believed to be in association with over-expression of Pit-1. This is the first case report of double primary tumors comprising a plurihormonal pituitary macroadenoma and rectal carcinoid tumor.
Acromegaly* ; Adenoma ; Carcinoid Tumor* ; Colonoscopy ; Female ; Growth Hormone ; Headache ; Humans ; Hyperprolactinemia ; Middle Aged ; Neurologic Manifestations ; Pituitary Neoplasms* ; Polyps ; Prolactin ; Thyrotropin

Empty sella is defined as a sella which, regardless of its size, is completely or partly filled with cerebrospinal fluid (CSF), and is divided into two groups, primary or secondary. Secondary empty sella syndrome is associated with a prior surgery, radiation therapy or medical treatment of pituitary gland, and otherwise primary. The endocrine function of primary empty sella syndrome is usually normal, but sometimes, this syndrome is associated with complete or partial pituitary insufficiency and rarely pituitary hypersecretion. In the cases of hypersecretion of pituitary hormones, hyperprolactinemia, acromegaly and hypersecretion of adrenocorticotrophin (ACTH) has been reported. but, hypersecretion of ACTH is rarely reported. We describe a patient of primary empty sella syndrome combined with Cushing's disease. So we present this case with a review of the literature.
Acromegaly ; Adrenocorticotropic Hormone ; Cerebrospinal Fluid ; Cushing Syndrome ; Empty Sella Syndrome* ; Humans ; Hyperprolactinemia ; Hypopituitarism ; Pituitary Gland ; Pituitary Hormones

ABSTRACT Acromegaly is a devastating chronic slowly progressive disease. Its early diagnosis is a challenging issue that necessitates clinical suspicion of signs and symptoms as a first step. This report introduces an unusual early sign in the oral cavity that lead to the early diagnosis of an acromegaly case. A case of a healthy 40-year-old male patient presented with progressively growing multiple hard swellings in the upper and lower jaws. Clinical examination revealed bony hard multiple small spiky exostosis-like swellings, located at the maxillary and mandibular alveolar bones. An array of investigations revealed a 2-mm diameter pituitary tumour in MRI of sella. To the best of the author’s knowledge, this is the first report of spiky exostosis-like growths in the alveolar bone as an early sign of acromegaly. In this case, thorough examination of oral signs and symptoms was the first step for early diagnosis and hence, better prognosis for acromegaly.
Acromegaly--diagnosis

No abstract available.
Hyperprolactinemia*

Hyperprolactinemia is a well-known neuroendocrine side effect to antipsychotic agent. Combined treatment of aripiprazole is recognized as an effective solution against hyperprolactinemia caused by antipsychotic agent. We report 2 progressive clinical cases where both are treated with combined use of aripiprazole which has a unique mechanism of action to resolve olanzapine and amisulpride-induced hyperprolactinemia.
Hyperprolactinemia*

Background: To investigate factors influencing disconnection hyperprolactinemia, including tumour volume, degree of pituitary stalk displacement and extent of tumour growth based on a modified Wilson-Hardy classification in a non-functioning pituitary macroadenoma and to confirm reductions in serum prolactin levels after endoscopic transphenoidal surgery. Methods: This prospective, descriptive study was conducted in the Department of Neurosurgery, General Hospital Kuala Lumpur from Jan 1, 2011 to Jan 1, 2013. Forty patients fulfilling the inclusion criteria were enrolled. All patients underwent endoscopic transphenoidal resection of non-functioning pituitary macroadenoma. Pituitary stalk angle, tumour volume and extent of tumour growth were measured from Magnetic Resonance Imaging (MRI) pre- and post-operatively. These variables were compared to serum prolactin levels measured pre and post operatively. SPSS 21 was used to perform statistical analyses. Results: In 40 patients, the mean tumour volumes were 10.58 cm3 (SD 7.81) pre-operatively and 3.1 cm3 (SD 3.45) post-operatively. There was a 70% reduction in tumour volume post-operatively (P < 0.01). The mean serum prolactin was 457 mIU/L (SD 66.93) pre-operatively and 297 mIU/L (SD 6.73) post-operatively. There was a 65% reduction in prolactin serum levels after surgery (P < 0.01). The mean pituitary stalk angles were 93.45 ± 3.89 degrees pre-operatively and 51.45 ± 1.46 degrees post-operatively (P = 0.01). The mean pituitary stalk angle in the control group was 50.4 ± 8.80 degrees. Hence, there was a 98% reduction in pituitary stalk angle after surgery (P < 0.01). This study showed a linear correlation between the pre-operative and post-operative tumour volumes and serum prolactin levels (P = 0.01 pre-and post-operative) and between serum prolactin levels and pituitary stalk angle (P = 0.20 pre-operative; P = 0.01 post-operative). Conclusion: Tumour volume and pituitary stalk angle displacement have positive predictive values for disconnection hyperprolactinemia in non-functioning pituitary macroadenoma. However, a larger sample size and further objective studies are needed to confirm these findings.
Hyperprolactinemia