Abstract. The McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies including growth hormone (GH) excess. Polyostotic bone lesions and café-au-lait macules are common while monostotic bone lesions are rare. Similarly, acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon and in most of the instances somatotropinoma has not been documented. We report 3 patients, two of them had monostotic lesion, none had café-au-lait macules and all had GH secreting pituitary macroadenoma. All of them underwent transfrontal pituitary adenomectomy and had histopathological confirmation of GH secreting pituitary adenoma. A brief review of literature is also presented.

Endocrine causes of paraparesis are few and include thyrotoxic periodic paralysis, adrenomyeloneuropathy, acromegaly, primary hyperparathyroidism, and Paget disease. We report a middle-aged man who presented with recurrent paraparesis resulting from polyostotic Paget disease of the spine and well responded to alendronate therapy. (The Endocrinologist 2006;16: 64 -65) P aget disease of bone is a disorder of disorganized bone remodeling characterized by a marked increase in osteoclastic bone resorption followed by the replacement of normal marrow by vascular, fibrous, and connective tissue. 1 It is a disease of unknown etiology that affects approximately 3% of the European population but is very unusual in the Indian subcontinent. Most (90%) cases occur over the age of 40 years, and the disease affects men more commonly than women (male: female ratio 1.5:1). 2 The common clinical manifestations of disease include bone and joint pains, deformity, fracture, spinal stenosis, sensorineural deafness, and congestive heart failure. However, recurrent paraparesis as a presenting feature of Paget disease is rare and has not previously reported. We describe a middle-aged man who presented with recurrent paraparesis resulting from Paget disease of the spine and who responded well to alendronate therapy. CASE REPORTA 43-year-old man presented with backache for 1.5 years followed by weakness, stiffness, and numbness of both the lower limbs over the past year. Family members noticed that his hearing ability deteriorated over the last 6 months. He had no history of fever, cough, weight loss, trauma, or spontaneous fractures, or any bladder or bowel incontinence.His height was 170 cm and weight 83 kg; blood pressure 130/80 mm Hg. The neurologic examination revealed bilateral conductive deafness, paraparesis with hypertonia, and exaggerated deep tendon reflexes in both lower limbs. Pain, fine touch, vibration, and joint position senses were impaired below the D 3 spinal level.The complete blood count, electrolytes, and renal function tests were normal. The corrected mean serum calcium was 9.5 mg/dL (normal, 8.5-10.5 mg/dL), phosphate 4.1 mg/dL (normal, 3.5-5 mg/dL), and alkaline phosphatase was 44 KA units (normal, 3-13 KA units). Audiometric findings were consistent with conductive deafness. X-ray of the spine showed compression of the D 3 vertebrae. Magnetic resonance imaging of the spine with T1 and T2-weighted images showed a crush fracture of the D 3 vertebrae and compression of the spinal cord at D 3 level (Fig. 1). For this, he underwent T 3 discoidectomy. Two months postoperatively, the patient improved and resumed his duties. The histopathology revealed a mosaic pattern of bone with disorganization and formation of cement lines consistent with Paget disease (Fig. 2). Subsequently, he was lost to follow up and 4 years later again presented with paraparesis. Alkaline phosphatase was 64 KAU (normal, 3-13 KAU) with a normal calcium profile. Magnetic resonance imaging of the spine was normal except for ferromagnetic artifac...

Ectopic Cushing is an uncommon disorder and comprises only 10% to 20% of patients with Cushing syndrome. Bronchial carcinoid is the most common cause of ectopic Cushing syndrome. The liver is a common site for carcinoid metastasis, but primary hepatic carcinoid presenting with advanced manifestation of Cushing syndrome has not been reported previously. Extensive search and vigorous follow up for 3 years failed to reveal primary elsewhere.E ndogenous Cushing syndrome is an uncommon disorder and the majority (80%) of patients have pituitary microadenoma. 1 Ectopic adrenocorticotropin (ACTH) and/or corticotropin-releasing hormone (CRH) producing tumors contribute only 10% to 20% of patients with Cushing syndrome. The remainder are adrenal neoplasms. Ectopic Cushing syndrome is caused by small cell carcinoma of the lung, carcinoid tumors of the thymus and bronchus, and sometimes gut carcinoids. 2,3 In rare instances, ectopic secretion of ACTH has been documented from the adrenal medulla, ovary, islet cell tumors, and medullary thyroid carcinoma. 4 The liver is a common site for carcinoid metastasis, but primary hepatic carcinoid (PHC) is a rare entity and fewer than 60 cases have been reported in the English literature. 5 Primary hepatic carcinoid usually presents with abdominal pain and hepatomegaly and rarely manifests with an endocrine syndrome. We describe a patient with Cushing syndrome resulting from ectopic secretion of ACTH from a primary hepatic carcinoid. This association has not been reported previously. CASE REPORTA 33-year-old woman presented with chronic diarrhea for 6 months for which no cause could be found. She was not a known diabetic, did not have hypertension, and there was no history of steroid therapy. She reported weight gain, striae over the abdomen, and swelling of the feet for 6 months. Her last child was born 6 years earlier and she was amenorrheic for 2 years. Her height was 163 cm, weight 63 kg, and body mass index (BMI) was 23.7 kg/m 2 . She had a plethoric face, broad purple striae over her abdomen, proximal muscle weakness, and bruises over venipuncture sites. Her blood pressure was 140/90 mm Hg and she had knuckle pigmentation. She had grade II acne and few terminal hairs in the sideburn areas. She had smooth, nontender hepatomegaly and extensive esophageal and vaginal candidiasis.Serum Na ϩ was 139 meq/L, K ϩ 4.2 meq/L, FPG 105 mg/dL, and 2-hour postprandial glucose was 220 mg/dL. Liver and renal function tests were normal. Levels of serum cortisol at 8 AM was 1200 nmol/L (normal, 400 -600 nmol/L), 8 PM 1200 nmol/L with paired ACTH 191 pg/mL (normal, Ͻ35 pg/mL), and dehydroepiandrosterone sulfate (DHEAS) was 530 g/dL (normal, 45-270 g/dL). Serum cortisol after low-dose dexamethasone (0.5 mg every 6 hours for 2 days) was 900 nmol/L and after high-dose dexamethasone suppression (2 mg every 6 hours for 2 days) was 1100 nmol/L. Contrast-enhanced computed tomography (CECT) revealed bilateral adrenal hyperplasia ( Fig. 1) with multiple heterogenous nodules with central necrosis in the live...