Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0007095 (carcinoid)
 6,990 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 31-year-old woman presented with progressive weight gain, facial acne, round facies, hirsutism, and secondary amenorrhea. Her plasma cortisol, urinary free cortisol, and plasma ACTH were elevated. CT scan of abdomen revealed bilateral diffuse adrenal enlargement. MRI of pituitary failed to identify a lesion. CT scan of chest revealed an 8 mm nodule in the lower lobe of the left lung. The diagnosis of ectopic Cushing's syndrome was made. The lung tumor was surgically removed. The tumor measured up to 1.5 cm in diameter. By light microscopy, a tumor with characteristic features of bronchial carcinoid was noted. Immunostains were positive for neuron-specific enolase, synaptophysin, chromogranin, low-molecular-weight keratin, ACTH, beta endorphin, corticotropin-releasing hormone, bombesin, alpha subunit, and somatostatin. Electron microscopy revealed an endocrine neoplasm. The secretory granules displayed great variation subdividing the endocrine neoplasm. The secretory granules displayed great variation subdividing the cell population into an undetermined number of phenotypes. In situ hybridization demonstrated positivity for pro-opiomelanocortin mRNA in the tumor cells. Postoperatively, plasma cortisol had fallen. The patient remained symptom-free one year later. The case presented here was regarded as a plurihormonal bronchial carcinoid associated with ectopic Cushing's syndrome. This unique plurihormonal bronchial carcinoid tumor produced three hormones, ACTH, CRH, and bombesin, that may have contributed to the patient's ectopic Cushing's syndrome.
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PMID:Plurihormonal Bronchial Carcinoid Associated with Ectopic Cushing's Syndrome. 1211 73

Ectopic ACTH secretion accounts for less that 10% of all causes of endogenous Cushing's syndrome. Carcinoids are rare thymic tumors, and when associated with ACTH hypersecretion display local or distant aggressive behavior. A 32-year-old woman was admitted to the Endocrinology Unit for obesity, moon face, facial hirsutism, hyperpigmentation, and secondary amenorrhea. Laboratory test confirmed the hypercortisolism and excess ACTH, while dexamethasone suppressive test was negative. Thorax computed tomography (CT) showed an antero-superior mediastinal tumor invading the pericardium and left mediastinal pleura. A complete resection through median sternotomy of the tumor, pericardium and left mediastinal pleura was performed. After a one-year symptom-free period, hypercortisolism recurred, confirmed by laboratory findings. Although no signs of local recurrence were seen on thorax CT, left internal mammary lymph nodes involvement and vertebral body metastases at C7 and LI were found. Refractory electrolyte disturbances could not be corrected resulting in severe cardiac arrhythmia and death from cardiac arrest. The reported case draws attention on the aggressiveness of ACTH-secralso due to the refractory electrolyte disturbances with fatal outcome.
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PMID:Ectopic adrenocorticotropic hormone syndrome caused by a carcinoid tumor of the thymus. A case report. 2187 Jul 29