Small cell carcinoma of the vagina causing Cushing’s syndrome by ectopic production and secretion of ACTH

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Small cell carcinoma of the vagina causing Cushing’s syndrome by ectopic production and secretion of ACTH: a case report.

K M Colleran, M R Burge, L A Crooks, R I Dorin

Department of Medicine, Albuquerque VA Medical Center and University of New Mexico School of Medicine, 87108, USA.

BACKGROUND: Small cell carcinomas of pulmonary or extrapulmonary origin are neuroendocrine tumors classically associated with ectopic hormone production, particularly ACTH secretion resulting in Cushing’s syndrome. However, ectopic Cushing’s syndrome has not previously been reported in the setting of small cell carcinoma of the vagina.

METHODS: A primary vaginal tumor with hepatic metastases was evaluated with light microscopy. Serum cortisol and plasma ACTH levels were evaluated by radioimmunoassay and immunoradiometric assay, respectively, during a standard high-dose (8 mg) overnight dexamethasone suppression test.

RESULTS: Vaginal small cell carcinoma with hepatic metastases was demonstrated. Electrolyte abnormalities, elevated cortisol and ACTH levels, and failure to suppress ACTH secretion during high-dose dexamethasone administration confirmed the diagnosis of ectopic ACTH syndrome.

CONCLUSIONS: This case report establishes a clinical association between vaginal small cell carcinoma and ectopic Cushing’s syndrome, confirming the neuroendocrine potential of this malignancy and features common to small cell neoplasms originating in other sites.

More information and citations here: http://lib.bioinfo.pl/pmid:9190988

 

Medical treatment of Cushing’s disease: Overview and recent findings

Published Date October 2009

Journal: International Journal of General Medicine

Stephanie Smooke Praw1, Anthony P Heaney1,2

1Department of Medicine, 2Department of Neurosurgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA

Abstract: Cushing’s disease, due to pituitary adrenocorticotropic hormone (ACTH) hypersecretion, is the most common etiology of spontaneous excess cortisol production. The majority of pituitary tumors causing Cushing’s disease measure <1 cm and the excess morbidity associated with these tumors is mostly due to the effects of elevated, nonsuppressible, ACTH levels leading to adrenal steroid hypersecretion. Elevated circulating cortisol levels lead to abnormal fat deposition, hypertension, diabetes, coronary artery disease, osteoporosis, muscle weakness and psychological disturbances. At experienced centers, initial surgical remission rate via transnasal, transphenoidal resection approaches 80% for tumors less than 1 cm, but may be as low as 30% for larger lesions and long-term recurrence in all groups approaches 25%. Residual disease may be managed with more radical surgery, pituitary-directed radiation, bilateral adrenalectomy, or medical therapy. This paper addresses current and novel therapies in various stages of development for Cushing’s disease.

Keywords: Cushing’s disease, treatment, pasireotide, PPAR-γ, 11 β-hydroxysteroid dehydrogenase inhibitors, dopamine agonists

Download article (Free!): http://dovepress.com/getfile.php?fileID=5388