Ectopic ACTH-syndrome due to a thymic carcinoid tumor. 2002

S Van Schaeybroeck, and S Van Imschoot, and P Cochez
Department of Endocrinology, AZ Sint Jan, Ruddershove 10, 8000 Bruges. Sandra.Vanschaeybroeck@uz.kuleuven.ac.be

Investigating a recently developed Cushing Syndrome, we diagnosed in a 47-year-old woman an ectopic ACTH syndrome due to a metastatic carcinoid tumor, most likely a thymic carcinoid tumor. Combined therapy with sandostatin and nizoral and later on with sandostatin, metopirone and orimeten, was not able to suppress the hypercortisolism. A few weeks after surgical adrenalectomy, clinical deterioration ensued, culminating in the patient's death 7 months after diagnosis.

UI MeSH Term Description Entries
D007654 Ketoconazole Broad spectrum antifungal agent used for long periods at high doses, especially in immunosuppressed patients. Nizoral,R-41400,R41,400,R41400,R 41400
D008797 Metyrapone An inhibitor of the enzyme STEROID 11-BETA-MONOOXYGENASE. It is used as a test of the feedback hypothalamic-pituitary mechanism in the diagnosis of CUSHING SYNDROME. Methbipyranone,Methopyrapone,Metopiron,Metopirone,Métopirone,SU 4885
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D002276 Carcinoid Tumor A usually small, slow-growing neoplasm composed of islands of rounded, oxyphilic, or spindle-shaped cells of medium size, with moderately small vesicular nuclei, and covered by intact mucosa with a yellow cut surface. The tumor can occur anywhere in the gastrointestinal tract (and in the lungs and other sites); approximately 90% arise in the appendix. It is now established that these tumors are of neuroendocrine origin and derive from a primitive stem cell. (From Stedman, 25th ed & Holland et al., Cancer Medicine, 3d ed, p1182) Argentaffinoma,Carcinoid,Carcinoid, Goblet Cell,Argentaffinomas,Carcinoid Tumors,Carcinoids,Carcinoids, Goblet Cell,Goblet Cell Carcinoid,Goblet Cell Carcinoids,Tumor, Carcinoid,Tumors, Carcinoid
D004791 Enzyme Inhibitors Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction. Enzyme Inhibitor,Inhibitor, Enzyme,Inhibitors, Enzyme
D005260 Female Females
D006728 Hormones Chemical substances having a specific regulatory effect on the activity of a certain organ or organs. The term was originally applied to substances secreted by various ENDOCRINE GLANDS and transported in the bloodstream to the target organs. It is sometimes extended to include those substances that are not produced by the endocrine glands but that have similar effects. Hormone,Hormone Receptor Agonists,Agonists, Hormone Receptor,Receptor Agonists, Hormone
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000182 ACTH Syndrome, Ectopic Symptom complex due to ACTH production by non-pituitary neoplasms. Ectopic ACTH Syndrome,ACTH Syndromes, Ectopic,Ectopic ACTH Syndromes,Syndrome, Ectopic ACTH,Syndromes, Ectopic ACTH
D000616 Aminoglutethimide An aromatase inhibitor that is used in the treatment of advanced BREAST CANCER. Cytadren,Orimeten

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