![]() Because the primary tumor could not be identified by imaging, biopsy of the presumed metastatic liver lesions was performed. Serum markers of carcinoid, medullary thyroid carcinoma, and pheochromocytoma were in the normal range. Laboratory testing was positive for elevated serum carcinoembryonic antigen and the neuroendocrine marker chromogranin A. CT imaging did not reveal any discrete lung lesions but there were mediastinal and abdominal lymphadenopathy and multiple liver lesions suspicious for metastatic disease. Pituitary imaging was normal and cortisol did not suppress with a high dose dexamethasone test, consistent with a diagnosis of ectopic ACTH. ResultsĪ 56 year old woman presented with clinical and laboratory features consistent with ACTH-dependent CS. We review the literature of the types of tumors associated with this unusual syndrome and the behavior of these tumors by endocrine testing. We describe the clinical presentation, course, laboratory values and pathologic findings of a patient with isolated ectopic CRH causing CS. Cushing's Syndrome (CS) which is caused by isolated Corticotropin-releasing hormone (CRH) production, rather than adrenocorticotropin (ACTH) production, is extremely rare.
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