Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma.
A patient is described with Cushingoid features and a tumor of the paranasal sinuses. Laboratory studies revealed hyperglycemia, hypokalemia, alkalosis, hypercortisolemia, and elevated serum ACTH levels. The cortisol level increased briskly in response to parenteral ACTH, but was only partially suppressed by dexamethasone. Microscopically, the tumor was found to be a paraganglioma containing many neurosecretory granules.. Treatment with methyrapone, o,p'DDD, and irradiation of the tumor resulted in a lasting remission. It is concluded that this unique patient suffered from Cushing's syndrome due to ectopic ACTH production by a paraganglioma of the paranasal sinuses.
D000182:ACTH Syndrome, Ectopic / Q000188:drug therapy / Q000209:etiology* / Q000532:radiotherapy; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D003401:Creatine / Q000097:blood / Q000652:urine; D003480:Cushing Syndrome / Q000188:drug therapy / Q000209:etiology* / Q000532:radiotherapy; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood / Q000652:urine; D008797:Metyrapone / Q000627:therapeutic use; D008875:Middle Aged; D010235:Paraganglioma / Q000188:drug therapy / Q000378:metabolism* / Q000532:radiotherapy; D010255:Paranasal Sinus Neoplasms / Q000188:drug therapy / Q000378:metabolism* / Q000532:radiotherapy; D009384:Paraneoplastic Endocrine Syndromes / Q000209:etiology*
D000324:Adrenocorticotropic Hormone; D003401:Creatine; D006854:Hydrocortisone; D008797:Metyrapone