Lipid cell tumor of the ovary in reference to adult-onset congenital adrenal hyperplasia and polycystic ovary syndrome. A case report.
A 30-year-old woman with a virilizing lipid tumor was initially suspected of having adult-onset congenital adrenal hyperplasia (CAH) when her plasma 17-hydroxyprogesterone (17OHP) concentration was found to be very high (298-3,170 ng/dL), to drop in response to an overnight dexamethasone (dex) suppression test (79 ng/dL) and to rise briskly 15 minutes after ACTH administration (751 ng/dL). However, the effect of dex was not sustained or complete: the pregnanetriol excretion dropped only from 5.5 to 4.4 mg daily. Furthermore, the plasma testosterone was inappropriately high (235-537 ng/dL) for adult-onset CAH and was more responsive to endogenous and exogenous gonadotropin stimulation than to ACTH. In addition, there was no evidence of 11 beta-hydroxylation of 21-deoxycorticoids, as would be expected in CAH. Removal of the tumor completely reversed the virilization and the abnormal responses to ACTH and human chorionic gonadotropin. The contralateral ovary bore lipid-laden stromal cells in the deep paracortex that bore a striking resemblance to the tumor cells, and a cyst in that ovary had fluid with a steroid pattern virtually identical to that of the tumor, with an androstenedione: 17OHP ratio of 5:1. There was not evidence of polycystic ovary disease. The clinical picture of type II polycystic ovary syndrome (PCOS) gradually evolved over a one-year period postoperatively: plasma-free testosterone became mildly elevated and was not dex suppressible. The 17OHP response to ACTH became slightly excessive. However, there was no evidence of tumor on computed tomography.(ABSTRACT TRUNCATED AT 250 WORDS)
D019326:17-alpha-Hydroxyprogesterone; D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis*; D000314:Adrenal Rest Tumor / Q000150:complications* / Q000473:pathology; D000328:Adult; D005260:Female; D006801:Humans; D006908:Hydroxyprogesterones / Q000097:blood; D010051:Ovarian Neoplasms / Q000150:complications* / Q000473:pathology; D010053:Ovary / Q000473:pathology; D011085:Polycystic Ovary Syndrome / Q000209:etiology*
D006908:Hydroxyprogesterones; D019326:17-alpha-Hydroxyprogesterone