An unusual case of cushing's syndrome, hilus cell tumor and polycystic ovaries

An unusual case of Cushing's syndrome in a 10-yr-old girl is presented. The patient originally exhibited features of Cushing's syndrome and acanthosis nigricans, but still maintained dexamethasone suppressibility. Following adrenalectomy, virilization became more severe, leading to the diagnosis of polycystic ovary syndrome and hilus cell tumor of the right ovary. After bilateral wedge resection and removal of the ovarian tumor, virilism decreased only slightly. Pigmentation changes became more severe and extremely high levels of ACTH with incomplete suppression after hydrocortisone were noted. At this time, prolactin levels were elevated. A primary hypothalamic lesion is suggested in this patient, beginning as an unusual form of Cushing's syndrome with Nelson's syndrome following adrenalectomy. The process progressed over a 6-yr period to involve the LHovarian axis with resultant virilism, polycystic ovaries and hilus cell tumor. Prolactin hypersecretion and acanthosis nigricans probably also reflect the hypothalamic disease process.