Activation of virilizing adrenal rest tissues by excessive ACTH production: An unusual presentation of Nelson's syndrome

Abstract

Nelson's syndrome occurs in 10 to 35 percent of patients treated for Cushing's disease by bilateral adrenalectomy and features an ACTH-producing pituitary tumor and hyperpigmentation. Cortisol-producing testicular tumors activated by markedly elevated ACTH levels have been described in male patients with Nelson's syndrome. We now describe a female patient with Nelson's syndrome who presented with virllization. Abdominal exploration revealed adrenal rest tumors in the paraovarian tissues and adrenal beds. Iodocholesterol scanning gave negative results. This case illustrates the need for follow-up study of patients with bilateral adrenalectomy for Cushing's disease and describes some of the unusual features these patients may present.