EXCESSIVE TESTOSTERONE PRODUCTION IN A PATIENT WITH NELSON SYNDROME AND BILATERAL TESTICULAR-TUMORS

Bilateral primary testicular tumors are rare and usually consist of ei ther interstitial cells or hypertrophic testicular adrenal remnant tis sue. Their differentiation on clinical presentation and histologic exa mination remains difficult but is essential because of the different t herapeutic approaches, We report a rare case of excessive testosterone production by bilateral testicular tumors in a patient with Nelson sy ndrome (ACTH-secreting pituitary adenoma after bilateral adrenalectomy in patients with Gushing's disease). Increased ACTH stimulation in th is patient supports the thesis of pluripotent cells within the testis which can undergo differentiation to cells which are not only morpholo gically similar to Leydig cells but also have the functional property of these cells, Our clinical findings support the diagnosis of hyperpl asia of adrenal remnant or pluripotent cells rather than a true Leydig cell tumor. We emphasize the need for hormonal evaluations which shou ld be assessed in the context of the size of these nodular tumors prio r to therapeutic decisions, In cases with elevated serum ACTH and smal l nodular hyperplasia, we would favor a 'wait-and-see' strategy with a ppropriate hormonal therapy. In large tumors with clinical signs of ho rmonal activity, patient noncompliance with steroid replacement regime ns or with local symptoms, scrotal exploration and tumor enucleation a re indicated.