M. Shekarriz et al., EXCESSIVE TESTOSTERONE PRODUCTION IN A PATIENT WITH NELSON SYNDROME AND BILATERAL TESTICULAR-TUMORS, Urologia internationalis, 56(3), 1996, pp. 200-203
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.