Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia can cause severe testicular damage.

摘要

OBJECTIVE: To evaluate the histological features of testicular tumors and residual testicular parenchyma in male patients with congenital adrenal hyperplasia (CAH) and longstanding bilateral testicular adrenal rest tumors (TART). DESIGN: Descriptive study. SETTING: University medical center in the Netherlands. PATIENT(S): Seven male patients who had CAH with longstanding bilateral TART and who were treated with testis-sparing surgery. INTERVENTION(S): Enucleation of TART and taking biopsies of the surrounding testicular parenchyma. MAIN OUTCOME MEASURE(S): Description of the histological features of TART and residual testicular parenchyma. RESULT(S): All tumors had a similar histological appearance, with sheets of polygonal cells separated by dense fibrous tissue with focal lymphocytic infiltrates and without Reinke crystals. All biopsies showed a decrease in tubular diameter with peritubular fibrosis and, in four patients, tubular hyalinization. The germinative layer showed decreased spermatogenesis and reduced Johnsen scores. CONCLUSION(S): Testicular adrenal rest tumors can lead to end-stage damage of testicular parenchyma, most probably as a result of longstanding obstruction of the seminiferous tubules. Therefore, treatment at an early stage is advised.