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

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.