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

ObjectiveTo 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).DesignDescriptive study.SettingUniversity 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.