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).


METHODS
Descriptive study.


METHODS
University medical center in the Netherlands.


METHODS
Seven male patients who had CAH with longstanding bilateral TART and who were treated with testis-sparing surgery.


METHODS
Enucleation of TART and taking biopsies of the surrounding testicular parenchyma.


METHODS
Description of the histological features of TART and residual testicular parenchyma.


RESULTS
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.


CONCLUSIONS
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.
Claahsen-van der Grinten
Hedi L
HL
Department of Paediatric Endocrinology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands. h.claahsen@cukz.umcn.nl
Otten
Barto J
BJ
Hermus
Ad R M M
AR
Sweep
Fred C G J
FC
Hulsbergen-van de Kaa
Christina A
CA
D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D000314:Adrenal Rest Tumor / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D000328:Adult; D001706:Biopsy; D005355:Fibrosis; D006801:Humans; D007248:Infertility, Male / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology / Q000517:prevention & control; D008297:Male; D012671:Seminiferous Tubules / Q000473:pathology; D012720:Severity of Illness Index; D013091:Spermatogenesis; D013736:Testicular Neoplasms / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D013737:Testis / Q000473:pathology* / Q000503:physiopathology / Q000601:surgery