Sperm retrieval and concomitant tumor resection in azoospermic men with congenital adrenal hyperplasia and bilateral testicular adrenal rest tumors: a case report

Abstract

PurposeThe objective of this study was to offer a new treatment approach for sperm retrieval simultaneously with tumor resection in azoospermic men with congenital adrenal hyperplasia (CAH), orchialgia, and bilateral testicular adrenal rest tumors (TARTs) who fail to respond to medical treatment.MethodsThis is a retrospective chart review from a couple’s fertility center.ResultsBetween May 2013 and May 2015, two azoospermic men with CAH and bilateral TARTs, with orchialgia, and desire to conceive underwent bilateral TART resection in the same surgical setting as sperm retrieval after remaining azoospermic with normalization of gonadotropins with treatment with human chorionic gonadotropin (hCG). Both men had adequate sperm retrieved for in vitro fertilization/intracytoplasmic sperm retrieval (IVF/ICSI) at the time of bilateral TART resections. They had complete TART resections with resolution of orchialgia. The wife of one patient had a successful pregnancy with use of retrieved sperm resulting in a live birth, and the sperm from the other man is cryopreserved for future use.ConclusionsIt is feasible to perform successful sperm retrieval simultaneously with TART resection in azoospermic men with CAH after medical treatments with persistent azoospermia, rather than subjecting these men to multiple invasive procedures.

Two cases of reversible male infertility due to congenital adrenal hyperplasia combined with testicular adrenal rest tumor

“…The other man had his sperm cryopreserved for future use. Both men had a complete resolution of orchialgia from the TART …”

“…It is believed that remnants of the adrenogonadal primordium can persist and proliferate to TARTs, with the preservation of adrenal‐like, hormone‐producing properties . The reported prevalence of TARTs in patients with CAH ranges from 27% to 47% …”

“…In the case of no possible reason for infertility, except for seminiferous tubal obstruction, an operation for the removal of the TART is a reasonable option . Corticosteroid therapy itself can induce HPG dysfunction, especially if patients are overdosed . When all the treatments fail to recover spermatogenesis, testicular sperm retrieval for IVF/intracytoplasmic sperm injection (ICSI) may be proposed for the goal of conception.…”

“…Congenital adrenal hyperplasia (CAH) is one of the most commonly found autosomal recessive diseases (~1:16 000 births) . The defective conversion of 17‐hydroxyprogesterone (17‐OHP) to 11‐deoxycortisol, which is mediated by 21‐hydroxylase, accounts for >90% of the cases of CAH, (Figure ), resulting in increased adrenocorticotropic hormone (ACTH) production and an associated hyperplasia of the adrenal glands, with the subsequent overproduction of adrenal androgens .…”

MANAGEMENT OF ENDOCRINE DISEASE: Gonadal dysfunction in congenital adrenal hyperplasia

“…This was confirmed by testis biopsies. Furthermore, surgery may contribute to irreversible damage of the testes due to scarring of the testicular tissue (52,57). Therefore, patients should be carefully informed by a multidisciplinary team about the consequences of large TART, and cryopreservation of sperm should be considered.…”