Bilateral Synchronous Testicular Mass from Testicular Adrenal Rest Tumors, in a Patient with Congenital Adrenal Hyperplasia, or Testicular Leydig Cell Tumors? Dilemma for Bilateral Orchiectomy or Not

Abstract

Congenital adrenal hyperplasia (CAH) refers to a group of autonomic disorders due to enzyme deficiency for the biosynthesis of steroid hormones. These disorders entail an increase in ACTH levels and as followed by adrenal hyperplasia. CAH is categorized into two types, classic and non-classic. In the common type we have a deficiency of 21-hydroxylase observed in a prevalence of 1 per 5000 per 145,000 births. We have insufficient aldosterone and cortisol production and as a result, elevated plasma ACTH levels, with subsequent disorders that this entails, depending on the level of deficiency. Testicular adrenal rest tumor (TART) develops from isletsectopic adrenal tissue within the gonads, directly affected by ACTH overproductions a complication of CAH, with a prevalence ranging from 27% to 47%. These are benign tumors that are recognized as palpable masses. A biopsy of these tumors is recommended as well as their surgical removal, so as to rule out malignancy. In this case report we present a case of bilateral synchronous TART tumor in association with medullolipoma in a patient with CAH. The dilemma arises regarding the decision of bilateral orchiectomy.