Abstract
A 33-year-old male with a history of left testis Leydig cell tumor (LCT), 3-month status after left radical orchiectomy, presented with a rapidly enlarging (0.6 cm to 3.7 cm) right testicular mass. He underwent a right radical orchiectomy, sections interpreted as showing a similar Leydig cell-like oncocytic proliferation, with a differential diagnosis including metachronous bilateral LCT and metachronous bilateral testicular tumors associated with congenital adrenal hyperplasia (a.k.a. “testicular adrenal rest tumors” (TARTs) and “testicular tumors of the adrenogenital syndrome” (TTAGS)). Additional workup demonstrated a markedly elevated serum adrenocorticotropic hormone (ACTH) and elevated adrenal precursor steroid levels. He was diagnosed with congenital adrenal hyperplasia, 3β-hydroxysteroid dehydrogenase deficiency (3BHSD) type, and started on treatment. Metachronous bilateral testicular masses in adults should prompt consideration of adult presentation of CAH. Since all untreated CAH patients are expected to have elevated serum ACTH, formal exclusion of CAH prior to surgical resection of a testicular Leydig-like proliferation could be accomplished by screening for elevated serum ACTH.
Highlights
Testicular tumors in children and adults may be due to neoplasms or reactive hyperplasias
This paper considers the differential diagnosis of metachronous bilateral Leydig-like testicular tumors in an adult
Because testicular adrenal rest tumors (TART) frequently mimic Leydig cells cytologically, a large unifocal unilateral TART that displaces testicular parenchyma could be confused with an Leydig cell tumor (LCT) in an adult in whom Congenital adrenal hyperplasia (CAH) is unrecognized. This diagnostic puzzle is less likely in a child, because precocious puberty would usually prompt an endocrine workup that would distinguish LCT from TART
Summary
Testicular tumors in children and adults may be due to neoplasms or reactive hyperplasias. Because TARTs frequently mimic Leydig cells cytologically, a large unifocal unilateral TART that displaces testicular parenchyma could be confused with an LCT in an adult in whom CAH is unrecognized. This diagnostic puzzle is less likely in a child, because precocious puberty would usually prompt an endocrine workup that would distinguish LCT from TART. This paper describes a case in which an adult presented with metachronous bilateral Leydig cell-like testicular tumors, prompting a workup for congenital adrenal hyperplasia (CAH), and was found to have CAH due to 3β-hydroxysteroid dehydrogenase deficiency
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