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.