Metachronous Bilateral Testicular Leydig-Like Tumors Leading to the Diagnosis of Congenital Adrenal Hyperplasia (Adrenogenital Syndrome).
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.
Department of Urology, University of North Carolina, Chapel Hill, NC 27599, USA.
Josip
J
Vukina
Division of Hematology and Oncology, Department of Medicine, University of North Carolina, Chapel Hill, NC 27599, USA ; Lineberger Comprehensive Cancer Center, Chapel Hill, NC 27599, USA.
David D
DD
Chism
Lineberger Comprehensive Cancer Center, Chapel Hill, NC 27599, USA ; Division of Endocrinology and Metabolism, Department of Medicine, University of North Carolina, Chapel Hill, NC 27599, USA.
Julie L
JL
Sharpless
Department of Urology, University of North Carolina, Chapel Hill, NC 27599, USA.
Mathew C
MC
Raynor
Division of Hematology and Oncology, Department of Medicine, University of North Carolina, Chapel Hill, NC 27599, USA ; Lineberger Comprehensive Cancer Center, Chapel Hill, NC 27599, USA.
Matthew I
MI
Milowsky
Lineberger Comprehensive Cancer Center, Chapel Hill, NC 27599, USA ; Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, NC 27599, USA.
William K
WK
Funkhouser