Testicular adrenal rest tumors are commonly seen in congenital adrenal hyperplasia.
The tumors are typically bilateral and arise from ACTH dependent aberrant adrenal
cells in the testes. Diagnosis is clinically confirmed by ultrasound imaging. These
tumors are characterized by their response to steroid replacement and biopsy is not
routinely required. Differentiating the tumor from Leydig cell tumor can be difficult.
Management and prognosis for these two pathologies are different, so extensive investigations
may be required to confirm the diagnosis. We present a 5 year old boy who had an unusual
presentation of a testicular tumor and detail the investigations undertaken to differentiate
a testicular adrenal rest tumor from a Leydig cell tumor.
Key-words:
Congenital adrenal hyperplasia (CAH) - testicular adrenal rest tumor (TART) - Leydig
cell tumor - Synoptophysin
