Bilateral testicular adrenal rests after bilateral adrenalectomies in a cushingoid patient with von Hippel-Lindau disease.
We report a case of bilateral testicular masses in a 25-year-old man with von Hippel-Lindau disease presenting with cushingoid symptoms. His medical history was significant for bilateral adrenalectomies secondary to pheochromocytomas, and he began steroid therapy at that time. After exhaustive endocrinologic, radiographic, and physical examinations, the testicular masses were postulated to be active adrenal rest tissue. Bilateral testicular venous sampling found elevated glucocorticoids that were responsive to dexamethasone suppression, which confirmed the testicular masses as testicular adrenal rests without the need for surgical intervention. Successful conservative management consisted of appropriate steroid manipulation and radiographic evaluation and resulted in the resolution of presenting symptoms, a decrease in size of the bilateral testicular masses, and testicular conservation in this young man.
Weeks
D Champ
DC
Urologic Oncology Branch, National Cancer Institute, Bethesda, Maryland, USA.
Walther
McClellan M
MM
Stratakis
Constantine A
CA
Hwang
Jonathan J
JJ
Linehan
W Marston
WM
Phillips
John L
JL
D000310:Adrenal Gland Neoplasms / Q000601:surgery; D000314:Adrenal Rest Tumor / Q000097:blood / Q000378:metabolism*; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000097:blood / Q000378:metabolism*; D000328:Adult; D003480:Cushing Syndrome / Q000097:blood / Q000175:diagnosis; D006801:Humans; D006854:Hydrocortisone / Q000097:blood / Q000378:metabolism*; D008297:Male; D010673:Pheochromocytoma / Q000601:surgery; D010859:Pigmentation Disorders / Q000188:drug therapy; D013736:Testicular Neoplasms / Q000097:blood / Q000378:metabolism*; D006623:von Hippel-Lindau Disease / Q000097:blood* / Q000150:complications
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone