Conservative management of bilateral Sertoli cell tumors of the testicle in association with the Carney complex: a case report.
Large cell calcifying Sertoli cell tumor of the testicle is a rare, hormonally active sex cord-stromal tumor seen in patients with Carney complex. When such tumors occur bilaterally, treatment options for preserving fertility and addressing the secondary effects of excess hormone production must be considered. The availability of specific antiestrogen drugs means that bilateral orchiectomy for this benign tumor may no longer be warranted. Testicular-sparing surgery and advances in reproductive technology may also improve the overall prognosis for fertility. Gynecomastia in prepubescent boys can be emotionally very distressing. Approximately two thirds of teenaged boys will develop some degree of breast enlargement that spontaneously regresses as testosterone levels rise (Ill Med J 1938;73:113). In all cases, a thorough history and physical examination are required to exclude nonphysiologic causes such as drugs, pulmonary disease, chronic liver disease, exogenous estrogens, and estrogen-producing tumors (Seashore J. Disorders of the breast. In: Rowe MI, O'Neill JA, Grosfeld JL et al, editors. Pediatric surgery, 5th ed. St Louis (MO): Mosby Year Book, 1998). We report on a child who presented with a 2-year history of gynecomastia with associated bilateral testicular swellings and discuss a novel treatment strategy for managing bilateral testicular tumors in the context of the Carney complex.
Brown
B
B
Royal Manchester Children's Hospital, Hospital Road, Pendlebury, Manchester M27 4HA, UK. bazil.brown@gmail.com
Ram
A
A
Clayton
P
P
Humphrey
G
G
D002648:Child; D006177:Gynecomastia / Q000150:complications / Q000628:therapy; D006801:Humans; D008297:Male; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis* / Q000628:therapy; D012707:Sertoli Cell Tumor / Q000150:complications / Q000473:pathology / Q000628:therapy*; D013736:Testicular Neoplasms / Q000150:complications / Q000473:pathology / Q000628:therapy*