Ovarian fibromas in pediatric patients with basal cell nevus (Gorlin) syndrome.

Background: Gorlin syndrome is a rare genetic condition consisting of multiple basal cell nevi associated with other entities such as medulloblastoma, skeletal abnormalities, and ovarian fibromas.
Case: A 15-year-old girl presented with abdominal discomfort. Magnetic resonance imaging showed multiple bilateral solid adnexal masses, the largest measuring 5.5 cm × 6.1 cm × 5.6 cm. At laparoscopy, 10 ovarian fibromas, ranging from 3 mm to 7 cm in size, were removed from each ovary. Concurrent with her gynecologic course, she was found to have maxillary sinus cysts and multiple basal cell nevi. The patient's history was also significant for a medulloblastoma as an infant. Given this constellation of findings, a diagnosis of Gorlin syndrome was made.
Conclusion: The development of ovarian fibromas in the pediatric population is rare. When diagnosed, the possibility of Gorlin syndrome must be considered. Furthermore, females with Gorlin syndrome would benefit from regular gynecologic surveillance.
(Copyright © 2011 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)