Ovarian Fibromas and the Association With Gorlin ’s Syndrome: A Case Report and Review

Ovarian tumors are rare in children and adolescents, with an incidence of approximately 2 per 100,000 girls. Specifically, ovarian fibromas represent only 4% of these tumors. Ovarian fibromas have been associated with a genetic syndrome called Gorlin syndrome, also known as Gorlin-Goltz syndrome, nevoid basal cell carcinoma syndrome (NBCCS), and basal cell nevus syndrome (BCNS). This is a rare, autosomal-dominant, inherited mutation of the patched (PTCH) tumor-suppressor gene. Gorlin ’s syndrome consists of a constellation of symptoms including musculoskeletal abnormalities, dermatologic findings of multiple basal cell nevi, and a predisposition to certain neoplasms, including ovarian fibromas, medulloblastoma, and fetal rhabdomyomas.

Source: Journal of Pediatric and Adolescent Gynecology - April 1, 2017 Category: OBGYN Authors: Mariam Hanna, Meredith Loveless, Margaret Abraham, Elizabeth Bonagura, Rachael Polis, S. Paige Hertweck Source Type: research