Gorlin syndrome presenting with primary infertility and bilateral calcified ovarian fibromas

摘要

Gorlin syndrome (GS) is a genetically inherited multisystem disorder with a rare incidence. We report a young woman with GS who presented with primary infertility and had undergone excision of odontogenic keratocysts of mandible. She had frontal bossing, ptosis and bilateral ovarian calcified fibromas. Calcified ovarian fibromas are present in 15-25% of women with GS and are bilateral in 75% of cases. As GS is associated with other pathologies, such as melanoma, medul-loblastoma, mandibular odontogenic cysts and multiple skeletal deformities, these women need a thorough evaluation.