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Ovarian Fibromas in Gorlin Syndrome

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Nevoid basal cell carcinoma syndrome (Gorlin Syndrome): It is characterized by multiple basal cell carcinomas, odontogenic keratocysts of the jaw, skeletal abnormalities, ectopic calcification, and various tumors (ovarian and cardiac fibromas, meningiomas, medulloblastomas, ameloblastoma, thyroid neoplasms and many other rare tumors).

Ovarian fibromas develop in 75% of females with Gorlin syndrome, often presenting at younger age (under 30 years) than conventional ovarian fibromas (50-60 years). They are often bilateral, multinodular, multifocal, and heavily calcified.

The image shows an ovarian fibroma (not a case of Gorlin syndrome) partially replacing the ovary. The tumor is solid, homogenous, tan-white, and has small pseudocystic areas. Note the corpora lutea and albicantia in the right half of the image. Image courtesy of Ed Uthman, MD, Houston, Texas. Used with permission.

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