Basal Cell Nevus Syndrome

Basal Cell Nevus Syndrome (Gorlin Syndrome)

The risk for ovarian cancer is increased with basal cell nevus syndrome (also called Gorlin syndrome and nevoid basal cell carcinoma), a rare autosomal dominant cancer genetic syndrome. Approximately 600 cases have been identified. Features associated with basal cell nevus syndrome may include the following:

Basal Cell Nevus Syndrome

  • Development of more than two basal cell carcinomas (cancer of the outer layer of the skin) before the age of 30
  • Cysts in the jaw
  • Characteristic facial appearance (60% of people)
  • Calcification of the falx (a variation in the appearance of the skull that is visible on X-rays)
  • Pits in the palms and soles of the feet
  • Eye abnormalities
  • Rib or vertebral abnormalities
  • Increased risk of medulloblastoma
  • Increased risk of cardiac and ovarian fibromas (benign, or noncancerous, tumors)
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