What is nevoid basal cell carcinoma syndrome (NBCCS)?
Nevoid basal cell carcinoma syndrome (NBCCS), also known as “Gorlin syndrome,” is a hereditary cancer syndrome characterized by: • A type of skin cancer known as basal cell carcinoma (BCC) • Specific growths within the jaw bones known as keratocysts • Distinctive small pits within the palms of the hands and soles of the feet • Skeletal abnormalities, such as differences in the shape of the ribs and vertebrae • Characteristic facial features, which can include: • Hypertelorism (wide-set eyes) • Macrocephaly (larger than average head size) with or without hydrocephalus (build up of fluid inside the brain) • Milia (tiny white cysts embedded in the skin, commonly appearing on the cheeks and around the eyes and lips) In addition to developing one or more basal cell carcinomas, individuals with nevoid basal cell carcinoma syndrome (NBCCS) are also at increased risk to develop other tumor types including: • Fibromas (benign connective tissue tumors) of the heart and ovaries • Medulloblastoma,
