What is BCCNS?
Basal Cell Carcinoma Nevus (aka Gorlin or Gorlin – Goltz) Syndrome is a rare disorder that can affect many organ systems in the human body. This syndrome is caused by a genetic mutation that can be inherited or appear on its own. The incidence is low, in that approximately 1 in 27,000 people have BCCNS.
Affected individuals can have a wide variety of manifestations, the most common being basal cell carcinoma (BCC). Other expressions include jaw cysts (keratocystic odontogenic tumors (KCOTS)), pits of the palms and soles, enlarged skulls and prominent foreheads, benign skin cysts, calcifications in the midline of the brain, rib abnormalities, and spina bifida of the thoracic and cervical spine. Additional problems that may occur include ovarian cysts, hydrocephalus, boney abnormalities of the hands and feet. There are several less common issues associated with BCCNS that affect a smaller population in our community.