First described in 1960 by Gorlin and Goltz,[5] NBCCS is an autosomal dominant condition that can cause unusual facial appearances and a predisposition for basal-cell carcinoma, a type of skin cancer which rarely spreads to other parts of the body.

[7] SUFU codes for the suppressor of fused and inhibits the Hh signaling pathway further downstream by binding to glioma-associated (GLI) transcription factors to prevent translocation to the nucleus.

However, a NBCCS diagnosis can also be made by geneticists, dentists, orthodontists, primary care physicians, Mohs surgeons, and oncologists.

[8] Genetic testing is a sufficient way to confirm the diagnosis when there is suspicion but a lack of clinical diagnostic criteria.

[13] Proper sun protection is extremely important for patients with suspected and confirmed diagnoses of NBCCS.

Patients and their families should monitor for signs of NBCCS including developmental delays, abnormal skin lesions, and odontogenic keratocysts in between visits with their multidisciplinary team.

One aspect of NBCCS is that basal-cell carcinomas will occur on areas of the body which are not generally exposed to sunlight, such as the palms and soles of the feet and lesions may develop at the base of palmar and plantar pits.

One of the prime features of NBCCS is development of multiple BCCs at an early age, often in the teen years.

Within the entirety of the GSA community, there is a great amount of support, education, and drive for furthering research.