Nevoid Basal Cell Carcinoma Syndrome: Causes, Symptoms, and Treatment
Written by Sumiran Racherla, MD. Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 2, 2026.
What kind of doctor treats Nevoid Basal Cell Carcinoma Syndrome?
Care usually involves a team: dermatologists treat the skin cancers, oral and maxillofacial surgeons remove jaw cysts, geneticists and genetic counselors help with testing, and primary care doctors coordinate the rest. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What it is
Nevoid basal cell carcinoma syndrome, also called Gorlin syndrome, is a rare inherited condition. People who have it tend to develop basal cell carcinoma, a slow-growing skin cancer. It can also cause noncancerous jaw growths, palm and sole pits, and other features.
Symptoms
The most common sign is many basal cell skin cancers, often starting young and sometimes in places rarely in the sun. Other signs include pits on the palms and soles and benign jaw tumors called odontogenic keratocysts, which can swell or shift teeth. Some people have calcium deposits in the brain, wide-set eyes, or rib and spine differences. Symptoms vary even within families.
Causes and who gets it
The syndrome is caused by a mutation in a gene that normally controls how cells grow. Most people inherit it from a parent, but sometimes the change appears first in a child with no family history. A person with the changed gene has a 50 percent chance of passing it to each child.
How doctors diagnose it
Doctors use medical and family history, a physical exam, and test results. Multiple basal cell skin cancers at a young age, jaw cysts, and palm or sole pits are strong clues. Genetic testing can confirm the diagnosis.
Treatment
There is no cure for the syndrome itself, so care focuses on monitoring. Skin cancers can be removed with surgery, scraping, freezing, or medicated creams. Jaw cysts are usually removed surgically. Regular skin checks, dental X-rays, and sun protection are key.
When to see a doctor
See a doctor for a new skin growth, a sore that does not heal, or a changing spot. Jaw swelling, loose teeth, or facial pain also deserve a prompt visit. If it runs in your family, ask about genetic testing.
How a direct primary care or concierge doctor fits in
Direct primary care and concierge doctors offer longer visits and easier access, often with same-day appointments. That helps people with a rare condition stay on top of frequent skin checks and quick referrals while coordinating care among many providers.
Common questions
Is Nevoid Basal Cell Carcinoma Syndrome the same as regular skin cancer?
No. Regular basal cell carcinoma is usually a single skin cancer from sun exposure. The syndrome is inherited and causes many of these cancers, often at a young age and in sun-protected spots. People with the syndrome need closer monitoring.
Can it be passed to my children?
Yes, a parent with the changed gene has a one in two chance of passing it to each child. Some cases appear for the first time with no family history. Genetic counselors can explain testing options.
Will the jaw cysts come back?
Jaw cysts can return after removal, which is why regular dental X-rays and follow-up are part of care. Some people need more than one procedure over the years. Your care team will set a monitoring schedule.
Can people with this syndrome live normal lives?
Many people with the syndrome do well with regular monitoring and prompt treatment. Outcomes differ depending on which features they have. Staying out of the sun and keeping up with checkups makes a big difference.
Sources
- MedlinePlus Genetics - Gorlin syndrome
- NIH Genetic and Rare Diseases Information Center - Gorlin syndrome
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
