Gorlin syndrome is a rare condition. It is also sometimes called naevoid basal cell carcinoma syndrome (NBCCS). You may also see it written as Gorlin's Syndrome. It affects about 1 in 31,000 people, although the true figure may be higher as mild cases can go unrecognised. Between 70 and 80 out of every 100 people (70 to 80%) with Gorlin syndrome have someone else in their family with it, and have inherited a genetic mutation from one of their parents.
Gorlin Syndrome is separated into 2 types – major and minor. People who have Gorlin syndrome may have a number of different medical conditions including
- Skin problems – skin tags and skin cysts
- Cysts on your jaw that usually develop during your teens
- Medulloblastoma (PNET)
- Changes in the bones – they may be longer and larger than usual
- Pits on the palms of the hands and soles of the feet
- Benign tumours of the ovary – these develop in 20 out of every 100 people (20%) with the syndrome
Your doctor decides whether you have major or minor Gorlin syndrome by looking at how you are affected.
Although people with Gorlin syndrome have an increased risk of developing PNET, this risk is small. PNET is a type of brain tumour which usually affects children between the ages of 2 and 5. They develop in about 5 out of every 100 children (5%) who have Gorlin syndrome. Only a few children who have PNET also have Gorlin syndrome.
You may feel overwhelmed when you hear that you have a condition that can increase your risk of developing medical problems. It may help you to know that many of the conditions are manageable. For some of the conditions you won’t need any medical treatment. Even if you do need treatment, it is likely to work well, including the treatment for skin cancer. We have a page with information about treating skin cancer when you have Gorlin syndrome. It may be reassuring for you to know that people who have Gorlin syndrome usually live as long as people who don’t have the syndrome.
The Gorlin Syndrome Group have detailed information on their website and offer support to people affected by Gorlin syndrome.
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