Management of Condition

Treatment is best provided by specialists with experience of the condition and should include:  

Surgical excision of keratocysts (jaw cysts)

Early treatment of BCCs to ensure their complete eradication and to preserve normal tissue to prevent disfigurement

Preservation of ovarian tissue whenever ovarian fibromas require surgical treatment.

Radiotherapy is to be avoided unless other therapies are unavailable and the use of diagnostic X-rays should be used sparingly. It is essential that individuals protect themselves from the sun by the use of a complete sun block and by covering exposed skin with long sleeves, high collars and hats.

Ref: Neviod Basal Cell Carcinoma Syndrome. Synonyms: Basal Cell Nevus syndrome (BCNS), Gorlin syndrome, NBCCS. D Gareth Evans, MD, FRCP and Peter A Farndon, MD, FRCP Gene Reviews 20301330 Oct 2015

To see full article see Gene Reviews

Next Review January 2018

 Originally produced by Professor P. A. Farndon, Clinical Geneticist, Jim Costello (deceased) and Margaret Costello.  We are reliant on a team of medical advisors for the clinical content of the website. We are grateful for their continuing support. 
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