basal cell nevus syndrome

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Nevoid basal cell carcinoma syndrome
The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) (also known as Basal Cell Nevus Syndrome, Multiple Basal Cell Carcinoma Syndrome, Gorlin syndrome, or Gorlin-Goltz syndrome) is an inherited group of multiple defects involving the skinnervous systemeyesendocrine system, and bones.First described in 1960, NBCCS is an autosomal dominant condition that can cause unusual facial appearances and a predisposition for skin cancer. The prevalence is reported to be 1 case per 56,000-164,000 population. Recent work in molecular genetics has shown NBCCS to be caused by mutations in the PTCH (patched) gene found on chromosome arm 9q. If a child inherits the defective gene from either parent, he or she will have the disorder.
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Basal Cell Nevus Syndrome
Basal cell nevus syndrome Nevoid basal cell carcinoma syndrome, basal cell carcinoma syndrome, Gorlin-Goltz syndrome A rare AD condition [more]Basal Cell Nevus Syndrome - Community and Resources


NCI Dictionary of Cancer Terms Download this dictionary
basal cell nevus syndrome
An inherited condition that causes unusual facial features and disorders of the skin, bone, nervous system, eyes, and endocrine glands. People with this syndrome have an increased chance of developing basal cell carcinoma. Also called Gorlin syndrome. 

A Service of the National Cancer Institute.

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