Basal cell nevus syndrome

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Basal cell nevus syndrome
TermBasal cell nevus syndrome
Short definitionBasal cell nevus syndrome - (pronounced) (BAY-sul sel NEE-vus SIN-drome) rare, inherited condition that affects many organs and tissues in the body. People with this disorder have a very high risk of developing basal cell skin cancer during adolescence or early adulthood. 
TypeCancer terms
SpecialtyOncology
LanguageEnglish
SourceNCI
Comments


Basal cell nevus syndrome - (pronounced) (BAY-sul sel NEE-vus SIN-drome) rare, inherited condition that affects many organs and tissues in the body. People with this disorder have a very high risk of developing basal cell skin cancer during adolescence or early adulthood. They are also at risk of developing medulloblastoma (a type of brain tumor) and other types of cancer. Basal cell nevi syndrome can also cause benign (noncancerous) tumors in the jaw, heart, or ovaries. Other signs and symptoms include a large head and unusual facial features; small dimples in the skin on hands and feet; abnormalities of the spine, ribs or skull; eye problems; and development problems. Basal cell nevus syndrome is caused by a mutation (change) in the PTCH1 gene. Also called BCNS, Gorlin syndrome, NBCCS and nevoid basal cell carcinoma syndrome

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