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What is Nevoid Basal Cell Carcinoma Syndrome?

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What is Nevoid Basal Cell Carcinoma Syndrome?

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10

Nevoid basal cell carcinoma syndrome (NBCCS) is also known as Gorlin syndrome. NBCCS is a hereditary condition characterized by multiple basal cell skin cancers or basal cell nevus syndrome. Other common findings include jaw cysts, pits on the palms of the hands or soles of the feet, calcium deposits in soft tissues, and skeletal (bone) changes. The appearance of a person with NBCCS may include a larger head size, a prominent forehead, broad bridge of the nose, wide spaced eyes, skin cysts, and small skin bumps called milia. The jaw cysts and basal cell skin cancers usually develop in the first ten years of a person’s life, but they may not appear until teenage years or early adulthood. Children with NBCCS may have the appearance features described above, including pits on their hands and feet. There is a small (5%) chance for children with NBCCS to develop a type of brain cancer called medulloblastoma. Multiple basal cell skin cancers and jaw cysts are the most common features of NBCC

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