Nevoid basal-cell syndrome: literature review and case report in a family.
The Nevoid Basal-Cell Carcinoma Syndrome (NBCC), or as it is also referred to, basal-cell nevus syndrome or Gorlin-Goltz syndrome, is characterized by multiple early-appearing basal cell carcinomas, keratocytosis of the mandible, and anomalies of the ocular, skeletal reproductive system. We describe four patients in the same family, all of them possessing a large number of skin tumors associated with other typical clinical and X-Ray anomalies of NBCC. The definitive treatment of NBCC has yet to be established, however, early diagnosis is very important as well as the periodical follow-up examination of ten patients, mainly due to the transformations in the skin lesions that may occur.
José Tincani
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Head and Neck Surgical Services, Medical School of UNICAMP, Campinas, SP, Brazil.
Santos Martins
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Gomes Andrade
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Franco Mello
E J
EJ
Camargo Bueno
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MA
D000328:Adult; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery