title: Nevoid basal-cell syndrome: literature review and case report in a family.
abstract: 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.
authors.affiliation: Head and Neck Surgical Services, Medical School of UNICAMP, Campinas, SP, Brazil.
authors.forename: A
authors.initials: A
authors.lastname: José Tincani
authors.forename: A
authors.initials: A
authors.lastname: Santos Martins
authors.forename: R
authors.initials: R
authors.lastname: Gomes Andrade
authors.forename: E J
authors.initials: EJ
authors.lastname: Franco Mello
authors.forename: M A
authors.initials: MA
authors.lastname: Camargo Bueno
mesh_terms: 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