title: Nevoid Basal Cell Carcinoma Syndrome: A Long-Term Study in a Family.
abstract: We present a family case series with 10 individuals having nevoid basal cell carcinoma syndrome (NBCCS) with a 10-year follow-up. All articles published in the literature between 1967 and 2011 on familial Gorlin-Goltz syndrome in any language were surveyed to determine the mapping of cases per country of occurrence of this disease. All patients in the present series were presented with calcification of the falx cerebri, mild hypertelorism, and frontal bossing. Odontogenic keratocystic tumors, palmar and plantar pits, and multiple basal cell carcinomas occurred in 90, 40, and 20%, respectively, of the patients. One of the patients died of skin cancer. Diagnosis of odontogenic keratocyst tumors was confirmed by histopathological examination. NBCCS is a rare autosomal dominant cancer predisposition syndrome; it is important to recognize it when a patient has multiple odontogenic keratocyst tumors because life-long monitoring is essential for patient management.
authors.affiliation: Hospital Universitário, Universidade Federal de Sergipe, Rua Claudio Batista, Aracaju, Sergipe, Brazil.
authors.forename: Thiago
authors.initials: T
authors.lastname: de Santana Santos
authors.affiliation: Department of Oral and Maxillofacial Surgery, Pernambuco School of Dentistry, Camaragibe, Pernambuco, Brazil.
authors.forename: André
authors.initials: A
authors.lastname: Vajgel
authors.affiliation: Department of Dentistry, Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil.
authors.forename: Paulo Ricardo Saquete
authors.initials: PR
authors.lastname: Martins-Filho
authors.affiliation: Department of Oral and Maxillofacial Surgery, Pernambuco School of Dentistry, Camaragibe, Pernambuco, Brazil.
authors.forename: Almir Walter
authors.initials: AW
authors.lastname: de Albuquerque Maranhao Filho
authors.affiliation: Department of Oral and Maxillofacial Surgery, University of Pernambuco, Recife, Pernambuco, Brazil.
authors.forename: Ricardo José
authors.initials: RJ
authors.lastname: De Holanda Vasconcellos
authors.affiliation: Department of Oral and Maxillofacial Surgery, Pernambuco School of Dentistry, Camaragibe, Pernambuco, Brazil.
authors.forename: Riedel
authors.initials: R
authors.lastname: Frota
authors.affiliation: Department of Oral and Maxillofacial Surgery, Pernambuco School of Dentistry, Camaragibe, Pernambuco, Brazil.
authors.forename: José Rodrigues Laureano
authors.initials: JR
authors.lastname: Filho
keywords: Gorlin–Goltz syndrome; basal cell carcinoma; nevoid basal cell carcinoma syndrome; odontogenic keratocystic tumor