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10.1055/s-0035-1558454
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Craniomaxillofacial Trauma & Reconstruction is published by MDPI from Volume 18 Issue 1 (2025). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with Sage.
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Case Report

Nevoid Basal Cell Carcinoma Syndrome: A Long-Term Study in a Family

by
Thiago de Santana Santos
1,*,
André Vajgel
2,
Paulo Ricardo Saquete Martins-Filho
3,
AlmirWalter de Albuquerque Maranhao Filho
2,
Ricardo José De Holanda Vasconcellos
4,
Riedel Frota
2 and
José Rodrigues Laureano Filho
2
1
Hospital Universitário, Universidade Federal de Sergipe, Rua Claudio Batista, Aracaju, Sergipe, Brazil
2
Department of Oral and Maxillofacial Surgery, Pernambuco School of Dentistry, Camaragibe, Pernambuco, Brazil
3
Department of Dentistry, Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil
4
Department of Oral and Maxillofacial Surgery, University of Pernambuco, Recife, Pernambuco, Brazil
*
Author to whom correspondence should be addressed.
Craniomaxillofac. Trauma Reconstr. 2016, 9(1), 94-104; https://doi.org/10.1055/s-0035-1558454 (registering DOI)
Submission received: 27 December 2014 / Revised: 22 February 2015 / Accepted: 22 February 2015 / Published: 3 August 2015

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.
Keywords: nevoid basal cell carcinoma syndrome; Gorlin-Goltz syndrome; basal cell carcinoma; odontogenic keratocystic tumor nevoid basal cell carcinoma syndrome; Gorlin-Goltz syndrome; basal cell carcinoma; odontogenic keratocystic tumor

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MDPI and ACS Style

de Santana Santos, T.; Vajgel, A.; Martins-Filho, P.R.S.; de Albuquerque Maranhao Filho, A.; De Holanda Vasconcellos, R.J.; Frota, R.; Filho, J.R.L. Nevoid Basal Cell Carcinoma Syndrome: A Long-Term Study in a Family. Craniomaxillofac. Trauma Reconstr. 2016, 9, 94-104. https://doi.org/10.1055/s-0035-1558454

AMA Style

de Santana Santos T, Vajgel A, Martins-Filho PRS, de Albuquerque Maranhao Filho A, De Holanda Vasconcellos RJ, Frota R, Filho JRL. Nevoid Basal Cell Carcinoma Syndrome: A Long-Term Study in a Family. Craniomaxillofacial Trauma & Reconstruction. 2016; 9(1):94-104. https://doi.org/10.1055/s-0035-1558454

Chicago/Turabian Style

de Santana Santos, Thiago, André Vajgel, Paulo Ricardo Saquete Martins-Filho, AlmirWalter de Albuquerque Maranhao Filho, Ricardo José De Holanda Vasconcellos, Riedel Frota, and José Rodrigues Laureano Filho. 2016. "Nevoid Basal Cell Carcinoma Syndrome: A Long-Term Study in a Family" Craniomaxillofacial Trauma & Reconstruction 9, no. 1: 94-104. https://doi.org/10.1055/s-0035-1558454

APA Style

de Santana Santos, T., Vajgel, A., Martins-Filho, P. R. S., de Albuquerque Maranhao Filho, A., De Holanda Vasconcellos, R. J., Frota, R., & Filho, J. R. L. (2016). Nevoid Basal Cell Carcinoma Syndrome: A Long-Term Study in a Family. Craniomaxillofacial Trauma & Reconstruction, 9(1), 94-104. https://doi.org/10.1055/s-0035-1558454

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