Pediatric Radiology

, Volume 43, Issue 5, pp 620–627

Neuroimaging of nevoid basal cell carcinoma syndrome (NBCCS) in children

Authors

    • Department of RadiologyHoward University College of Medicine
  • Adam Kaplan
    • Department of Oral and Maxillofacial SurgeryWashington Hospital Center
  • George Obeid
    • Department of Oral and Maxillofacial SurgeryWashington Hospital Center
  • Nadja Kadom
    • Department of Diagnostic Imaging and RadiologyChildren’s National Medical Center
Pictorial Essay

DOI: 10.1007/s00247-012-2516-x

Cite this article as:
Sartip, K., Kaplan, A., Obeid, G. et al. Pediatr Radiol (2013) 43: 620. doi:10.1007/s00247-012-2516-x

Abstract

Nevoid basal cell carcinoma syndrome (NBCCS, Gorlin syndrome) is an autosomal dominant condition with a wide range of manifestations, including multiple basal cell carcinomas, medulloblastoma, odontogenic keratocysts (OKC) and skeletal abnormalities. Children with NBCCS also have a predisposition for secondary cancers after exposure to ionising radiation. In children undergoing imaging for posterior fossa mass and/or maxillofacial cysts, certain additional findings can raise the possibility of NBCCS. Making the diagnosis can significantly impact patient management, especially for children with medulloblastoma.

Keywords

Nevoid basal cell carcinoma Odontogenic keratocysts Medulloblastoma Gorlin syndrome Children

Copyright information

© Springer-Verlag 2012