Pictorial Essay

Pediatric Radiology

, Volume 43, Issue 5, pp 620-627

First online:

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

  • Kamyar SartipAffiliated withDepartment of Radiology, Howard University College of Medicine Email author 
  • , Adam KaplanAffiliated withDepartment of Oral and Maxillofacial Surgery, Washington Hospital Center
  • , George ObeidAffiliated withDepartment of Oral and Maxillofacial Surgery, Washington Hospital Center
  • , Nadja KadomAffiliated withDepartment of Diagnostic Imaging and Radiology, Children’s National Medical Center

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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