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

MedStar author(s):
Citation: Pediatric Radiology. 43(5):620-7, 2013 Mar.PMID: 23151728Institution: MedStar Washington Hospital CenterDepartment: Medicine/Oral and Maxillofacial SurgeryForm of publication: Journal ArticleMedline article type(s): Journal ArticleSubject headings: *Algorithms | *Basal Cell Nevus Syndrome/di [Diagnosis] | *Decision Support Systems, Clinical | *Magnetic Resonance Imaging/mt [Methods] | *Neuroimaging/mt [Methods] | *Tomography, X-Ray Computed/mt [Methods] | Adolescent | Child | Child, Preschool | Diagnosis, Differential | Female | Humans | Infant | Infant, Newborn | Male | Reproducibility of Results | Sensitivity and Specificity | Young AdultISSN:
  • 0301-0449
Name of journal: Pediatric radiologyAbstract: 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.All authors: Kadom N, Kaplan A, Obeid G, Sartip KDigital Object Identifier: Date added to catalog: 2014-02-24
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Journal Article MedStar Authors Catalog Article Available 23151728

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.

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