title: Neuroimaging of nevoid basal cell carcinoma syndrome (NBCCS) in children.
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.
authors.affiliation: Department of Radiology, Howard University College of Medicine, 2041 Georgia Ave, Washington, DC 20060, USA. ksartip@yahoo.com
authors.forename: Kamyar
authors.initials: K
authors.lastname: Sartip
authors.forename: Adam
authors.initials: A
authors.lastname: Kaplan
authors.forename: George
authors.initials: G
authors.lastname: Obeid
authors.forename: Nadja
authors.initials: N
authors.lastname: Kadom
mesh_terms: D000293:Adolescent; D000465:Algorithms; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D020000:Decision Support Systems, Clinical; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D059906:Neuroimaging / Q000379:methods*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D014057:Tomography, X-Ray Computed / Q000379:methods*; D055815:Young Adult