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

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.


English

ISSN: 0301-0449

Subjects--Topical Terms:
*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 Adult

Subjects--Geographic Terms:
MedStar Washington Hospital Center

Index Terms--Occupation:
Medicine/Oral and Maxillofacial Surgery

Index Terms--Function:
Journal Article