title: Successful radiation therapy for supratentorial primitive neuroectodermal tumor and epidermolysis bullosa simplex.
abstract: Epidermolysis bullosa simplex (EBS) is a heritable skin disorder characterized by skin fragility and blistering. While its most severe variant, dystrophic epidermolysis bullosa (DEB) is associated with squamous cell carcinoma (SCC), the development of extracutaneous neoplasms in EBS is extremely rare. We report a novel case of supratentorial primitive neuroectodermal tumor (sPNET) in a 7-year male with EBS. Experience of radiation therapy and its challenges in children with EBS has rarely been reported.
authors.affiliation: Department of Radiation Oncology, Childrens Hospital Los Angeles, Los Angeles, California, USA.
authors.forename: Sejal
authors.initials: S
authors.lastname: Bavishi
authors.forename: Kenneth
authors.initials: K
authors.lastname: Wong
authors.forename: Thamani
authors.initials: T
authors.lastname: Delgardo
authors.forename: Araz
authors.initials: A
authors.lastname: Marachelian
authors.forename: Soumen
authors.initials: S
authors.lastname: Khatua
mesh_terms: D002294:Carcinoma, Squamous Cell / Q000473:pathology / Q000532:radiotherapy*; D002648:Child; D016110:Epidermolysis Bullosa Simplex / Q000473:pathology / Q000532:radiotherapy*; D005240:Feasibility Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018242:Neuroectodermal Tumors, Primitive / Q000473:pathology / Q000532:radiotherapy*; D012878:Skin Neoplasms / Q000473:pathology / Q000532:radiotherapy*