Successful radiation therapy for supratentorial primitive neuroectodermal tumor and epidermolysis bullosa simplex

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 rar...

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Bibliographic Details
Published in:Pediatric blood & cancer 2010-01, Vol.54 (1), p.170-172
Main Authors: Bavishi, Sejal, Wong, Kenneth, Delgardo, Thamani, Marachelian, Araz, Khatua, Soumen
Format: Article
Language:English
Subjects:
Carcinoma, Squamous Cell - pathology
Carcinoma, Squamous Cell - radiotherapy
Child
epidermolysis bullosa simplex
Epidermolysis Bullosa Simplex - pathology
Epidermolysis Bullosa Simplex - radiotherapy
Feasibility Studies
Humans
Magnetic Resonance Imaging
Male
Neuroectodermal Tumors, Primitive - pathology
Neuroectodermal Tumors, Primitive - radiotherapy
radiation therapy
Skin Neoplasms - pathology
Skin Neoplasms - radiotherapy
supratentorial PNET
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Summary: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. Pediatr Blood Cancer 2010; 54:170–172. © 2009 Wiley‐Liss, Inc.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.22281