Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors.
Ewing sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and other tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults. The overlapping histologic, immunohistochemical and cytogenetic and molecular genetic features create diagnostic challenges despite significant clinical and prognostic differences. Ewing sarcoma is the 3rd most common sarcoma in children and adolescents, and desmoplastic small round cell tumor is a rare neoplasm that occurs more often in older children, adolescents, and young adults. Pathologic examination is complemented by immunohistochemistry, cytogenetics, and molecular genetics. This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell tumor in the spectrum of tumors with EWS gene rearrangements. Other tumors with different histopathologic features and an EWS gene rearrangement are discussed elsewhere in this volume.
Department of Pathology, National Institutes of Health, Bethesda, MD, USA.
SourcePediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society 15:1 Suppl 2012 pg 108-26
Desmoplastic Small Round Cell Tumor
Neuroectodermal Tumors, Primitive, Peripheral
RNA-Binding Protein EWS
Pub Type(s)Journal Article