Ewing family tumours: a paediatric perspective

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• 作者：Gino R. Somers
• 关键词：angiomatoid fibrous histiocytoma ; clear cell sarcoma ; desmoplastic small round cell tumour ; Ewing sarcoma ; EWSR1 ; paediatric
• 刊名：Diagnostic Histopathology
• 出版年：February, 2014
• 年：2014
• 卷：20
• 期：2
• 页码：49-55
• 全文大小：4241 K

文摘

Sarcomas are malignant tumours of the connective tissues and are proportionately much more common in children than in adults. The Ewing family of tumours (EFT) is a group of sarcomas sharing rearrangement of the EWSR1 gene on 22q12, and include Ewing sarcoma/primitive neuroectodermal tumour, desmoplastic small round cell tumour, angiomatoid fibrous histiocytoma and clear cell sarcoma. Other tumours harbouring EWSR1 rearrangements include myoepithelial tumours, myxoid liposarcoma and extraskeletal chondrosarcoma. In addition, a group of Ewing-like primitive round cell sarcomas have been recently described in a paediatric population, further expanding the list of EFT. This review will focus on the histopathological, immunohistochemical and molecular genetic features of EFT, with an emphasis on those predominantly occurring in the paediatric population.