Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors.
Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors.
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DOI：
10.2350/11-08-1078-pb.1
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发表时间：
2012
期刊：
影响因子：
--
通讯作者：
Dickman PS
中科院分区：
文献类型：
--
作者：
Tsokos M;Alaggio RD;Dehner LP;Dickman PS
尤文氏肉瘤/周围原始神经外胚层肿瘤（EWS/pPNET）和其他EWS基因重排的肿瘤包括一种恶性和中间肿瘤，具有广泛的解剖分布和广泛的年龄范围，但偏爱于儿童，青少年和年轻人的软组织。尽管存在显著的临床和预后差异，但重叠的组织学、免疫组织化学、细胞遗传学和分子遗传学特征给诊断带来了挑战。尤文氏肉瘤是儿童和青少年中第三常见的肉瘤，而结缔组织增生小圆细胞瘤是一种罕见的肿瘤，多见于年龄较大的儿童、青少年和年轻人。病理检查辅以免疫组织化学、细胞遗传学和分子遗传学。本文综述了EWS基因重排肿瘤谱中EWS/pPNET和促结缔组织增生小圆细胞瘤的临床病理特征。具有不同组织病理学特征和EWS基因重排的其他肿瘤在本卷的其他地方讨论。
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.