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一例经分子细胞遗传学分析的肠系膜原发性外周型尤因肉瘤/原始神经外胚层肿瘤:罕见病例报告及文献复习

A mesenteric primary peripheral Ewing's sarcoma/primitive neuroectodermal tumor with molecular cytogenetic analysis: Report of a rare case and review of literature.

作者信息

Liao Yi-Shu, Chiang I-Han, Gao Hong-Wei

机构信息

Department of Pathology, Taichung Armed Forces General Hospital, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, ROC.

Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, ROC.

出版信息

Indian J Pathol Microbiol. 2018 Apr-Jun;61(2):248-251. doi: 10.4103/IJPM.IJPM_546_17.

Abstract

Rare cases of Ewing's sarcoma/primitive neuroectodermal tumors (EWS/PNETs) arising from mesenteric tissue have been reported. This report describes an EWS/PNET in a 25-year-old woman who presented with abdominal pain lasting 3 days. Radiologic evaluation revealed a 9 cm × 6 cm homogeneous mass in the lower abdomen with homogeneous enhancement and invasion of the ileum. Surgical resection was completed during exploratory laparotomy. Immunohistochemically, the tumor cells revealed CD99, friend leukemia virus integration-1 and NKX2.2 (NK2 Homeobox 2, a protein coding gene) and subsequently showed EWSR1 rearrangement. The histological feature, immunohistochemical results and genetic fluorescence in situ hybridization analysis of this case were confirming the diagnosis of EWS/PNET. Adjuvant chemotherapy was suggested, but the patient was lost to follow-up.

摘要

已有文献报道罕见的起源于肠系膜组织的尤因肉瘤/原始神经外胚层肿瘤(EWS/PNETs)。本报告描述了一名25岁女性的EWS/PNET,该患者出现持续3天的腹痛。影像学评估显示下腹部有一个9 cm×6 cm的均匀肿块,呈均匀强化并侵犯回肠。在剖腹探查术中完成了手术切除。免疫组织化学检查显示,肿瘤细胞表达CD99、Friend白血病病毒整合1和NKX2.2(NK2同源盒2,一种蛋白质编码基因),随后显示EWSR1重排。该病例的组织学特征、免疫组织化学结果和基因荧光原位杂交分析均证实了EWS/PNET的诊断。建议进行辅助化疗,但患者失访。

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