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原发性空肠单相滑膜肉瘤 1 例临床病理特征。

Clinicopathologic features of a patient with primary monophasic synovial sarcoma of the jejunum.

出版信息

Zhejiang Da Xue Xue Bao Yi Xue Ban. 2022 Aug 1;51(4):474-479. doi: 10.3724/zdxbyxb-2022-0117.

Abstract

A case of primary synovial sarcoma of the jejunum was collected and analyzed retrospectively. A 19-year-old man who presented to hospital with abdominal pain. The CT scan showed a large mixed abdominal mass with bleeding. Laparotomy revealed that the tumor originated from the jejunum, accompanied by rupture and hemorrhage. Microscopically, the tumor was composed of spindle cells. The tumor cells demonstrated diffuse expression of vimentin, transducin-like enhancer (TLE)-1, B-cell lymphoma protein (Bcl)-2, CD99 and focal expression of epithelial membrane antigen (EMA). The presence of specific gene rearrangement was confirmed in tumor cells. The patient received 6 cycles of chemotherapy after jejunal tumor resection. And 12 months later, the patient presented pancreatic metastasis and had radiotherapy. The patient died 15 months after the diagnosis.

摘要

本文回顾性收集并分析了一例原发性空肠滑膜肉瘤病例。一名 19 岁男性因腹痛就诊。CT 扫描显示腹部有一个大的混杂性肿块,伴有出血。剖腹探查显示肿瘤来源于空肠,伴有破裂和出血。显微镜下,肿瘤由梭形细胞组成。肿瘤细胞弥漫表达波形蛋白、转导素样增强子(TLE)-1、B 细胞淋巴瘤蛋白(Bcl)-2、CD99,局灶性表达上皮膜抗原(EMA)。肿瘤细胞中存在特定的基因重排。空肠肿瘤切除术后,患者接受了 6 个周期的化疗。12 个月后,患者出现胰腺转移,接受了放疗。诊断后 15 个月,患者死亡。

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