原发性空肠单相型滑膜肉瘤临床病理学观察

一例19岁男性患者因腹痛就诊，影像学检查提示腹腔巨大混合性包块并出血。剖腹探查见肿瘤起源于屈氏韧带处空肠，伴破裂出血。镜下见肿瘤由成片或交织束状排列的梭形细胞组成，肿瘤细胞形态较一致，胞质较少，边界不清，核呈梭形或卵圆形，染色质细致，核仁不明显。免疫组织化学显示波形蛋白、上皮膜抗原、转导样增强子蛋白-1、B淋巴细胞瘤蛋白-2、CD99阳性，荧光原位杂交检测检出SS18基因重排，确诊为滑膜肉瘤。患者空肠肿瘤切除术后接受6个周期的化疗，12个月后复查CT提示胰腺转移灶，行1周期放疗后未能继续治疗，于确诊小肠滑膜肉瘤15个月后死亡。

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

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 SS18 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.