原发性空肠单相型滑膜肉瘤临床病理学观察

doi:10.3724/zdxbyxb-2022-0117

浙江大学学报（医学版）

2022, Vol. 51

Issue (4): 474-479 DOI: 10.3724/zdxbyxb-2022-0117

病例报告

原发性空肠单相型滑膜肉瘤临床病理学观察

王亚健,蒋娜,蒋依娜,王鸿雁

西安交通大学第一附属医院病理科，陕西西安 710061

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

WANG Yajian,JIANG Na,JIANG Yina,WANG Hongyan

Department of Pathology, the First Affiliated Hospital of Xi’an Jiaotong University, Xi’an 710061, China

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摘要：

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。

关键词： 滑膜肉瘤; 空肠; 临床病理学; 病例报告

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 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.

Key words: Synovial sarcoma Jejunum Clinical pathology Case report

收稿日期: 2022-03-21 出版日期: 2022-11-16

CLC:

R735.3

通讯作者: 王鸿雁

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引用本文:

王亚健,蒋娜,蒋依娜,王鸿雁. 原发性空肠单相型滑膜肉瘤临床病理学观察[J]. 浙江大学学报（医学版）, 2022, 51(4): 474-479.

WANG Yajian,JIANG Na,JIANG Yina,WANG Hongyan. Clinicopathologic features of a patient with primary monophasic synovial sarcoma of the jejunum. J Zhejiang Univ (Med Sci), 2022, 51(4): 474-479.

链接本文:

https://www.zjujournals.com/med/CN/10.3724/zdxbyxb-2022-0117 或 https://www.zjujournals.com/med/CN/Y2022/V51/I4/474

图1 本病例原发性空肠滑膜肉瘤的CT影像学表现腹腔及盆腔内偏左巨大占位性病变（箭头所示），肠系膜走行区淋巴结大.

图2 本例原发性空肠滑膜肉瘤的组织病理学表现 A：肿瘤主要位于肠壁内, 边界较清楚, 伴黏膜面糜烂（标尺 =400 μm）；B：肿瘤局部区呈囊性（标尺 =500 μm）；C：肿瘤由交织束状排列的梭形细胞组成（标尺 =200 μm）；D：肿瘤细胞形态较一致, 核浆比大, 核呈梭形, 染色质细致, 核仁不明显, 核分裂少见（标尺= 50 μm）；E：肿瘤局灶区EMA阳性（标尺 =100 μm）；F：肿瘤弥漫TLE-1阳性（标尺 =100 μm）. EMA：上皮膜抗原；TLE：转导样增强子蛋白.

图3 本例原发性空肠滑膜肉瘤肿瘤细胞荧光原位杂交检测结果细胞核内出现红、绿分离信号，提示存在基因重排. 标尺 =100 μm.

表1 16例下消化道原发性滑膜肉瘤的临床病理学特征

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8	ROMEO S , ROSSI S , ACOSTA MARÍN M , et al.Primary synovial sarcoma (SS) of the digestive system: a molecular and clinicopathological study of fifteen cases[J]Clin Sarcoma Res, 2015, 5( 1): 7. doi: 10.1186/s13569-015-0021-3
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11	HOSTETTER R B , YAN M , VAGHEFI H , et al.The first documented case of high-grade synovial cell sarcoma of the rectum[J]J Natl Compr Canc Netw, 2012, 10( 8): 947-950. doi: 10.6004/jnccn.2012.0098
12	ERIKSEN C , BURNS L , BOHLKE A , et al.Management of monophasic synovial sarcoma of the small intestine[J]JSLS, 2010, 14( 3): 421-425. doi: 10.4293/108680810x12924466006846
13	PARFITT J R , XU J , KONTOZOGLOU T , et al.Primary monophasic synovial sarcoma of the colon[J]Histopathology, 2007, 50( 4): 521-523. doi: 10.1111/j.1365-2559.2007.02603.x
14	WONG N A C S , CAMPBELL F , SHEPHERD N A . Abdominal monophasic synovial sarcoma is a morphological and immunohistochemical mimic of gastrointestinal stromal tumour[J]Histopathology, 2015, 66( 7): 974-981. doi: 10.1111/his.12593
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