Division of Surgical Pathology, Tokyo Medical and Dental University Hospital, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
Department of Pathology, Soka Municipal Hospital, 2-21-1, Soka, Saitama, 340-8560, Japan.
Clin J Gastroenterol. 2021 Aug;14(4):1020-1026. doi: 10.1007/s12328-021-01408-4. Epub 2021 Apr 12.
Worldwide, 5-10% of soft tissue sarcoma cases in adults have been attributed to synovial sarcoma. It is often reported to occur near the joints of the arm, neck, and leg but rarely in the gastrointestinal tract. In this study, we report a case of synovial sarcoma arising in the stomach of a 59-year-old woman. Gastrointestinal endoscopy revealed an ulcerative and hemorrhagic tumor with marginal elevation in the fundus. Histological study showed that the tumor was composed of tightly packed spindle cells in bundles, and one of its component demonstrated significant mitotic activity (> 40/10 high-power fields) in several areas. The diagnosis was confirmed by the evidence of SS18 gene rearrangement, according to immunohistochemistry study, (including a novel SS18-SSX fusion-specific antibody), fluorescent in situ hybridization, and the identification of the SS18-SSX1 and SS18-SSX1/2/4 fusion transcripts using reverse-transcript polymerase chain reaction. No evidence of local recurrence or distant metastasis has been found in the more than 5 years since. Distinguishing synovial sarcoma in the digestive tract from other mesenchymal neoplasms, such as gastrointestinal stromal tumor, may be difficult, especially when spindle-shaped cell proliferation is predominant, as in our patient. Therefore, morphological, immunohistological, and molecular evaluations are important for a comprehensive diagnosis.
在全球范围内,成人软组织肉瘤病例中有 5-10%归因于滑膜肉瘤。它通常发生在手臂、颈部和腿部关节附近,但很少发生在胃肠道。在本研究中,我们报告了一例发生在 59 岁女性胃中的滑膜肉瘤。胃肠内窥镜检查显示在胃底部有一个溃疡性和出血性肿瘤,边缘隆起。组织学研究显示,肿瘤由束状紧密排列的梭形细胞组成,其中一个成分在几个区域具有明显的有丝分裂活性(>40/10 高倍视野)。根据免疫组织化学研究(包括一种新型的 SS18-SSX 融合特异性抗体)、荧光原位杂交和逆转录聚合酶链反应检测 SS18-SSX1 和 SS18-SSX1/2/4 融合转录本的证据,诊断得到了确认。自 5 年多以来,未发现局部复发或远处转移的证据。从其他间叶性肿瘤(如胃肠道间质瘤)中鉴别出消化道中的滑膜肉瘤可能具有一定难度,尤其是当以梭形细胞增殖为主时,如我们的患者。因此,形态学、免疫组织化学和分子评估对于全面诊断很重要。