Su Hao, Liu Wen-Sheng, Ren Wen-Hao, Wang Peng, Shi Lei, Zhou Hai-Tao
Hao Su, Peng Wang, Lei Shi, Hai-Tao Zhou, Department of Colorectal Surgery, National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
World J Gastroenterol. 2017 Mar 28;23(12):2258-2265. doi: 10.3748/wjg.v23.i12.2258.
Clear-cell sarcoma is a rare, malignant soft tissue tumor that displays melanocytic differentiation with a distinct molecular profile. It is rarely localized in the gastrointestinal tract. Herein we reported a case of multiple synchronous clear-cell sarcomas of the gastrointestinal tract with parotid gland metastasis. A 51-year-old male patient presented with a growing painless mass under the right ear. A preoperative positron emission tomography/computed tomography showed multiple intestinal masses and a mass in the right parotid with increased glucose uptake, and he underwent operative treatment with resection of three tumors in the jejunum and ileum and then received a right parotidectomy. Postoperative pathological examination showed that cells in the intestinal tumor were consistent with clear-cell sarcoma of the gastrointestinal tract, and the malignant cells in the parotid gland were similar to the intestinal tumor. Immunohistochemical studies revealed positive expression of HMB-45, Melan-A, and S-100. EWSR1 gene fusion transcripts were undetectable by fluorescence hybridization.
透明细胞肉瘤是一种罕见的恶性软组织肿瘤，具有黑素细胞分化特征及独特的分子谱。它很少局限于胃肠道。在此我们报告一例胃肠道多发同步透明细胞肉瘤伴腮腺转移的病例。一名51岁男性患者因右耳下无痛性肿物逐渐增大就诊。术前正电子发射断层扫描/计算机断层扫描显示肠道多发肿物及右侧腮腺肿物，葡萄糖摄取增加，患者接受手术治疗，切除空肠和回肠的三个肿瘤，随后行右侧腮腺切除术。术后病理检查显示肠道肿瘤细胞符合胃肠道透明细胞肉瘤，腮腺中的恶性细胞与肠道肿瘤相似。免疫组织化学研究显示HMB-45、Melan-A和S-100呈阳性表达。荧光杂交未检测到EWSR1基因融合转录本。
