Wang L, Tan C, Ni S J, Jiang W H, Xu J, Cai X, Huang D, Sheng W Q, Chang B
Department of Pathology, Fudan University Shanghai Cancer Center; Department of Oncology, Shanghai Medical School, Fudan University, Shanghai 200032, China.
Zhonghua Bing Li Xue Za Zhi. 2021 Jun 8;50(6):632-637. doi: 10.3760/cma.j.cn112151-20201224-00963.
To investigate the clinicopathological features, immunohistochemical characteristics, differential diagnosis and prognosis of gastric SWI/SNF-complex deficient undifferentiated/rhabdoid carcinomas. Two cases of gastric SWI/SNF-complex deficient undifferentiated/rhabdoid carcinoma were collected at Fudan University Shanghai Cancer Center, Shanghai, China from 2017 to 2018. The clinicopathological characteristics were analyzed. Hematoxylin and eosin, and immunohistochemical stains were performed, and the relevant literatures were reviewed. The two patients were both male, aged 60 and 74 years, respectively. Their symptoms were both abdominal pain. The tumor arose in the esophagogastric junction in case 1, and the cardia to the fundus and the posterior wall of the upper part of gastric body in case 2. Both tumors were present as an ulcerative mass. The patients died of tumor 11 months and 8 months after surgery, respectively. Histologically, the tumor cells arranged in sheets, nests, cords or trabecular patterns, and pseudoavleolar structure. The tumor cells were epithelioid with uniform morphology, while the tumors showed scant stroma and massive necrosis. Variable rhabdoid cells and multinucleated giant cells were seen in both cases. SMARCA4 encoding protein BRG1 was undetectable in both tumors, while SMARCB1 encoding protein INI1 was detected. The tumor cells were diffusely positive for vimentin and negative for epithelial marker (CKpan), gastrointestinal stromal tumor markers (CD117 and DOG1), myogenic markers (desmin and myogenin), melanoma markers (S-100 protein, SOX10 and HMB45), and lymphohematopoietic markers (LCA and CD20). Gastric SWI/SNF-complex deficient undifferentiated/rhabdoid carcinoma is a rare and highly aggressive tumor with poor prognosis. The detection of subunits protein expression of SWI/SNF complex is important for diagnosis of the tumor.
探讨胃SWI/SNF复合物缺陷型未分化/横纹肌样癌的临床病理特征、免疫组化特点、鉴别诊断及预后。2017年至2018年在中国上海复旦大学附属肿瘤医院收集2例胃SWI/SNF复合物缺陷型未分化/横纹肌样癌病例。分析其临床病理特征。进行苏木精-伊红染色及免疫组化染色,并复习相关文献。2例患者均为男性,年龄分别为60岁和74岁。症状均为腹痛。病例1肿瘤位于食管胃交界部,病例2肿瘤位于贲门至胃底及胃体上部后壁。两例肿瘤均表现为溃疡性肿块。患者分别于术后11个月和8个月死于肿瘤。组织学上,肿瘤细胞呈片状、巢状、条索状或小梁状排列,并可见假腺泡结构。肿瘤细胞呈上皮样,形态一致,肿瘤间质少,大片坏死。两例均可见不同程度的横纹肌样细胞和多核巨细胞。两例肿瘤中均未检测到编码蛋白BRG1的SMARCA4,而检测到编码蛋白INI1的SMARCB1。肿瘤细胞波形蛋白弥漫阳性,上皮标志物(CKpan)、胃肠道间质瘤标志物(CD117和DOG1)、肌源性标志物(结蛋白和肌细胞生成素)、黑色素瘤标志物(S-100蛋白、SOX10和HMB45)及淋巴造血标志物(LCA和CD20)均阴性。胃SWI/SNF复合物缺陷型未分化/横纹肌样癌是一种罕见且侵袭性强、预后差的肿瘤。检测SWI/SNF复合物亚基蛋白表达对该肿瘤的诊断有重要意义。