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结肠恶性横纹肌样瘤:一例报告

Malignant rhabdoid tumor of the colon: report of a case.

作者信息

Nakamura I, Nakano K, Nakayama K, Ishii Y, Ohta K, Takahashi M, Yamada T, Yamaguchi K, Sakuma H, Tsuchiya A, Takenoshita S

机构信息

Department of Surgery II, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima 960-1295, Japan.

出版信息

Surg Today. 1999;29(10):1083-7. doi: 10.1007/s005950050649.

Abstract

A malignant rhabdoid tumor of the colon is very rare and only three cases have been previously described. A 76-year-old man was admitted to the hospital complaining of epigastralgia. An elastic mass was palpable in the right upper abdomen. A barium enema and endoscopic examination showed a giant gyrate tumor arising from the cecum. Abdominal ultrasonography and a computed tomography scan revealed the tumor to be located in the colon associated with multiple liver metastases and gallbladder stones. A right colectomy and cholecystectomy were thus performed. The tumor was histologically composed of sheets of large round and polygonal nuclei with vesicular chromatin, and abundant acidophilic cytoplasm, often containing hyalin-like inclusion. The cytoplasm was positive for vimentin and neuron-specific enolase, and hyaline globules of the rhabdoid tumor cells stained positive for cytokeratin in some cells. Transmission electron microscopy showed characteristic rhabdoid cells with an aggregation of intermediate filaments. A histologic diagnosis of malignant rhabdoid tumor of the colon was made. The tumor demonstrated several unusual findings for malignant rhabdoid tumors including diploidy by a flow cytometric analysis, and positive nuclear immunohistochemical staining for p53 protein and Ki-67 antigen. We report herein the third known case of a pure colonic rhabdoid tumor.

摘要

结肠恶性横纹肌样瘤非常罕见,此前仅报道过3例。一名76岁男性因上腹部疼痛入院。右上腹可触及一个质地较硬的肿块。钡剂灌肠和内镜检查显示一个巨大的回旋状肿瘤起源于盲肠。腹部超声和计算机断层扫描显示肿瘤位于结肠,伴有多发肝转移和胆囊结石。因此实施了右半结肠切除术和胆囊切除术。肿瘤组织学表现为大片具有泡状染色质的大圆形和多边形细胞核,以及丰富的嗜酸性细胞质,常含有透明样包涵体。细胞质波形蛋白和神经元特异性烯醇化酶呈阳性,横纹肌样瘤细胞的透明小球在部分细胞中细胞角蛋白染色呈阳性。透射电子显微镜显示具有中间丝聚集的特征性横纹肌样细胞。作出了结肠恶性横纹肌样瘤的组织学诊断。该肿瘤表现出一些恶性横纹肌样瘤的异常特征,包括流式细胞术分析显示为二倍体,以及p53蛋白和Ki-67抗原的细胞核免疫组化染色呈阳性。我们在此报告第三例已知的单纯结肠横纹肌样瘤病例。

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