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由鳞状细胞癌成分和胚胎性横纹肌肉瘤成分组成的胸腺癌肉瘤。1例报告并文献复习。

Thymic carcinosarcoma consisting of squamous cell carcinomatous and embryonal rhabdomyosarcomatous components. Report of a case and review of the literature.

作者信息

Okudela K, Nakamura N, Sano J, Ito T, Kitamura H

机构信息

Department of Pathology, Yokohama City University School of Medicine, Yokohama, Japan.

出版信息

Pathol Res Pract. 2001;197(3):205-10. doi: 10.1078/0344-0338-00035.

DOI:10.1078/0344-0338-00035
PMID:11314786
Abstract

A case of thymic carcinosarcoma in an 83-year-old Japanese man is presented. He died of superior vena cava syndrome caused by a rapidly enlarged anterior mediastinum tumor eight months after initial symptoms. Autopsy revealed a 16 x 12 x 25 cm-sized, tan yellow, whitish tumor with a multinodular and microcystic appearance located in the left anterior mediastinum, which involved the residual thymus. The tumor had directly invaded the left pleura, and had metastasized to the right lung and spleen. Histologic examinations of the primary tumor showed a sarcomatous component consisting of racquet- or spindle-shaped cells with cross striations, and small nests of atypical squamous cells scattered throughout the tumor; neither transition between the two components nor intermediate cells with both epithelial and mesenchymal features was seen. Electron microscopic and immunohistochemical examinations confirmed the rhabdomyomatous differentiation of the sarcomatoid component. To our knowledge, there have been only two reported cases showing histologic features similar to the present tumor. For the histogenesis of thymic carcinosarcoma, we propose two hypotheses. The first is that sarcomatous cells are derived from carcinomatous cells by tumoral metaplasia. Secondly, that this type of tumor originates from thymic primitive cells with multidirectional differentiation potential. In accordance with the latter, we consider that the present tumor originated from thymic primitive cells. Thymic carcinosarcoma is a highly malignant tumor, and most patients die within a year. Appropriate therapies must be developed.

摘要

本文报告了一例83岁日本男性的胸腺癌肉瘤病例。患者在出现初始症状8个月后,因前纵隔肿瘤迅速增大导致上腔静脉综合征死亡。尸检发现左前纵隔有一个大小为16×12×25 cm的黄褐色、灰白色肿瘤,呈多结节和微囊状外观,累及残余胸腺。肿瘤直接侵犯左胸膜,并转移至右肺和脾脏。原发肿瘤的组织学检查显示,肉瘤成分由具有横纹的球拍状或梭形细胞组成,肿瘤内散在分布着小巢状非典型鳞状细胞;未观察到两种成分之间的过渡,也未发现具有上皮和间充质特征的中间细胞。电子显微镜和免疫组织化学检查证实了肉瘤样成分的横纹肌样分化。据我们所知,仅有两例报告病例显示出与本肿瘤相似的组织学特征。对于胸腺癌肉瘤的组织发生,我们提出两种假说。第一种假说是肉瘤细胞由癌细胞通过肿瘤化生而来。第二种假说是这种类型的肿瘤起源于具有多向分化潜能的胸腺原始细胞。基于后者,我们认为本肿瘤起源于胸腺原始细胞。胸腺癌肉瘤是一种高度恶性肿瘤,大多数患者在一年内死亡。必须开发适当的治疗方法。

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