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伴有骨骼肌分化的胸腺肉瘤样癌:2例报告,其中1例进行了细胞遗传学分析。

Thymic sarcomatoid carcinoma with skeletal muscle differentiation: report of two cases, one with cytogenetic analysis.

作者信息

Eimoto T, Kitaoka M, Ogawa H, Niwa H, Murase T, Tateyama H, Inagaki H, Soji T, Wang H J

机构信息

Department of Pathology, Nagoya City University School of Medicine, Kawasumi, Mizuho-ku, Nagoya 467-8601, Japan.

出版信息

Histopathology. 2002 Jan;40(1):46-57. doi: 10.1046/j.1365-2559.2002.01310.x.

Abstract

AIMS

Malignant thymic tumour histologically resembling a soft tissue sarcoma is extremely rare and defined as sarcomatoid carcinoma in the recent World Health Organization (WHO) classification. We report two such cases in which the tumour cells showed a prominent rhabdomyoblastic differentiation and analyse whether these tumours retain an epithelial nature at least in part.

METHODS AND RESULTS

One tumour occurred in a 51-year-old man (Case 1) and the other in a 40-year-old woman (Case 2). Microscopically, both tumours consisted essentially of two types of tumour cells: spindle and large round cells, with no apparent epithelial components. Osteosarcomatous small foci were also found in Case 2. Immunohistochemically, desmin and muscle-specific actin were positive in the majority of both types of tumour cells, whereas myogenin was predominant in the spindle cells and myoglobin in the large round cells. Some of both types of cells expressed cytokeratin with co-expression of myoglobin in the large round cells, but with no myogenin in the spindle cells. Some cytokeratin-positive spindle cells were also negative for desmin. Ultrastructural examination of a recurrent tumour in Case 2 revealed some epithelial features among the spindle cells. Cytogenetic study of the same tumour showed a complex abnormality including der(16)t(1;16)(q12;q12.1), an identical pattern previously reported in a case of thymic squamous cell carcinoma.

CONCLUSIONS

The findings support the definition in the WHO classification of sarcomatoid carcinoma that includes purely sarcomatous tumour as in the present cases. Occurrence of this type of tumour may indicate a relationship between thymic epithelial cells and myoid cells and/or a potential for divergent differentiation in thymic epithelial tumours.

摘要

目的

组织学上类似于软组织肉瘤的恶性胸腺肿瘤极为罕见,在世界卫生组织(WHO)最新分类中被定义为肉瘤样癌。我们报告两例此类病例,其中肿瘤细胞显示出显著的横纹肌母细胞分化,并分析这些肿瘤是否至少部分保留上皮性质。

方法与结果

一例肿瘤发生于一名51岁男性(病例1),另一例发生于一名40岁女性(病例2)。显微镜下,两例肿瘤均主要由两种类型的肿瘤细胞组成:梭形细胞和大圆形细胞,无明显上皮成分。病例2中还发现了骨肉瘤样小病灶。免疫组织化学检查显示,两种类型的肿瘤细胞多数中结蛋白和肌特异性肌动蛋白呈阳性,而肌生成素在梭形细胞中占主导,肌红蛋白在大圆形细胞中占主导。两种类型的部分细胞均表达细胞角蛋白,大圆形细胞中细胞角蛋白与肌红蛋白共表达,但梭形细胞中无肌生成素表达。部分细胞角蛋白阳性的梭形细胞结蛋白也呈阴性。病例2复发性肿瘤的超微结构检查显示梭形细胞中有一些上皮特征。同一肿瘤的细胞遗传学研究显示存在复杂异常,包括der(16)t(1;16)(q12;q12.1),这与先前报道的一例胸腺鳞状细胞癌的模式相同。

结论

这些发现支持WHO分类中肉瘤样癌的定义,其中包括如本病例中的纯肉瘤性肿瘤。此类肿瘤的发生可能表明胸腺上皮细胞与肌样细胞之间的关系和/或胸腺上皮肿瘤中存在分化潜能。

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