一种独特的胰腺导管腺癌,具有癌肉瘤的组织学、hcg - β的免疫组织化学分布和血清甲胎蛋白的升高。

K Yamazaki
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摘要

具有肿瘤间质成分的胰腺未分化癌(癌肉瘤)迄今尚未得到很好的描述。作者经历了一个独特的胰腺导管腺癌与癌肉瘤组织学解剖病例。患者是一名90岁的日本男性,死于肿瘤广泛性扩展的恶质。尸检显示在肿瘤组织中发现了一些独特的或有代表性的成分。一种是分化良好的导管腺癌。第二个也是主要的发现是未分化的短梭形或小圆形肉瘤细胞,缺乏上皮性质,但CD10+, CD56+, ki67++, p53++呈阳性,Desmin和vimentin呈局部阳性。这两种成分混合在一起,构成了癌肉瘤的组织学。在另一个区域,间变性的、大的、多形性的肿瘤细胞显示甲胎蛋白和人绒毛膜促性腺激素的局灶性免疫组织化学分布。超微结构研究显示腺癌细胞具有顶端分泌粘蛋白颗粒和发达的导管分化,而未分化的肉瘤细胞表现为原始的成纤维细胞或间充质细胞特征,没有特异性分化。总之,这些结果提示,这种分化良好的腺癌逐渐扩大,积累了遗传改变,然后转化为大而未分化的肿瘤细胞,快速生长的小肉瘤细胞,组织学上为癌肉瘤。
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A unique pancreatic ductal adenocarcinoma with carcinosarcomatous histology, immunohistochemical distribution of hCG-beta, and the elevation of serum alpha-feto-protein.

Pancreatic undifferentiated carcinomas with a neoplastic mesenchymal component (carcinosarcoma) have not been well described to date. The author experienced an autopsy case of a unique pancreatic ductal adenocarcinoma with carcinosarcomatous histology. The patient was a 90 year old Japanese male who died of cahexia with generalized tumor extension. Post-mortem examinations revealed some distinctive or representative components discerned in the tumor tissue. One was the well differentiated ductal adenocarcinoma. The second and the major finding was undifferentiated short spindle shaped or small round sarcomatous cells, which lacked an epithelial nature but showed positivity for CD10+, CD56+, Ki67++, p53++, and were focally positive for Desmin and vimentin. These two components were mixed and constituted the histology of the carcinosarcoma. In another area, anaplastic, large, pleomorphic tumor cells showed the focal immunohistochemical distribution of alpha-feto-protein and human chorionic gonadotropin. An ultrastructural study revealed adenocarcinoma cells with apical mucin secreting granules and well developed ductal differentiation, whereas undifferentiated sarcomatous cells showed primitive fibroblastic or mesenchymal characters without specific differentiation. Conclusively these findings suggested that this well differentiated adenocarcinoma gradually enlarged, accumulated genetic alternations, and then transformed into large and undifferentiated tumor cells, rapidly growing small sarcomatous cells, and a histology of carcinosarcoma.

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