Carcinosarcoma of the stomach: four cases that expand the morphologic spectrum of gastric cancer with a primitive phenotype
Carcinosarcoma of the stomach: four cases that expand the morphologic spectrum of gastric cancer with a primitive phenotype
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DOI：
10.1007/s00428-022-03307-6
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发表时间：
2022-03-03
期刊：
影响因子：
3.5
通讯作者：
Ushiku，Tetsuo
中科院分区：
文献类型：
--
作者：
Yamazawa，Sho;Ushiku，Tetsuo
胃癌肉瘤是一种罕见的肿瘤，其特征是同时存在上皮性和间叶性恶性成分。我们描述了四个例子，重点描述了上皮成分的特征和这种独特肿瘤的组织发生学含义。所有患者都是40-79岁的男性。所有患者都发生了转移性疾病，其中3人在确诊后4-19个月死亡。肉瘤成分包括分化不明显的低分化梭形细胞肉瘤(n= 4)、软骨肉瘤(n= 2)和横纹肌肉瘤(n= 1)。在两个病例中，肉瘤成分仅在转移性病变中被识别。值得注意的是，肿瘤成分具有多谱系和原始细胞分化的特征，包括肠母细胞癌(n= 4)、肝样癌(n= 3)、卵黄囊瘤样癌(n= 1)、滋养细胞癌(n= 1)和神经内分泌分化(n= 1)，以及常规管状腺癌(n= 4)。免疫组织化学显示4例均有不同程度的原始表型标记物阳性表达，包括甲胎蛋白(AFP)、GLYPICA-3或SALL4。所有肿瘤均呈突变型P53染色，或呈弥漫性阳性，或完全阴性。根据这些发现，至少一些胃癌肉瘤可能来自原始表型的癌，包括产生AFP的腺癌。我们的观察表明，肉瘤分化以及上皮成分的多系分化可能代表了具有原始表型的胃癌细胞可塑性增加。
Carcinosarcoma of the stomach is a rare neoplasm characterized by the presence of both epithelial and mesenchymal malignant components. We describe four examples with a focus on the characterization of the epithelial components and the histogenetic implications for this unique tumor. All patients were men aged 40–79 years. All patients developed metastatic disease, and three of them died 4–19 months after the diagnosis. Sarcomatous elements included poorly differentiated spindle cell sarcoma without distinctive differentiation (n= 4), chondrosarcoma (n= 2), and rhabdomyosarcoma (n= 1). In two cases, the sarcomatous component was recognized only in metastatic lesions. Notably, carcinomatous components were characterized by multilineage and primitive cellular differentiation, including carcinoma with enteroblastic (n= 4), hepatoid (n= 3), yolk sac tumor-like (n= 1), trophoblastic (n= 1), and neuroendocrine (n= 1) differentiation, as well as conventional tubular adenocarcinoma (n= 4). On immunohistochemistry, all four cases showed varying degrees of positive expression of primitive phenotypic markers, including alpha-fetoprotein (AFP), glypican-3, or SALL4. All tumors showed mutant patterns of p53 staining, exhibiting either diffusely positive or completely negative staining. On the basis of these findings, at least some gastric carcinosarcomas are likely to be derived from carcinoma with a primitive phenotype, including AFP-producing adenocarcinoma. Our observations suggest that sarcomatous differentiation, as well as multilineage differentiation of epithelial components, may represent increased cellular plasticity of gastric carcinoma with a primitive phenotype.