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肉瘤样肾细胞癌:肾上皮恶性肿瘤最终的共同去分化途径。

Sarcomatoid renal carcinoma: the final common dedifferentiation pathway of renal epithelial malignancies.

作者信息

Delahunt B

机构信息

Department of Pathology, Wellington School of Medicine, University of Otago, New Zealand.

出版信息

Pathology. 1999 Aug;31(3):185-90. doi: 10.1080/003130299104945.

DOI:10.1080/003130299104945
PMID:10503259
Abstract

Sarcomatoid renal carcinoma (SRC) is an aggressive neoplasm with an age and gender distribution similar to that of conventional (clear cell) renal cell carcinoma (RCC). Genetic and morphologic evidence indicates that the tumor results from de-differentiation of renal epithelial malignancy and associations with RCC, papillary renal carcinoma, chromophobe renal carcinoma and collecting duct carcinoma have been reported. The tumor is composed of sheets of malignant spindle cells that have immunohistochemical and ultrastructural features of both stromal and epithelial cells, and may contain myxoid areas containing osteoclast-like giant cells or pleomorphic sarcomatoid spindle cells resembling rhabdomyoblasts. Rare cases of osteogenic SRC have been described. The tumor shows marked proliferative activity in growth kinetic studies and is usually associated with a poor patient survival that is best predicted by staging.

摘要

肉瘤样肾细胞癌(SRC)是一种侵袭性肿瘤,其年龄和性别分布与传统(透明细胞)肾细胞癌(RCC)相似。遗传学和形态学证据表明,该肿瘤是肾上皮恶性肿瘤去分化的结果,并且已报道其与RCC、乳头状肾细胞癌、嫌色性肾细胞癌及集合管癌有关。肿瘤由成片的恶性梭形细胞组成,这些细胞具有基质细胞和上皮细胞的免疫组化及超微结构特征,并且可能含有包含破骨细胞样巨细胞的黏液样区域或类似成肌母细胞的多形性肉瘤样梭形细胞。已有罕见的成骨性SRC病例的描述。在生长动力学研究中,该肿瘤显示出显著的增殖活性,并且通常与患者的不良生存相关,分期最能预测其预后。

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