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伴有大量骨肉瘤样异向分化的去分化嫌色肾细胞癌:腹膜后钙化性肿瘤谱系中的一种独特实体。

Dedifferentiated chromophobe renal cell carcinoma with massive osteosarcoma-like divergent differentiation: a singular entity in the spectrum of retroperitoneal calcifying tumors.

作者信息

Gira Federica A, Barbieri Antonio, Fellegara Giovanni, Zompatori Maurizio, Corradi Domenico

机构信息

Department of Clinical Sciences, University of Parma, Parma, Italy.

出版信息

Int J Surg Pathol. 2010 Oct;18(5):419-23. doi: 10.1177/1066896908329414. Epub 2008 Dec 18.

Abstract

Sarcomatoid change in renal cell carcinoma is the result of the dedifferentiation of the "parent" tumor into a high-grade malignancy characterized by sarcoma-like features and associated with an accelerated clinical course and poorer prognosis. Any of the renal cell carcinoma subtypes can undergo sarcomatoid dedifferentiation, with the chromophobe variant being the most prone. The present report describes the case of a woman affected by a classic chromophobe renal cell carcinoma that developed dedifferentiation accompanied by a very rare osteosarcoma-like divergent differentiation, which constituted about 90% of the entire retroperitoneal mass. In addition to presenting the relevant imaging, histopathological, and immunohistochemical findings, this article briefly discusses the main differential diagnosis of retroperitoneal ossifying/ calcifying masses, which could give rise to diagnostic problems either in small biopsies or at imaging.

摘要

肾细胞癌中的肉瘤样改变是“母”肿瘤去分化为具有肉瘤样特征的高级别恶性肿瘤的结果,其临床病程加速且预后较差。任何肾细胞癌亚型都可发生肉瘤样去分化,其中嫌色细胞亚型最易发生。本报告描述了一例患有经典嫌色性肾细胞癌的女性病例,该肿瘤发生了去分化,并伴有非常罕见的骨肉瘤样异向分化,这种异向分化构成了整个腹膜后肿块的约90%。除了展示相关的影像学、组织病理学和免疫组化结果外,本文还简要讨论了腹膜后骨化/钙化肿块的主要鉴别诊断,这些肿块在小活检或影像学检查中都可能引发诊断问题。

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