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    The retroperitoneal neoplasm with perivascular epithelioid cel differentiation (PEComa) is an extremely rare path-ological entity. In this article, we reported one case of a 45-year-old woman who was admitted to our hospital (The Second People’s Hospital of Hefei, China) for retroperitoneal neoplasm with perivascular epithelioid cel dif erentiation. The B ultra-sonic examination showed echopoor in the region of cavitas pelvis. The histologic characteristics and immunohistochemical phenotype both revealed the neoplasm with perivascular epithelioid cel differentiation.

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