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Abstract
Background: Perivasuclar epithelioid cell tumor (PEComa) is a rare group of mesenchymal tumors with unique histological and immunophenotypes. Retroperitoneal PEComa is extremely rare and almost benign lesion. Herein, we will present a case from China, focusing on histological and immunohistochemical characteristics of the tumor, follow-up and long-term outcome and the related literature review.
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