Multicentric Aggressive Angiomyolipomas: A Rare Form of PEComas

4School of Medicine, China Medical University, Taichung, Taiwan. eoplasms with perivascular epithelioid cell differentiation (PEComas) are mesenchymal tumors composed of distinctive perivascular epithelioid cells [1]. PEComas can be found in various organs [2]. Angiomyolipoma (AML) is one of the PEComa family, which is benign in entity and the most common mesenchymal tumor of the kidney [1, 2]. Malignant AML is extremely rare and most of them are found to be epithelioid AML histopathologically [1, 3]. Imaging features of multifoci AMLs have rarely been reported in the literature [3–5]. We describe the case of a 60-year-old woman with a huge left renal AML who subsequently developed multiple malignant AMLs in the lungs, lymph nodes, mediastinum, and cervical and thoracic spine. The imaging and pathologic features are presented. The pathogenesis of multicentric AMLs is discussed.