We report two patients with extrarenal retroperitoneal
angiomyolipoma masquerading as perinephric
liposarcoma. Patient 1 : A 66-year-old man was diagnosed with a retroperitoneal
tumor near the right renal hilum on an abdominal computed tomography (CT) performed before surgery for
gastric cancer. A diagnosis of extrarenal retroperitoneal
angiomyolipoma was made on the basis of negative uptake of
fluorine- 18 2-deoxy-2-fluoro-D-glucose positron emission tomography (18F-FDG PET)/CT. However, because the
tumor was found to have gradually enlarged at 18 months afterward, he underwent resection of the extrarenal fat tissue together with the right kidney. Patient 2 : A 56-year-old man underwent abdominal ultrasound during a periodic medical examination, which revealed a right retroperitoneal
tumor. Because of the findings in the contrast-enhanced CT and positive uptake of
18F-FDG PET/CT, he underwent resection of the extrarenal fat tissue together with the right kidney. The pathological examination of the two
tumors confirmed extrarenal
angiomyolipoma. The differential diagnosis of extrarenal retroperitoneal
angiomyolipoma from
retroperitoneal liposarcoma is difficult even with the use of
18F-FDG PET/CT.