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目的：分析不典型肾脏血管平滑肌脂肪瘤的ＣＴ表现及其病理基础，以提高对该病的认识。方法：１２例经ＣＴ扫描平扫和（或）增强扫描。结果：该瘤的不典型ＣＴ特点为（１）肿瘤内缺乏脂肪成分；（２）肿瘤位于特殊部位；（３）并发明显肾周血肿；（４）伴腹膜后淋巴结肿大。不典型肾脏血管平滑肌脂肪瘤虽有各种各样的表现，但１２例中９例在病灶内仍可发现少量脂肪成分。结论：无论不典型肾血管平滑肌脂肪瘤的ＣＴ表现如何，发现肿瘤内脂肪仍是该病诊断的关键。
Objective: To analyze the CT manifestation and pathological basis of atypical renal angiomyolipoma in order to improve the understanding of the disease. METHODS: Twelve patients underwent plain and/or enhanced CT scans. RESULTS: The atypical CT characteristics of this tumor were (1) the lack of fat in the tumor; (2) the tumor was located at a specific site; (3) was invented to show a hepatic hematoma; and (4) with retroperitoneal lymphadenopathy. Although atypical renal angiomyolipoma has a variety of manifestations, 9 of 12 cases still have a small amount of fat found in the lesion. CONCLUSIONS: Regardless of the CT appearance of atypical renal angiomyolipoma, the finding of intratumoral fat is still the key to the diagnosis of this disease.