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作者报道第一例来自肾周筋膜的肾外血管肌脂瘤,其导致自发性肾周出血。血管肌脂瘤是由成熟脂肪、血管和平滑肌组成的错构瘤,常出现于肾脏。50～80％的结节性硬化患者伴有肾血管肌脂瘤。在一组8,501例肾肿瘤的报道中,仅有27例血管肌脂瘤(0.3％)。肾血管肌脂瘤多为良性,但最近报道1例复发性血管肌脂瘤侵及左侧结肠、膈肌和腹主动脉外膜。尚有2例血管肌脂瘤具有明显侵润性,1例切除术后局部复发。上述4例的病理检查均见明显增加的分裂数目、非典型细胞及细胞多形性。肾外血管肌脂瘤很罕见,迄今报道24例。12例位于肝脏,均为良性,6例经尸检发现,6例于术中发现,仅3例有症状。7例肿瘤位于子宫,均为良性,症状与子宫纤维病相同。1例见于硬腭,1
The authors report the first case of extrarenal vascular myoma of the perirenal fascia, which leads to spontaneous perirenal hemorrhage. An angiomyolipoma is a hamartoma composed of mature fats, blood vessels, and smooth muscle, often present in the kidney. 50 to 80% of patients with tuberous sclerosis have renal angiomyolipoma. In a group of 8,501 renal tumor reports, there were only 27 angiomyolipomas (0.3%). Renal angiomyolipoma is mostly benign, but recently it has been reported that one case of recurrent angiomyolipoma invades the adventitia of the left colon, diaphragm and abdominal aorta. There were 2 cases of angiomyolipoma with obvious invasiveness, and 1 case had local recurrence after resection. The pathological examination of the above 4 cases all showed significantly increased number of divisions, atypical cells and cell polymorphism. Extrarenal vascular myoma is rare and reported so far in 24 cases. Twelve cases were located in the liver and all were benign. Six cases were found by autopsy. Six cases were found during surgery. Only three cases had symptoms. Seven tumors were located in the uterus and were all benign. The symptoms were the same as those of uterine fibrosis. One case was found in hard palate, 1