Ferry J A, Malt R A, Young R H
Department of Pathology, Harvard Medical School, Massachusetts General Hospital, Boston 02114.
Am J Surg Pathol. 1991 Nov;15(11):1083-8. doi: 10.1097/00000478-199111000-00008.
We report the case of a 49-year-old woman with a large renal angiomyolipoma that invaded the liver. In some areas, the tumor had the appearance of a typical angiomyolipoma; however, it also had foci where the spindle cells of the lesion showed marked cytologic atypia and mitotic activity, giving it the appearance of a high-grade sarcoma. Immunohistochemical studies demonstrated expression of vimentin, desmin, and muscle-specific actin by the sarcoma cells; these findings were consistent with leiomyosarcoma. A second, small typical angiomyolipoma was also present in the kidney. In addition, the liver exhibited focal nodular hyperplasia. Three weeks after resection of the primary renal tumor, pulmonary metastases were diagnosed by fine-needle aspiration biopsy. This is the first report of a case of angiomyolipoma with sarcomatous transformation and biopsy-proven metastatic disease.
我们报告了一例49岁女性，患有巨大肾血管平滑肌脂肪瘤并侵犯肝脏。在某些区域，肿瘤呈现典型血管平滑肌脂肪瘤的外观；然而，病变的梭形细胞也有灶性区域显示出明显的细胞学异型性和有丝分裂活性，使其看起来像高级别肉瘤。免疫组织化学研究显示肉瘤细胞表达波形蛋白、结蛋白和肌肉特异性肌动蛋白；这些发现与平滑肌肉瘤一致。肾脏中还存在一个较小的典型血管平滑肌脂肪瘤。此外，肝脏表现为局灶性结节性增生。原发性肾肿瘤切除三周后，通过细针穿刺活检诊断出肺转移。这是首例经活检证实的血管平滑肌脂肪瘤发生肉瘤样转化并伴有转移性疾病的病例报告。
