Tawfik O W, Moral L A, Richardson W P, Lee K R
Department of Pathology and Oncology, University of Kansas Medical Center, Kansas City 66103.
Pediatr Pathol. 1993 May-Jun;13(3):289-98. doi: 10.3109/15513819309048215.
We report a unique case of multicentric bilateral renal cell carcinomas and a simultaneous large renal vascular leiomyoma in an 11-year-old child with sickle cell anemia. The patient presented with several episodes of massive hematuria. Abdominal sonography and computed tomography demonstrated bilateral renal neoplasms and the patient was clinically thought to have bilateral Wilms' tumor. An initial biopsy of the lower pole of right kidney revealed a renal cell carcinoma. Accordingly, bilateral renal angiography followed by right total nephrectomy and left upper pole partial nephrectomy were performed. Pathologic studies showed multicentric, bilateral renal cell carcinomas (two in the right kidney and one in the left kidney), of clear, granular, and oncocytic cell types. A simultaneous large vascular leiomyoma was also present in the right kidney. The smooth muscle nature of the leiomyoma was determined by light microscopy, immunohistology, and electron microscopy. The diagnostic difficulties in distinguishing them from other renal tumors are discussed.
我们报告了一例独特的病例，一名11岁患有镰状细胞贫血的儿童患有多中心双侧肾细胞癌及同时存在的巨大肾血管平滑肌瘤。该患者出现了几次大量血尿发作。腹部超声和计算机断层扫描显示双侧肾肿瘤，临床上认为该患者患有双侧威尔姆斯瘤。对右肾下极进行的首次活检显示为肾细胞癌。因此，进行了双侧肾血管造影，随后行右肾全切术和左肾上极部分切除术。病理研究显示多中心双侧肾细胞癌（右肾两个，左肾一个），有透明细胞型、颗粒细胞型和嗜酸性细胞型。右肾还同时存在一个巨大的血管平滑肌瘤。通过光学显微镜、免疫组织学和电子显微镜确定了平滑肌瘤的平滑肌性质。文中讨论了将它们与其他肾肿瘤区分开来的诊断难点。
