Primary carcinosarcoma of the ureteropelvic junction associated with ureteral duplication
Primary carcinosarcoma of the ureteropelvic junction associated with ureteral duplication
复制标题
与输尿管重复相关的原发性肾盂输尿管连接部癌肉瘤
DOI：
10.1097/md.0000000000016643
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发表时间：
2019
期刊：
影响因子：
1.6
通讯作者：
Oshiro Hisashi
中科院分区：
文献类型：
--
作者：
Tsuji Kentaro;Ito Atsushi;Kurokawa Shinsuke;Nakaya Takeo;Yoshimoto Taichiro;Kawata Hirotoshi;Tamba-Sakaguchi Mio;Fukushima Noriyoshi;Oshiro Hisashi
干预：患者接受了右肾输尿管切除术。切除肿瘤的病理检查显示一个由癌性和肉瘤性成分组成的双相肿瘤。肉瘤成分的波形蛋白、结蛋白、h-钙调蛋白和α-SMA免疫化学染色呈阳性，而泛细胞角蛋白（AE 1/AE 3）、低分子量细胞角蛋白（CAM 5.2）、EMA、E-钙粘蛋白、GATA 3、尿斑蛋白2和p63呈阴性。根据这些发现，我们诊断为癌肉瘤。结果：术后过程是顺利的。未给予其他治疗。病人自手术后至今已存活21个月，无复发。教训：重复输尿管可引起癌肉瘤。虽然大多数上尿路癌在晚期才被诊断出来，预后很差，但有些肿瘤的侵袭性较低。需要进一步的研究来确定输尿管重复畸形与恶性肿瘤之间的关系。
Interventions:The patient underwent right nephroureterectomy. Pathological examination of the resected tumor showed a biphasic neoplasm composed of carcinomatous and sarcomatous components. The sarcomatous component was immunohistochemically positive for vimentin, desmin, h-caldesmon, and α-SMA and negative for pancytokeratin (AE1/AE3), low molecular weight cytokeratin (CAM 5.2), EMA, E-cadherin, GATA3, uroplakin 2, and p63. Based on these findings, we diagnosed the tumor as carcinosarcoma.Outcomes:The postoperative course was uneventful. No additional therapy was administered. The patient has remained alive without recurrence for 21 months since surgery.Lessons:Carcinosarcoma can arise from ureteral duplication. Although the majority of carcinosarcomas of the upper urinary tract are diagnosed at an advanced stage and have a poor prognosis, some can have a less aggressive course. Further studies are needed to determine the association between ureteral duplication and malignancy.