• Yuhui Liu, Ailian Liu, Jingjun Wu, and Tieli Liu.
• College of Medical Imaging, Dalian Medical University.
• Medicine (Baltimore). 2019 Aug 1; 98 (34): e16537e16537.

RationalePrimary renal epithelioid hemangioendothelioma (EH) is a rare vascular tumor with intermediate biologic behavior and metastatic potential, and it is extremely rare and has only 4 cases in the current literatures.Patient ConcernsWe reported a 30-year-old woman who had a 3-month history of gross hematuria and aggravated for half a month. The imaging examination showed a cystic lesion in the mid pole of the left kidney pelvicaliceal.DiagnosesThe diagnosis was confirmed according to the specific anatomical location and pathological examination which was proved as EH.InterventionsThe patient underwent ureteroscopy and partial left nephrectomy.OutcomesHer postoperative condition was good without complications. No clinical evidence of local recurrence or metastatic disease was found during 6 months of follow-up clinical and ultrasound examinations. In addition, laboratory tests, including a urine examination, were normal.LessonsRenal EH is a rare low-grade malignant tumor with characteristic histological structure. Locally excision has been considered as the optimal treatment and regular follow-up is necessary. Our present study reviewed the clinical and biological information of previous cases which were diagnosed as renal EH and we supplemented more data for further study.

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