Rationale: Primary 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 concerns: We 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.
Diagnoses: The diagnosis was confirmed according to the specific anatomical location and pathological examination which was proved as EH.
Interventions: The patient underwent ureteroscopy and partial left nephrectomy.
Outcomes: Her 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.
Lessons: Renal 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.