目的

探讨原发性肾血管肉瘤（PRA）的临床表现、诊治及预后。

方法

回顾性分析1 例原发性肾血管肉瘤并出血、全身多处转移患者的临床资料，结合文献复习，对该病进行讨论。

结果

本例患者因“突发左侧腰腹部胀痛5 h”入院，CT 提示左肾肿瘤并出血、全身多处转移，该患者在全麻下行开放左肾姑息性切除术，病理结果证实为左肾血管肉瘤，术后予以化疗、靶向及免疫等全身综合治疗，治疗中患者病情稳定，3 个疗程后患者因经济原因放弃继续治疗，术后1 年死亡。

结论

原发性肾血管肉瘤临床罕见，侵袭性强，预后差，确诊主要依据病理，治疗首选根治性肾切除术，术后应辅以化疗、放疗。靶向及免疫治疗或许是PRA 的有效治疗方法。

Objective

To investigate the clinical characteristics，treatment and prognosis of primary renal angiosarcoma （PRA）.

Methods

One case of primary renal angiosarcoma complicated with hemorrhage and multiple systemic metastases was retrospectively analysed and relevant literatures were reviewed.

Results

The patient was admitted to our hospital for “acute left abdominal pain for 5 hours”，CT revealed a tumor in his left kidney complicated with hemorrhage，palliative left nephrectomy was performed and pathologic diagnosis confirmed renal angiosarcoma，though chemotherapy，targeted therapy and immunotherapy were carried out.The patient's condition was stable during treatment.After 3 courses of comprehensive treatment，the patient gave up for economic reasons，and dead one-year after operation.

Conclusions

Primary renal angiosarcoma is an exceedingly rare and highly aggressive neoplasm with a poor prognosis，the final diagnosis depends on pathology.Once diagnosed，radical nephrectomy should be performed early，chemotherapy and radiotherapy should be followed after surgery，targeted therapy and immunotherapy may be effective treatments for PRA.