Abstract:

Primary renal angiosarcoma （RA） is clinically rare and highly aggressive， and is easily misdiagnosed at the early stage. A 70-year-old female patient admitted to our hospital with sudden severe pain in the right lower abdomen for 10 h was reported. Contrast-enhanced whole abdominal CT showed right renal rupture with active retroperitoneal hemorrhage， and emergency right nephrectomy was performed. Postoperative pathological examination confirmed moderately differentiated RA； immunohistochemical staining results showed that CD31， CD34， and ETS-related gene protein （ERG） were diffusely strongly positive， and the Ki-67 index was 60%. Within one month after surgery， the patient experienced two sudden episodes of massive abdominal hemorrhage （>1 000 mL）， which were controlled by embolization of the right hepatic artery and adrenal artery， respectively. Subsequent treatments including cisplatin chemotherapy and disitamab vedotin combined with toripalimab were attempted， but liver metastasis still occurred after 7 weeks. The patient died 3 months after diagnosis. When elderly patients present with spontaneous renal rupture complicated by retroperitoneal hemorrhage， RA should be included in the differential diagnosis. Arterial embolization can be used for emergency hemostasis but is difficult to curb tumor progression， while current chemotherapy and immunotherapy regimens have limited efficacies， and there is an urgent need to explore targeted combination therapy.

Key words: Renal angiosarcoma, Primary renal malignancy tumor, Abdominal hemorrhage, Nephrectomy, Immunotherapy