Abstract:Objective To discuss the clinical and pathological characteristics of primary splenic angiosarcoma (PSA). Methods A retrospective analysis was performed on the clinical data,histomorphology and immunohistochemical staining results of one patient admitted to 2016-12-29 confirmed as PSA with postoperative liver metastasis.Using the terms "hematosarcoma","spleen" and "primary" as the search terms,search the Medline/PubMed database from January 2015 to July 2019. Results A 79-year-old male patient presented with left upper quadrant abdominal pain.CT scans revealed an enlarged spleen with heterogeneous enhancing hypodense lesions diffusely distributed in spleen parenchyma.Histopathology examination after splenectomy revealed an angiogenic malignant neoplasm,and immunohistochemistry showed that the tumor cells were positive for CD31,CD34,ERG,CD8 and negative for CD68,CD163,SMA,so the patient was diagnosed as primary splenic angiosarcoma.At 9 months after operation,multiple liver metastases foci were found by CT scanning.Finally,the patient succumbed to the pneumonia and respiratory failure with a survival of 10 months.A total of 20 foreign literature reports were retrieved in 20 patients,6 males and 14 females,aged 13 to 83 years.The shortest survival time after splenectomy was 2 weeks,and the longest was followed up for 13 years. Conclusions PSA is an extremely rare and mesenchymal malignant tumor of vascular origin often with a poor prognosis and high metastatic rate.The best chance for survival follows an early diagnosis and a prompt splenectomy prior to splenic rupture.