Primary angiosarcoma of inferior vena cava extending to right atrium: a case report

Primary angiosarcomas of the inferior vena cava are exceedingly rare. We report a case of a 32-year-old Chinese female, who presented with progressive debilitation and dyspnea. Color Doppler vascular ultrasonography showed a solid irregular space-occupying lesion in the right atrium. Postoperative gross observation indicated that the tumor infiltrated and surrounded the inferior vena cava, also extended into the pericardium, right ventricle and cardiac facies diaphragmatica. Furthermore, histology revealed that the tumor had a scattered spindle-cell morphology, and immunohistochemistry for CD31, CD34, and Vimentin was positive for the tumor cells. Additionally, MDM2 amplification was absent as detected by FISH, which supported the diagnosis of angiosarcoma. The patient was treated with AI regimen (doxorubicin + ifosfamide) in combination with Endostatin for six cycles. She achieved complete response in the ninth month post operation.