Intravascular Epithelioid Hemangiosarcoma of the Abdominal Aorta

Abstract

Introduction Angiosarcoma is a primary malignant tumor of vascular origin, accounting for 1–2% of all soft tissue sarcomas. Its occurrence in the aorta is extremely rare. Epithelioid angiosarcoma is a morphological variant of angiosarcoma, characterized by neoplastic endothelial cells with predominantly epithelioid features. Its incidence is extremely low, and it rarely originates in large arteries [1] . Case Presentation A 55-year-old man presented with complaints of foot pain for several days, burning sensation, itching. Ultrasound showed no blood flow in both legs below the knee. Computed tomography angiography (CTA) revealed that the infrarenal aortic lumen was irregularly thrombosed with extensive parietal thrombi (50%). Stenosis of the left renal artery (90%) and the right popliteal artery (70%) was discovered. A biopsy of aorta was performed. The patient underwent surgery, including resection of the abdominal aorta, aortobifemoral bypass grafting, left renal artery grafting, and thrombectomy of the right and left popliteal arteries. Blood circulation in the left leg was not restored, necessitating left leg amputation. Histological examination confirmed an intravascular epithelioid hemangiosarcoma. The patient was referred to an oncologist and prescribed chemotherapy consisting of 6 cycles of doxorubicin and ifosfamide. No tumor spread was observed on CT. After 2 months, due to obstructive thrombosis of the right popliteal artery, an amputation was performed. The patient was discharged for outpatient treatment. Discussion Primary aortic angiosarcoma is rare, and its preoperative diagnosis is highly challenging, often leading to a poor prognosis. The tumor may mimic an infected aortic aneurysm or a mural thrombus. This type of tumor typically develops without noticeable symptoms, with distal embolism often being the first indication. By the time it is identified, the tumor is usually extensive, and the prognosis remains poor [2,3] . Conclusions Clinical suspicion of angiosarcoma is vital for an early diagnosis and proper treatment.