Angiosarcoma of the abdominal aorta after previous endovascular aneurysm repair.

Primary aortic angiosarcoma is a rare and aggressive malignancy arising from endothelial cells of blood vessels and has a poor prognosis. We report 21 relevant cases from the literature, summarizing the number of reported cases without analyzing treatment outcomes or prognostic data. Additionally, we report our own case of a 75-year-old male who presented with fever, abdominal pain, and leg discomfort 10 years after endovascular aneurysm repair (EVAR) for an abdominal aortic aneurysm. Initial imaging suggested an infected EVAR, leading to explantation and reconstruction. Despite antibiotic treatment, negative cultures and surgical intervention, the patient's condition continued to deteriorate. A biopsy of cervical lymph nodes revealed a high-grade angiosarcoma. These cases highlight the diagnostic challenges of primary aortic angiosarcoma, especially in patients with prior EVAR, and emphasize the importance of considering rare malignancies in cases when a patient deteriorates despite therapy or does not show the expected treatment response.