Epithelioid angiosarcoma arising after an endovascular aneurysm repair: case report and review of the literature

Objectives: We report the case of a 68-year-old male who was hospitalized for suspicion of endoleak and thrombosis of an aortic endoprosthesis, arising after multiple endovascular interventions during the last few months. During the intervention, amass was discovered, and biopsies were made. The anatomopathology results were in favor of an epithelioid angiosarcoma with atypical expression of CD31 and ERG- and no amplification of c-MYC. The main objective of this review is to highlight the difficulty of differential diagnosis, but also to evaluate overall survival according to treatments. Methods: We performed a large review of the literature using PubMed for reports concerning angiosarcoma arising from Dacron grafts from 1981 to 2019. Articles presenting potentially relevant studies were read and analyzed. Results: In our review, most of the patients were male (10 cases over 11 described), with amedian age of 63 years old (50-84 years old, 11 cases described). The overall interval time for the diagnosis after the endoprosthesis placement was 7.8 years (from 3.5 years to 17 years, 10 cases with the description) and the overall survival was 5 months (from 0 to 10 months, the only patient alive is not included, and only 8 cases had the description). Conclusion: In most of the reviewed cases, there was no information concerning immunohistochemistry. Biopsies remain the standard for the diagnosis with immunochemistry and molecular test to avoid amisdiagnosis. Epithelioid angiosarcomas derived from Dacron grafts are a rare entity, which are difficult to diagnose because of the paucity of cases. Prognosis is poor, even if surgical option is possible.