ABSTRACT
INTRODUCTION: Epithelioid angiosarcoma is a rare soft tissue sarcoma with a poor prognosis. We report two cases of patients who presented with a history of lower back pain, inflammatory signs and weight loss 5 and 6 years after endovascular aortic repair (EVAR) of an elective infrarenal abdominal aortic aneurysm (AAA). Imaging suggested graft infection but tissue samples revealed an epithelioid angiosarcoma. The objective is to report the clinical presentation, investigative modalities and immunohistochemical findings of an angiosarcoma after EVAR. PATIENTS AND METHODS: Two cases are described of an angiosarcoma of the aorta after EVAR. A literature search using PubMed, Embase and Web of Science was performed in English about angiosarcoma after EVAR published between 2007 and 2021. Relevant reports were selected and analysed. RESULTS: Fifteen case reports were identified, including the current two cases. Time to tumour detection after EVAR ranged from 6 to 120 months with a mean interval of 68 months. Most patients underwent endovascular repair of an AAA (13/15). Males (13 male/2 female patients) were predominant with a median age of 72 years (IQR 68-78 years). Over half of the patients had metastases at the time of diagnosis (9/15), most frequently in bones and liver. CONCLUSION: Diagnosis of angiosarcoma after EVAR remains challenging due to indistinctive clinical and radiological findings mimicking graft infection or endoleak. Angiosarcoma should be included in the differential diagnosis in patients previously treated with EVAR presenting with unintended weight loss, abdominal back pain and contrast enhancement of the aortic wall.AbbreviationsAAAabdominal aortic aneurysmCTAcomputed tomography angiographyCRPc-reactive proteinEVARendovascular aortic repairESRerythrocyte sedimentation rateFDGfluoro-deoxyglucoseMRImagnetic resonance imagingMeSHmedical subject headings.
