Intimal angiosarcoma of the descending aorta presenting as atheroembolism: case report and description of intravascular biopsy

Aortic sarcoma is a rare entity. In most cases, the diagnosis is established late, owing to the course of the disease, with a median survival time of only a few months. We report the case of a 58-year-old patient with ischemic lesions in the lower limb. The lesions after several investigations were diagnosed by imaging studies (eg, magnetic resonance angiography, contrast-enhanced computed tomography [CT], CT angiography, or positron emission tomography-CT with fluorine-18 fluorodeoxyglucose) as possible primary angiosarcoma of the aorta. It was decided to perform endovascular aortic repair and endovascular biopsy of the lesion. This was chosen as a “palliative” treatment to avoid embolic events, given the presence of metastases, instead of open surgery, which would otherwise have been the treatment of choice. Subsequently, histologic examination confirmed aortic intimal angiosarcoma, and adjuvant chemotherapy was initiated. In the present report, we discuss the clinical presentation, diagnosis, and classification of primary aortic sarcomas. We also critically review the diagnostic and therapeutic management of these patients in previous series of studies to improve their treatment in subsequent cases.