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

Abstract

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.

Keywords: Aorta; Endovascular aortic repair; Intimal angiosarcoma; Malignant aortic tumor.

Fig 1

Computed tomography angiographic images showing, in the descending aorta, prominent, irregular, vegetating, dense soft tissue repletion defects (30-40 HU; marked in red), extending from L8 to L11 (62 mm long), and seated in the posterior and external wall of the aorta.

Fig 2

Magnetic resonance angiographic images showing a voluminous filling defect in the descending thoracic aorta dependent on the posterior aortic wall, with irregular contours and a low resonance signal, without significant enhancement and causing stenosis of >50% of the cross-sectional area (marked in red).

Fig 3

A, cTAG (W.L. Gore & Associates) 34 × 34 × 150-mm stent implant (arrow). B, Sweeping performed with a 6F Fogarty catheter over the 12F sheath, drawing tumor material with a whitish aspect into the sheath (circle). C, Completion angiography (bracket).