Pleomorphic undifferentiated aortic sarcoma presenting as persistent endoleak after endovascular aneurysm repair

Abstract

Sarcomas of the aorta are rare tumors with an unknown incidence and wide variety of clinical presentations. These malignant neoplasms are often manifested in an advanced state and with symptoms of nonmalignant vascular disease owing to a delay in diagnosis. We present the case of a 78-year-old man in whom workup was initially performed for a persistently enlarging abdominal aortic aneurysm after endovascular aortic repair but who was subsequently found to have a pleomorphic undifferentiated sarcoma of the aorta.

Keywords: Aneurysm; Angiosarcoma; EVAR; Endoleak.

Fig 1

Preoperative computed tomography angiography (CTA) image of infrarenal aortic aneurysm with aortic sarcoma.

Fig 2

Preoperative computed tomography angiography (CTA) image showing liver lesions reported as hepatic cysts.

Fig 3

Large exophytic neoplastic mass involving the retroperitoneum identified as an aortic sarcoma. The right of the image is the cranial direction. The red vessel loop is around the left renal vein.

Fig 4

Intraoperative aortic wall/retroperitoneal mass biopsy specimen showing pleomorphic sarcoma with spindle cell morphology, nuclear pleomorphism, and numerous mitoses (hematoxylin and eosin stain, magnification ×200).

Fig 5

Aortic biopsy specimen tumor cells positive for CD68 as denoted by brown staining (CD68 immunostain with hematoxylin counterstain, magnification ×100).