Angiosarcoma in the setting of vascular procedures: A systematic review and our experiences

Angiosarcoma is a rare, aggressive endothelial malignancy with a poor prognosis; diagnosis is often delayed by vague symptoms, nonspecific imaging and challenging histopathology. Case reports have suggested an association with prior vascular procedures, with or without prosthetic material, but no causal link is established. We reviewed angiosarcoma across the full spectrum of vascular reconstruction.

We systematically reviewed English-language human studies in PubMed, Embase and the Cochrane Library for histologically confirmed angiosarcomas diagnosed after and anatomically related to vascular procedures. The review was registered on PROSPERO (CRD42024602643).

We describe three patients with metastatic angiosarcoma contiguous with vascular prostheses: a 75-year-old male with left inguinal angiosarcoma seven years after PTFE bypass grafting; a 69-year-old male with angiosarcoma within the aneurysm sac seven years after endovascular aneurysm repair (EVAR); and an 80-year-old male with intraluminal angiosarcoma within an EVAR limb presenting as distal embolisation. Systematic review identified 44 studies comprising 46 additional cases. Including these, 49 patients were analysed: 27 associated with endovascular prostheses, 14 with open prosthetic or mixed grafts, and 8 with autologous-vein bypass. Mean diagnostic latency was 7 years (range 5 months to 18 years); mean age 70.5 years; 12.2% were female.

Angiosarcoma is difficult to diagnose given its nonspecific presentation but should be considered in atypical or refractory post-procedural presentations. This is the largest synthesis to date and the first to describe intraluminal EVAR-limb angiosarcoma with distal embolisation. Inclusion of autologous-vein reconstructions argues against a purely prosthetic-material explanation; further investigation is needed to clarify causality.