Angiosarcoma masquerading as popliteal artery aneurysm.

Background: This research was carried out to identify relevant steps to diagnosis and possible treatment regimens regarding angiosarcomas of the popliteal artery associated with true popliteal aneurysms, as they are very rare. Materials and methods: A systematic literature search was conducted in accordance with the Prisma Guidelines. Only publications regarding true popliteal aneurysms in association with true angiosarcomas were taken into account. Furthermore, three well-documented not yet published cases are depicted. Results: 13 case reports could be identified. Besides two initially diagnosed cases (ID), all other cases were diagnosed secondary (SD) and underwent surgery due to ipsilateral aneurysms in the past. For the reported cases, pain was the most frequent symptom (61%), followed by oedema (46%) and localised swelling in the popliteal fossa. 38% of patients showed central metastasis at the time of diagnosis. Both ID patients underwent primary resection of the tumour - in one case followed by arterial reconstruction, local radiotherapy and regional hyperthermal perfusion. 45% of SD patients underwent sole major amputation, the others received varying treatment regimens (chemotherapy, radiotherapy (or combination)) or sole palliative care. The short available follow-up of the two ID cases was uneventful. The median survival time for SD patients treated by major amputation was 8 months (average 19.2 months) and for the others in median 4.5 months (average 11 months). Conclusions: Non-ischaemic pain, swelling and/or sudden regrowth years after treatment of a popliteal aneurysm are highly suspect of malignancy and should be further evaluated (e.g. biopsy/positron emission tomography computed tomography).