Angiosarcoma in an arteriovenous fistula after kidney transplantation: Case report and review of treatment options.

We present a patient with an angiosarcoma in an arteriovenous fistula and we reviewed current treatments for angiosarcomas. We extended the systematic review by Oskrochi et al. on this topic in 2015, using the same search query. We searched systematically OVID, EMBASE and PubMed from April 2015 until January 2020 with the aim to update the state of the art in managing this rare but serious condition. We retrieved 12 new case reports about 11 unique patients. Mean age was 61.5 ± 11 years. Six arteriovenous fistulas had failed spontaneously. Two fistulas were operatively closed post-transplant. Nine patients (81.8%) were receiving ongoing immunosuppressive therapy. Pain and growing lesions, mass or swelling were the most frequent symptoms. Angiosarcoma mostly presents with a nonspecific clinical picture of pain, growing lesions and swelling of a previously normal arteriovenous fistula. Amputation of the limb was most frequently conducted as treatment in localized disease. Treatment of systemic disease included supportive care, chemotherapy, especially with paclitaxel and change of immunosuppressive regimen. Metastasized angiosarcoma has a very poor prognosis. Classical chemotherapy has rather low response rates. There is limited data supporting treatment of angiosarcomas with tyrosine kinase inhibitors or immunotherapy. Further comparative research is needed.