Angiosarcoma and Dialysis-related Arteriovenous Fistulae: A Comprehensive Review.

Objective/background: To conduct a comprehensive review of cases, presentation, diagnosis, and management of angiosarcoma in arteriovenous fistulae (AVF) created for haemodialysis.

Methods: Two authors independently conducted systematic searches and extraction of articles from the Embase, AMED, Health Management Information Consortium, and MEDLINE databases in keeping with the inclusion/exclusion criteria and Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards.

Results: Twenty-two unique patient cases were identified; 20 of the cases were men and mean ± SD age of presentation was 54.9 ± 13.6 years. Nineteen cases were post-transplant and 18 were on antirejection agents. The most common presenting symptom was pain, with or without a mass. The initial diagnosis was most often thrombosis/infection of the AVF and the diagnostic interval to a correct diagnosis of angiosarcoma was between 2 and 40 weeks. Mean ± SD time to presentation of symptoms from fistula formation was 118.9 ± 57.5 months, while from transplant it was 96.9 ± 70.0 months. Amputation was the most common treatment modality and mean ± SD survival was 8.8 ± 3.7 months.

Conclusion: Angiosarcoma should be suspected in previously quiescent AVF that presents with pain. The presence of a rapidly enlarging mass or bleeding/bruising should be taken as alarm indicators and warrant urgent investigation in accordance with local cancer guidelines. Any surgical procedure should involve histological samples as a matter of course.