Abstract

Background Angiosarcoma is an uncommon form of soft tissue sarcoma. Presentation and treatment are heterogeneous. We undertook a multicenter study evaluating the epidemiology, treatment and outcomes of AS pts in 8 sarcoma academic centres across Asia. Methods This is a retrospective chart review of AS pts seen at the study sites. Results In total 423 pts were included into study. Median follow-up was 12.4 mths. 59% and 41% had cutaneous (C-AS) and visceral AS (V-AS) respectively. Median age was 67 yr [74 yr vs 54 yr in C-AS vs V-AS, p < 0.001]. 58% presented with localized disease while 41% had locally advanced/metastatic disease. The most common primary site in pts with C-AS and V-AS was scalp and liver respectively. In pts with localized C-AS, primary treatment was surgery, chemotherapy and radiotherapy in 55%, 21% and 17% respectively. While in localized V-AS, primary treatment patterns included surgery, chemotherapy and best supportive care (BSC) in 73%, 11% and 8% respectively. In pts with localized disease for which surgery was performed and margin status known (n = 114), R0 margin was achieved in 69%, 66% vs 75% in C-AS vs V-AS (OR = 0.65, p = 0.3). In pts with locally advanced/metastatic disease (n = 173), 27%, 14% and 6% received 1, 2 and 3 lines of chemotherapy respectively; 7% of pts received >3 lines of chemotherapy. The most common first line chemotherapy regimen was paclitaxel (54%)followedbyliposomaldoxorubicin(15%).InptswithlocalizedASwhohad undergoneprimarysurgery,relapse-freesurvivalwas10.9mths,12.9vs8.1mthsinpts withC-ASvsV-AS(HR1.10,P=0.6).Medianoverallsurvival(OS)was23.7mths,24.0 mthsinC-ASvs23.0mthsinV-AS(HR1.22,P=0.3). Inptswithlocallyadvanced/ metastatic AS, median OS on BSC was 1.7 mths vs 17.1 mths for those who received at least1 lineofchemotherapy(HR5.30,P<0.001).MedianOSinptswithlocally advanced/metastaticC-ASvsV-ASwas11.5mthsvs8.3mthsinpts(HR0.94,P=0.7). Conclusions: This large observational study in AS is the first collaborative study by the ASC. This study highlights the complexity of AS and heterogeneity of treatment across Asia. Prognosis is poor and optimal treatment remains to be defined. Legal entity responsible for the study National Cancer Centre Singapore Funding N/A Disclosure R. Quek: Grants/research support: Novartis Pfizer, Janssen, Bayer and Eisai Honoraria or consultation fees: Novartis, Bayer, BMS, Merck, Roche, Eisai Participation in a company sponsored speaker's bureau: Novartis, Bayer, Merck, Eisai. V. Sriuranpong: Corporate sponsored research: Novartis, MSD, Astra Zeneca, Roche, Lilly, Sanofi, Pfizer, BMS. All other authors have declared no conflicts of interest.

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