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Anti-PDL-1 response in metastatic soft tissue angiosarcoma: Clinical case and systematic review

MO Angel, FD Waisberg, P Mando, MR Chacon and RD Chacon

Purpose: Angiosarcomas (AS) account for about only 1%-2% of soft tissue sarcomas, other than chemotherapy and target agents for metastatic disease there are few therapeutics options available. Immune checkpoint inhibitors have become the new paradigm in the management of malignancies but not yet in AS. The objective of this study was to report a case of a patient with angiosarcoma treated with atezolizumab, and to conduct a literature review including publications about the use of checkpoint inhibitors for AS. Design: We performed a systematic search of electronic databases such as PubMed, EMBASE and Cochrane Registries using the following key words: “angiosarcoma” and “immunotherapy” or “pembrolizumab”, “nivolumab”, “atezolizumab”, “ipilimumab” or “checkpoint”. Results: Institutional case: A 61-year-old man diagnosed with metastatic low grade angiosarcoma in May 2017. He received chemotherapy with doxorubicin, paclitaxel and target therapy with pazopanib. After progression, a Foundation One Heme assay was performed in July 2018, and reported a high Tumor Mutation Burden of 59 mutations/Mb, Treatment with atezolizumab was initiated in September 2018. After 3 cycles of immune checkpoint inhibitor immunotherapy, clinical response was evident. After a 12 month-follow up the patient remains on partial response. Literature Review: Database search was performed on 8/27/2019. Two hundred thirty-six (236) studies were screened, and 26 articles and conference presentations were selected for full text analysis. After applying inclusion and exclusion criteria, 8 publications were identified, which documented the clinical course of 16 patients. Our patient was also included in the analysis. Conclusion: The use of immune checkpoint inhibitors in angiosarcomas seems to be a promising and safe option demonstrating early responses in this case series. Confirmed responses were observed in our literature review, mainly in patients with an identified biomarker.

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