• Xia Wang, Jianping Wei, Zhimin Zeng, Jing Cai, Zhiqin Lu, and Anwen Liu.
• Department of Oncology, The Second Affiliated Hospital of Nanchang University, China.
• Medicine (Baltimore). 2021 Sep 3; 100 (35): e27132e27132.

RationalePrimary pleural angiosarcoma (PPA) is an extremely rare malignancy for which there is no consensus on treatment. The clinical course of PPA is usually quickly fatal, regardless of the treatment used.Patient ConcernsWe describe the rare case of a 52-year-old man who presented initially with hemoptysis and received emergency surgery for the primary.DiagnosesHe received a confirmed diagnosis of primary pleural angiosarcoma (PPA) by postoperative pathology and was subsequently treated with radiotherapy and chemotherapy, but had failed and was intolerant to chemotherapy.InterventionsThe patient had 5% tumor PD-L1 positivity with 22C3 pharmDx and received pembrolizumab (200 mg every 21 days) for 13 cycles.OutcomesThe disease remained well controlled according to the RECIST 1.1. criteria. He is currently under observation and waiting to start the next cycle of immunotherapy.LessonOur case report suggests that the use of anti-PD-1 therapy does show efficacy in the treatment of PPA and may provide a viable treatment option for patients.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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