biostudies-literatureUnknown10Wu XChordoma is a rare primary bone tumor that exhibits insensitivity to radiotherapy and chemotherapy and has a poor prognosis. Currently, resection is the primary treatment for affected patients, but the subsequent rate of recurrence is high, and both overall survival (OS) and progression-free survival (PFS) are consequentially relatively short. This case report describes a patient who was diagnosed with metastatic chordoma that was found to possess the A1209fs mutation of the <i>PBRM1</i> gene, which may be associated with beneficial responses to immunotherapies. The patient received pembrolizumab, an immune checkpoint inhibitor (ICI) that targets the PD-1 receptor of lymphocytes, as second-line therapy, which he tolerated well (the most frequent adverse events were abnormal liver function Frontiers in oncology565945https://www.ebi.ac.uk/biostudies/studies/S-EPMC7774333biostudies-literatureResponse of Metastatic Chordoma to the Immune Checkpoint Inhibitor Pembrolizumab: A Case Report.PMC7774333Lin XChen YYu ZXu JWu XKong WfalseResponse of Metastatic Chordoma to the Immune Checkpoint Inhibitor Pembrolizumab: A Case Report.Chordoma is a rare primary bone tumor that exhibits insensitivity to radiotherapy and chemotherapy and has a poor prognosis. Currently, resection is the primary treatment for affected patients, but the subsequent rate of recurrence is high, and both overall survival (OS) and progression-free survival (PFS) are consequentially relatively short. This case report describes a patient who was diagnosed with metastatic chordoma that was found to possess the A1209fs mutation of the <i>PBRM1</i> gene, which may be associated with beneficial responses to immunotherapies. The patient received pembrolizumab, an immune checkpoint inhibitor (ICI) that targets the PD-1 receptor of lymphocytes, as second-line therapy, which he tolerated well (the most frequent adverse events were abnormal liver function 2020-01-01T00:00:00Z20202025-06-01T02:18:31.07Z2021-02-20T17:24:12ZS-EPMC77743333339206910.3389/fonc.2020.565945