Pulmonary pleomorphic carcinoma (PPC) is a rare and highly malignant subtype of non-small-cell lung cancer (NSCLC), and chemotherapy and radiotherapy are insensitive. Some clinical trials have shown that targetable driver gene mutations, such as EGFR, ALK or BRAF, have rarely been detected in PPC patients, but the incidence of MET exon 14 mutations is more frequent. For these patients with driver gene mutations, corresponding molecular targeted therapy may be valid. In addition, limited cases have suggested that immunotherapy may be effective for PPC without sensitising EGFR or ALK alterations, but the efficacy in patients with other driver mutations remains unclear. Herein, we reported two PPC patients with different targetable gene mutations who both responded dramatically to the PD-1 inhibitor camrelizumab combined with the oral anti-angiogenic drug anlotinib: one harbouring a BRAF V600E mutation with positive PD-L1 expression, few tumour-infiltrating lymphocytes (TILs) and abundant tumour blood vessels; and the other exhibiting a MET exon 14 skipping mutation with PD-L1 overexpression, scattered TILs and abundant tumour blood vessels. Our findings suggest that PD-1 inhibitor combined with anlotinib may be a potential treatment for PPC patients, and abundant tumour vessels should be investigated as a possible therapeutic biomarker.
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