For four patients with BRCA1/2 mutation who received olaparib/pembrolizumab-based combined therapy as first-line treatment (the diagnosis was SCLC, rhabdomyosarcoma, cholangiocarcinoma and pancreatic cancer, respectively), disease was well controlled with three patients achieving PR and one patient achieving SD. This evidence concerns the gene BRCA1 and familial pancreatic carcinoma.