Although this was the only patient treated with cisplatinum in our cohort, this observation indicated that an acinar cell carcinoma with a loss-of-function mutation in BRCA2 may respond well to chemotherapy with cisplatinum, which indicates the particular importance of BRCA2 status as a biomarker for sensitivity to chemotherapy in acinar cell carcinomas. Here, BRCA2 is linked to acinar cell carcinoma.