BRCA mutations are associated with an increased risk of pancreatic cancer (PC). Olaparib, an oral poly (adenosine diphosphate-ribose) polymerase (PARP) inhibitor, has been approved for the treatment of metastatic PC with a germline BRCA mutation. In this report, we present the case of a metastatic PC harboring a germline BRCA2 mutation, and the daughter of the patient, who had bilateral breast cancers harboring the same germline mutation, suggesting that the PC was associated with hereditary breast and ovarian cancer syndrome. Although PC is an aggressive disease and has poor prognosis, olaparib was administered as maintenance therapy following modified FOLFIRINOX, providing clinical benefits for >12 months.
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