Long-term survival in a patient with BRCA2-mutated pleomorphic anaplastic pancreatic carcinoma treated with Olaparib maintenance therapy.

Anaplastic pancreatic carcinoma (APC) is an extremely rare and aggressive histological subtype of pancreatic cancer with a poor prognosis. The therapeutic significance of BRCA1/2 mutations, which are established targets in pancreatic ductal adenocarcinoma (PDAC), remains unclear in APC. We report the case of a man in his 70s diagnosed with metastatic pleomorphic APC. Germline testing revealed a pathogenic BRCA2 variant. After achieving stable disease with 5 months of platinum-based chemotherapy (mFOLFIRINOX), he was transitioned to maintenance therapy with the poly(ADP-ribose) polymerase (PARP) inhibitor olaparib. Olaparib maintained stable disease for 18 months. Although his clinical course was complicated by a concurrent sigmoid colon cancer, the patient remains alive at 47 months post-diagnosis. To the best of our knowledge, this case is the first to report long-term disease control with a PARP inhibitor in a patient with BRCA2-mutated APC of the pleomorphic (non-osteoclast-like giant cell) subtype. Our findings suggest the potential efficacy of a “platinum-based therapy followed by PARP maintenance” strategy in BRCA-mutated APC and highlight the importance of early germline BRCA testing.