Published online Dec 15, 2020. doi: 10.4251/wjgo.v12.i12.1456
Peer-review started: October 13, 2020
First decision: October 23, 2020
Revised: November 1, 2020
Accepted: November 10, 2020
Article in press: November 10, 2020
Published online: December 15, 2020
Pancreatic mucinous cystadenocarcinoma (MCAC) is a rare malignancy with a poor prognosis when it presents metastases at diagnosis. Due to its very low incidence, there are no clear recommendations for the treatment of advanced disease. Olaparib (an oral PARP inhibitor) has been approved for the maintenance treatment of patients with metastatic pancreatic adenocarcinoma harbouring germline BRCA1/2 mutations. Herein, we report the first case of a germline BRCA1 mutated unresectable MCAC which was effectively treated with olaparib.
A 41-year-old woman, without personal or family history of cancer, was diagnosed with ovarian and peritoneal metastases of MCAC. She underwent 12 cycles of gemcitabine plus oxaliplatin (GEMOX) obtaining a partial response and allowing radical surgery. One year later, local recurrence was documented, and other 12 cycles of GEMOX were administered obtaining a complete response. Seven years later, another local recurrence, not amenable to surgical resection, was diagnosed. She started FOLFIRINOX (oxaliplatin, irinotecan, leucovorin and fluorouracil), obtaining a partial response after 8 cycles. Given the excellent response to platinum-based chemotherapy, BRCA testing was performed, and a BRCA1 germline mutation was detected. She was switched to maintenance olaparib due to chemotherapy-related toxicities and achieved an almost complete metabolic response, with a reduction in the diameter of the lesion, after three months of therapy.
The current case suggests the beneficial effect of olaparib in BRCA mutated MCAC. However, further studies are required.
Core Tip: Pancreatic mucinous cystadenocarcinoma (MCAC) is a rare malignancy with a poor prognosis when it presents metastases at diagnosis. Due to its very low incidence, there are no clear recommendations for the treatment of advanced disease. Olaparib (an oral PARP inhibitor) has remarkable curative effects in BRCA mutated pancreatic ductal adenocarcinoma, breast and ovarian cancers. However, there are few data on its efficacy in the treatment of other types of pancreatic malignancies. Herein, we report the first case of a germline BRCA1 mutated MCAC which was effectively treated with olaparib. We also provide a brief overview of the most relevant clinical features of MCAC.