BACKGROUND 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 advanceddisease. 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. CASE SUMMARY 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. CONCLUSION The current case suggests the beneficial effect of olaparib in BRCA mutated MCAC. However, further studies are required.

Pancreatic mucinous cystadenocarcinoma in a patient harbouring BRCA1 germline mutation effectively treated with olaparib: A case report

Mariacristina Di Marco
Primo
Writing – Original Draft Preparation
;
Riccardo Carloni;Stefania De Lorenzo;Cristina Mosconi;Andrea Palloni;Elisa Grassi;Daria Maria Filippini;Angela Dalia Ricci;Alessandro Rizzo;Alessandro Di Federico;Daniela Turchetti;Claudio Ricci;Carlo Ingaldi;Laura Alberici;Francesco Minni;Rita Golfieri;Giovanni Brandi;Riccardo Casadei
2020

Abstract

BACKGROUND 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 advanceddisease. 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. CASE SUMMARY 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. CONCLUSION The current case suggests the beneficial effect of olaparib in BRCA mutated MCAC. However, further studies are required.
2020
Mariacristina Di Marco, Riccardo Carloni, Stefania De Lorenzo, Cristina Mosconi, Andrea Palloni, Elisa Grassi, Daria Maria Filippini, Angela Dalia Ricci, Alessandro Rizzo, Alessandro Di Federico, Donatella Santini, Daniela Turchetti, Claudio Ricci, Carlo Ingaldi, Laura Alberici, Francesco Minni, Rita Golfieri, Giovanni Brandi, Riccardo Casadei
File in questo prodotto:
File Dimensione Formato  
articolo di marco dicembre 2020 WJGO.pdf

accesso aperto

Tipo: Versione (PDF) editoriale
Licenza: Licenza per Accesso Aperto. Creative Commons Attribuzione - Non commerciale (CCBYNC)
Dimensione 3.47 MB
Formato Adobe PDF
3.47 MB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11585/790893
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 1
  • ???jsp.display-item.citation.isi??? 1
social impact