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 treat...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 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.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.展开更多
BACKGROUND Pancreatic acinar cell carcinoma (PACC) is a rare type of malignant pancreatic cancer that represents approximately 1%of all pancreatic neoplasms.Due to its very low incidence,only a few retrospective studi...BACKGROUND Pancreatic acinar cell carcinoma (PACC) is a rare type of malignant pancreatic cancer that represents approximately 1%of all pancreatic neoplasms.Due to its very low incidence,only a few retrospective studies are available.Although surgery is the first choice for treatment,most patients experience recurrence(mainly in the liver) and there are no clear recommendations for patients with advanced disease.CASE SUMMARY We report two patients with PACC treated with sturgery who experienced tumour recurrence in the liver.Patient 1 carried a germline mutation in the APC gene.Both patients were treated with gemcitabine plus oxaliplatin and gemcitabine plus capecitabine as first-and second-line therapies,respectively.After a favoturable response to chemotherapy,the patients underwent radiofrequency ablation of the remaining liver metastases.For patient 1,we documented a relapse in the liver after a disease-free period of 9 mo,and treatment with gemcitabine plus capecitabine was restarted.The patient achieved a complete response,and he remains alive without evidence of disease recurrence after six years.After radiofrequency ablation,patient 2 experienced disease-free survival for 21 mo,when peritoneal relapse was diagnosed and treated with chemotherapy.The patient achieved a stable disease state for nearly two years;nevertheless,further progressive disease was documented,and he died seven years after the first relapse.CONCLUSION PACC presents different biological behaviours than pancreatic adenocarcinoma.Multidisciplinary treatment involving local ablative therapies may be considered for PACC.展开更多
文摘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 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.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.
文摘BACKGROUND Pancreatic acinar cell carcinoma (PACC) is a rare type of malignant pancreatic cancer that represents approximately 1%of all pancreatic neoplasms.Due to its very low incidence,only a few retrospective studies are available.Although surgery is the first choice for treatment,most patients experience recurrence(mainly in the liver) and there are no clear recommendations for patients with advanced disease.CASE SUMMARY We report two patients with PACC treated with sturgery who experienced tumour recurrence in the liver.Patient 1 carried a germline mutation in the APC gene.Both patients were treated with gemcitabine plus oxaliplatin and gemcitabine plus capecitabine as first-and second-line therapies,respectively.After a favoturable response to chemotherapy,the patients underwent radiofrequency ablation of the remaining liver metastases.For patient 1,we documented a relapse in the liver after a disease-free period of 9 mo,and treatment with gemcitabine plus capecitabine was restarted.The patient achieved a complete response,and he remains alive without evidence of disease recurrence after six years.After radiofrequency ablation,patient 2 experienced disease-free survival for 21 mo,when peritoneal relapse was diagnosed and treated with chemotherapy.The patient achieved a stable disease state for nearly two years;nevertheless,further progressive disease was documented,and he died seven years after the first relapse.CONCLUSION PACC presents different biological behaviours than pancreatic adenocarcinoma.Multidisciplinary treatment involving local ablative therapies may be considered for PACC.
