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Long-term survival of two patients with recurrent pancreatic acinar cell carcinoma treated with radiofrequency ablation:A case report

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World Journal of Clinical Cases 2020年第7期8卷 1241-1250页

作者： mariacristina Di Marco Riccardo Carloni Stefania De Lorenzo Elisa Grassi Andrea Palloni Francesca Formica Stefano Brocchi daria maria filippini Rita Golfieri Giovanni Brandi Department of Experimental Diagnosticand Specialty Medicine-DIMESSant'Orsola-Malpighi HospitalUniversity of BolognaBologna 40138Italy Medical Oncology Ospedale degli InfermiFaenza 48018Italy Radiology Unit Department of Diagnostic Medicine and PreventionSant'Orsola-Malpighi HospitalUniversity of BolognaBologna 40138Italy

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.

关键词： Acinar cell carcinoma Pancreas Liver metastasis Radiofrequency ablation Case report

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

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World Journal of Gastrointestinal Oncology 2020年第12期12卷 1456-1463页

作者： 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 Department of Experimental Diagnosticand Specialty Medicine-DIMESSant'Orsola-Malpighi HospitalUniversity of BolognaBologna 40138Italy Division of Oncology Azienda Ospedaliero-Universitaria di BolognaBologna 40138Italy Radiology Unit Department of Diagnostic Medicine and PreventionSant'Orsola-Malpighi HospitalUniversity of BolognaBologna 40138Italy Medical Oncology Ospedale Degli InfermiFaenza 48018Italy Pathology Unit Sant'Orsola-Malpighi HospitalBologna 40138Italy Unit of Medical Genetics Sant'Orsola-Malpighi HospitalUniversity of BolognaBologna 40138Italy Division of Pancreatic Surgery Azienda Ospedaliero-Universitaria Di BolognaBologna 40138Italy Department of Internal Medicine and Surgery Alma Mater StudiorumUniversity of BolognaBologna 40138Italy

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.

关键词： Mucinous cystadenocarcinoma Pancreatic cancer BRCA1 gene Olaparib Case report

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