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Two rare cancers of the exocrine pancreas: to treat or not to treat like ductal adenocarcinoma?

Journal of cancer Metastasis and Treatment 2023年第1期9卷 342-367页

作者： Nebojsa Skorupan Shadin Ghabra JAlberto Maldonado Yang Zhang Christine Alewine Laboratory of Molecular Biology NCI Center for Cancer ResearchBethesdaMD 20892USA Medical Oncology Program NCI Center for Cancer ResearchBethesdaMD 20892USA Surgical Oncology Program NCI Center for Cancer ResearchBethesdaMD 20892USA Medical Research Scholars Program NCI Center for Cancer ResearchBethesdaMD 20892USA Gastrointestinal Pathology Joint Pathology CenterSilver SpringMD 20910USA

pancreatic cancer is an aggressive malignancy with increasing *** ductal adenocarcinoma(PDAC)accounts for>90%of pancreatic cancer diagnoses,while other exocrine tumors are much *** this review,we have focused on two rare cancers of the exocrine pancreas:adenosquamous carcinoma of the pancreas(ASCP)and pancreatic acinar cell carcinoma(PACC).The latest findings regarding their cellular and molecular pathology,clinical characteristics,prognosis,and clinical management are *** genetic and transcriptomic data suggest that ASCP is related to or overlaps with the basal transcriptomic subtype of *** tumors are highly aggressive and driven by activated KRAS and MYC *** outcomes remain poor and effective treatments are *** has no morphologic or genetic resemblance to PDAC and more favorable *** stage PACC patients have improved survival with surgical resection and patients with advanced disease benefit most from platinum-or fluoropyrimidine-containing *** of actionable genetic mutations is high in this disease and case reports suggest good outcomes when matched therapy is *** clinical studies examining ASCP and PACC are limited and difficult to *** research is needed to define optimal clinical management for these rare diseases.

关键词： Adenosquamous carcinoma of the pancreas acinar cell carcinoma of the pancreas rare exocrine pancreatic cancer

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