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Gastrinoma and Zollinger Ellison syndrome:A roadmap for the management between new and old therapies

World Journal of Gastroenterology 2021年第35期27卷 5890-5907页

作者： Roberta Elisa Rossi Alessandra Elvevi Davide Citterio jorgelina coppa Pietro Invernizzi Vincenzo Mazzaferro Sara Massironi HPB Surgery Hepatology and Liver TransplantationENETS Center of ExcellenceFondazione IRCCS Istituto Nazionale Tumori(INTNational Cancer Institute)Milan 20133Italy Department of Pathophysiology and Transplantation University of MilanMilan 20122Italy Division of Gastroenterology and Center for Autoimmune Liver Diseases Department of Medicine and SurgeryUniversity of Milano-BicoccaMonza 20900Italy European Reference Network on Hepatological Diseases(ERN RARE-LIVER) San Gerardo HospitalMonza 20033Italy Department of Oncology and Hemato-Oncology University of MilanMilan 20122Italy

Zollinger-Ellison syndrome(ZES)associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion,which typically leads to gastroesophageal reflux disease,recurrent peptic ulcers,and chronic *** symptoms of ZES are nonspecific and overlap with other gastrointestinal disorders,the diagnosis is often delayed with an average time between the onset of symptoms and final diagnosis longer than 5 *** critical step for the diagnosis of ZES is represented by the initial clinical *** is the hallmark of ZES;however,hypergastrinemia might recognize several causes,which should be ruled out in order to make a final *** levels>1000 pg/mL and a gastric pH below 2 are considered to be diagnostic for gastrinoma;some specific tests,including esophageal pH-recording and secretin test,might be useful in selected cases,although they are not widely *** ultrasound is very useful for the diagnosis and the local staging of the primary tumor in patients with ZES,particularly in the setting of multiple endocrine neoplasia type *** controversies about the management of these tumors also *** the localized stage,the combination of proton pump inhibitory therapy,which usually resolves symptoms,and surgery,whenever feasible,with curative intent represents the hallmark of gastrinoma *** high expression of somatostatin receptors in gastrinomas makes them highly responsive to somatostatin analogs,supporting their use as anti-proliferative agents in patients not amenable to surgical *** medical options for advanced disease are super-imposable to other neuroendocrine neoplasms,and studies specifically focused on gastrinomas only are scant and often limited to case reports or small retrospective *** multidisciplinary approach remains the cornerstone for the proper management of this composite ***,we reviewed available literature about gastrinoma-associated ZES wit

关键词： Gastrinoma Zollinger-Ellison syndrome Neuroendocrine neoplasms Pancreatic neuroendocrine neoplasm Duodenal neuroendocrine neoplasm Diagnosis Therapy

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One size does not fit all for pancreatic cancers: A review on rare histologies and therapeutic approaches

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World Journal of Gastrointestinal Oncology 2020年第8期12卷 833-849页

作者： Monica Niger Michele Prisciandaro Maria Antista Melissa Anna Teresa Monica Laura Cattaneo Natalie Prinzi Sara Manglaviti Federico Nichetti Marta Brambilla Martina Torchio Francesca Corti Sara Pusceddu jorgelina coppa Vincenzo Mazzaferro Filippo de Braud Maria Di Bartolomeo Medical Oncology Department Fondazione IRCCS Istituto Nazionale dei TumoriMilan 20133Italy First Pathology Division Department of Pathology and Laboratory MedicineFondazione IRCCS Istituto Nazionale dei TumoriMilan 20133Italy Hepato-biliary-pancreatic Surgery and Liver Transplantation Department Fondazione IRCCS Istituto Nazionale dei TumoriMilan 20133Italy Universitàdegli studi di Milano Milan 20133Italy

Exocrine pancreatic neoplasms represent up to 95%of pancreatic cancers(PCs)and are widely recognized among the most lethal solid cancers,with a very poor 5-year survival rate of 5%-10%.The remaining<5%of PCs are neuroendocrine tumors that are usually characterized by a better prognosis,with a median overall survival of 3.6 *** most common type of PC is pancreatic ductal adenocarcinoma(PDAC),which accounts for roughly 85%of all exocrine *** up to 10%of exocrine PCs have rare histotypes,which are still poorly *** subtypes can be distinguished from PDAC in terms of pathology,imaging,clinical presentation and ***,due to their rarity,any knowledge regarding these specific histotypes is mostly based on case reports and a small series of retrospective ***,treatment strategies are generally deduced from those used for PDAC,even if these patients are often excluded or not clearly represented in clinical trials for *** these reasons,it is essential to collect as much information as possible on the management of PC,as assimilating it with PDAC may lead to the potential mistreatment of these ***,we report the most significant literature regarding the epidemiology,typical presentation,possible treatment strategies,and prognosis of the most relevant histotypes among rare PCs.

关键词： Rare pancreatic cancers Pancreatic acinar cell cancer Pancreatic adenosquamous cancer Undifferentiated pancreatic cancer Pancreatoblastoma Pseudopapillary pancreatic cancer

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