@article{efa62526f6594673be63103fbccc1f60,
title = "The Italian Rare Pancreatic Exocrine Cancer Initiative",
abstract = "Introduction: Exocrine pancreatic cancers include common type pancreatic ductal adenocarcinoma and cystic neoplasms, which account for 85% and 10% of cases, respectively. The remaining 5% are rare histotypes, comprising adenosquamous carcinoma, acinar cell carcinoma, signet ring cell carcinoma, medullary carcinoma, pancreatoblastoma, hepatoid carcinoma, undifferentiated carcinoma and its variant with osteoclast-like giant cells, solid pseudopapillary carcinoma, and carcinosarcoma. Due to their low incidence, little knowledge is available on their clinical and molecular features as well as on treatment choices. The national initiative presented here aims at the molecular characterization of series of rare histotypes for which therapeutic and follow-up data are available. Methods: A nationwide Italian Rare Pancreatic Cancer (IRaPaCa) task force whose first initiative is a multicentric retrospective study involving 21 Italian cancer centers to retrieve histologic material and clinical and treatment data of at least 100 patients with rare exocrine pancreatic cancers has been created. After histologic revision by a panel of expert pathologists, DNA and RNA from paraffin tissues will be investigated by next-generation sequencing using molecular pathway–oriented and immune-oriented mutational and expression profiling panels constructed availing of the information from the International Cancer Genome Consortium. Bioinformatic analysis of data will drive validation studies by immunohistochemistry and in situ hybridization, as well as nanostring assays. Conclusions: We expect to gather novel data on rare pancreatic cancer types that will be useful to inform the design of therapeutic choices. {\textcopyright} Fondazione IRCCS Istituto Nazionale dei Tumori 2019.",
keywords = "biomolecular characterization, chemotherapy, pancreatic cancer, Rare tumors, CD163 antigen, CD20 antigen, CD4 antigen, CD68 antigen, CD8 antigen, DNA, RNA, transforming growth factor beta, acinar cell carcinoma, anaplastic carcinoma, Article, carcinosarcoma, cystic neoplasm, DNA damage, exocrine pancreas, follow up, human, immunohistochemistry, in situ hybridization, liver cell carcinoma, medullary carcinoma, next generation sequencing, pancreas adenocarcinoma, pancreas cancer, retrospective study, signet ring carcinoma, adenosquamous carcinoma, clinical trial, female, Italy, male, multicenter study, pancreas carcinoma, pancreas tumor, pathology, procedures, Carcinoma, Acinar Cell, Carcinoma, Adenosquamous, Carcinoma, Pancreatic Ductal, Female, Humans, Immunohistochemistry, Male, Pancreatic Neoplasms, Retrospective Studies",
author = "O. Brunetti and C. Luchini and A. Argentiero and S. Tommasi and A. Mangia and G. Aprile and P. Marchetti and E. Vasile and {Casadei Gardini}, A. and M. Scartozzi and S. Barni and S. Delfanti and {De Vita}, F. and {Di Costanzo}, F. and M. Milella and C.A. Cella and R. Berardi and I. Cataldo and D. Santini and C. Doglioni and E. Maiello and R.T. Lawlor and V. Mazzaferro and S. Lonardi and F. Giuliante and G. Brandi and A. Scarpa and S. Cascinu and N. Silvestris",
note = "Cited By :1 Export Date: 26 February 2020 CODEN: TUMOA Correspondence Address: Silvestris, N.; Medical Oncology Unit, IRCCS Cancer Institute “Giovanni Paolo II” of BariItaly; email: n.silvestris@oncologico.bari.it Chemicals/CAS: DNA, 9007-49-2; RNA, 63231-63-0 Funding details: Fondazione IRCCS Policlinico San Matteo Funding details: Universit{\`a} degli Studi di Verona Funding details: National Cancer Institute, NCI Funding details: Instituto Espa{\~n}ol de Oceanograf{\'i}a, IEO Funding details: Universit{\`a} di Bologna, UNIBO Funding details: Istituto Oncologico Veneto, IOV Funding details: 12182, 5x1000 Funding details: Associazione Italiana per la Ricerca sul Cancro, AIRC Funding text 1: 1Medical Oncology Unit, IRCCS Cancer Institute {"}Giovanni Paolo II{"} of Bari, Bari, Italy 2Department of Diagnostics and Public Health, Section of Pathology, University of Verona, Verona, Italy 3Molecular Diagnostics and Pharmacogenetics Unit, IRCCS Istituto Tumori {"}Giovanni Paolo II{"}, Bari, Italy 4Functional Biomorphology Laboratory, IRCCS-Istituto Tumori, Bari, Italy 5Medical Oncology Unit, Hospital of Vicenza, Vicenza, Italy 6Medical Oncology Unit, Sant{\textquoteright}Andrea Hospital, University of Rome La Sapienza, Rome, Italy 7Medical Oncology Unit, University Hospital of Pisa, Pisa, Italy 8Medical Oncology Unit, Scientific Institute of Romagna for the Study and Treatment of Cancer (IRST), Meldola, Italy 9Medical Oncology Unit, University of Cagliari, Cagliari, Italy 10Medical Oncology Unit, ASST Bergamo Ovest, Treviglio, Italy 11Medical Oncology Unit, IRCCS Foundation Polyclinic San Matteo, Pavia, Italy 12Medical Oncology Unit, II University of Naples, Naples, Italy 13Medical Oncology Unit, Careggi University Hospital, Florence, Italy 14Medical Oncology Unit, “Regina Elena” National Cancer Institute, Rome, Italy 15Gastrointestinal Medical Oncology and Neuroendocrine Tumors, European Institute of Oncology (IEO), Milan, Italy 16Medical Oncology Unit, Polytechnic University of the Marche, “Ospedali Riuniti Ancona,” Ancona, Italy Funding text 2: 17Department of Pathology and Diagnostics, University of Verona Hospital Trust, Policlinico GB Rossi, Verona, Italy 18Medical Oncology Unit, University Campus Biomedico, Rome, Italy 19Department of Medical Oncology, IRCCS San Raffaele Scientific Institute, Milan, Italy 20Medical Oncology Unit, IRCCS “Casa Sollievo della Sofferenza” Foundation, San Giovanni Rotondo, Italy 21Arc-Net Centre for Applied Research on Cancer, University and Hospital Trust of Verona, Verona, Italy 22Hepato-Biliary-Pancreatic Surgery, University of Milan, Istituto Nazionale Tumori, Fondazione IRCCS, Milan, Italy 23Medical Oncology Unit, IRCCS Veneto Institute of Oncology (IOV), Padua, Italy 24Hepatobiliary Surgery Unit, IRCCS A. 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year = "2019",
doi = "10.1177/0300891619839461",
language = "English",
volume = "105",
pages = "353--358",
journal = "Tumori",
issn = "0300-8916",
publisher = "SAGE Publications Ltd",
number = "4",
}