TY - JOUR
T1 - The Italian Rare Pancreatic Exocrine Cancer Initiative
AU - Brunetti, Oronzo
AU - Luchini, Claudio
AU - Argentiero, Antonella
AU - Tommasi, Stefania
AU - Mangia, Anita
AU - Aprile, Giuseppe
AU - Marchetti, Paolo
AU - Vasile, Enrico
AU - Casadei Gardini, Andrea
AU - Scartozzi, Mario
AU - Barni, Sandro
AU - Delfanti, Sara
AU - De Vita, Fernando
AU - Di Costanzo, Francesco
AU - Milella, Michele
AU - Cella, Chiara Alessandra
AU - Berardi, Rossana
AU - Cataldo, Ivana
AU - Santini, Daniele
AU - Doglioni, Claudio
AU - Maiello, Evaristo
AU - Lawlor, Rita T.
AU - Mazzaferro, Vincenzo
AU - Lonardi, Sara
AU - Giuliante, Felice
AU - Brandi, Giovanni
AU - Scarpa, Aldo
AU - Cascinu, Stefano
AU - Silvestris, Nicola
PY - 2019/1/1
Y1 - 2019/1/1
N2 - 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.
AB - 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.
KW - biomolecular characterization
KW - chemotherapy
KW - pancreatic cancer
KW - Rare tumors
U2 - 10.1177/0300891619839461
DO - 10.1177/0300891619839461
M3 - Article
JO - Tumori
JF - Tumori
SN - 0300-8916
ER -