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Tumori. 2019 Apr 9:300891619839461. doi: 10.1177/0300891619839461. [Epub ahead of print]

The Italian Rare Pancreatic Exocrine Cancer Initiative.

Author information

1
1 Medical Oncology Unit, IRCCS Cancer Institute "Giovanni Paolo II" of Bari, Bari, Italy.
2
2 Department of Diagnostics and Public Health, Section of Pathology, University of Verona, Verona, Italy.
3
3 Molecular Diagnostics and Pharmacogenetics Unit, IRCCS Istituto Tumori "Giovanni Paolo II", Bari, Italy.
4
4 Functional Biomorphology Laboratory, IRCCS-Istituto Tumori, Bari, Italy.
5
5 Medical Oncology Unit, Hospital of Vicenza, Vicenza, Italy.
6
6 Medical Oncology Unit, Sant'Andrea Hospital, University of Rome La Sapienza, Rome, Italy.
7
7 Medical Oncology Unit, University Hospital of Pisa, Pisa, Italy.
8
8 Medical Oncology Unit, Scientific Institute of Romagna for the Study and Treatment of Cancer (IRST), Meldola, Italy.
9
9 Medical Oncology Unit, University of Cagliari, Cagliari, Italy.
10
10 Medical Oncology Unit, ASST Bergamo Ovest, Treviglio, Italy.
11
11 Medical Oncology Unit, IRCCS Foundation Polyclinic San Matteo, Pavia, Italy.
12
12 Medical Oncology Unit, II University of Naples, Naples, Italy.
13
13 Medical Oncology Unit, Careggi University Hospital, Florence, Italy.
14
14 Medical Oncology Unit, "Regina Elena" National Cancer Institute, Rome, Italy.
15
15 Gastrointestinal Medical Oncology and Neuroendocrine Tumors, European Institute of Oncology (IEO), Milan, Italy.
16
16 Medical Oncology Unit, Polytechnic University of the Marche, "Ospedali Riuniti Ancona," Ancona, Italy.
17
17 Department of Pathology and Diagnostics, University of Verona Hospital Trust, Policlinico GB Rossi, Verona, Italy.
18
18 Medical Oncology Unit, University Campus Biomedico, Rome, Italy.
19
19 Department of Medical Oncology, IRCCS San Raffaele Scientific Institute, Milan, Italy.
20
20 Medical Oncology Unit, IRCCS "Casa Sollievo della Sofferenza" Foundation, San Giovanni Rotondo, Italy.
21
21 Arc-Net Centre for Applied Research on Cancer, University and Hospital Trust of Verona, Verona, Italy.
22
22 Hepato-Biliary-Pancreatic Surgery, University of Milan, Istituto Nazionale Tumori, Fondazione IRCCS, Milan, Italy.
23
23 Medical Oncology Unit, IRCCS Veneto Institute of Oncology (IOV), Padua, Italy.
24
24 Hepatobiliary Surgery Unit, IRCCS A. Gemelli Polyclinic Foundation, Catholic University of the Sacred Heart, Rome, Italy.
25
25 Oncology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
26
26 Medical Oncology Unit, Modena Cancer Center, University Hospital of Modena, Modena, Italy.
27
27 Scientific Direction, IRCCS Cancer Institute "Giovanni Paolo II" of Bari, Bari, Italy.

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.

KEYWORDS:

Rare tumors; biomolecular characterization; chemotherapy; pancreatic cancer

PMID:
30967031
DOI:
10.1177/0300891619839461

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