CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype

The intraductal oncocytic papillary neoplasm (IOPN) of the pancreas has been recognized by WHO classification as a unique intraductal papillary mucinous neoplasm (IPMN) category. IOPN is composed of oxyphil cells, usually expressing MUC5AC, MUC6, and Hep Par-1, and harboring <i>PRKACA</i>...

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Main Authors: Paola Mattiolo, Seung-Mo Hong, Gaetano Paolino, Borislav C. Rusev, Giovanni Marchegiani, Roberto Salvia, Stefano Andrianello, Paola Capelli, Paola Piccoli, Claudia Parolini, Aldo Scarpa, Rita T. Lawlor, Claudio Luchini
Format: Article
Language:English
Published: MDPI AG 2020-08-01
Series:International Journal of Molecular Sciences
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Online Access:https://www.mdpi.com/1422-0067/21/16/5794
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author Paola Mattiolo
Seung-Mo Hong
Gaetano Paolino
Borislav C. Rusev
Giovanni Marchegiani
Roberto Salvia
Stefano Andrianello
Paola Capelli
Paola Piccoli
Claudia Parolini
Aldo Scarpa
Rita T. Lawlor
Claudio Luchini
author_facet Paola Mattiolo
Seung-Mo Hong
Gaetano Paolino
Borislav C. Rusev
Giovanni Marchegiani
Roberto Salvia
Stefano Andrianello
Paola Capelli
Paola Piccoli
Claudia Parolini
Aldo Scarpa
Rita T. Lawlor
Claudio Luchini
author_sort Paola Mattiolo
collection DOAJ
description The intraductal oncocytic papillary neoplasm (IOPN) of the pancreas has been recognized by WHO classification as a unique intraductal papillary mucinous neoplasm (IPMN) category. IOPN is composed of oxyphil cells, usually expressing MUC5AC, MUC6, and Hep Par-1, and harboring <i>PRKACA</i>/<i>B</i> fusion genes as their genetic hallmark. Although IOPNs are associated with an infiltrative adenocarcinoma in up to 30% of cases, the survival rate after surgical resection approaches 100%. This highlights the importance of the correct IOPN diagnosis, above all in cases with an associated invasive component. In this study, the immunohistochemical expression of CD117 was investigated in 111 IPMNs, including 17 oncocytic, 45 gastric, 20 pancreatico-biliary, and 29 intestinal IPMNs. We also tested the expression of MUC5AC, MUC6, and Hep Par-1 in the IOPN cohort. CD117 positivity was significantly more frequent in IOPNs compared to the other IPMN subtypes (<i>p</i> < 0.0001). Furthermore, within IOPN, a lower or absent CD117, MUC5AC, MUC6, and Hep Par-1 expression tended to be associated with the presence of an infiltrative component. Our findings shed light into the biology of these complex lesions, which are confirmed to be a distinctive IPMN subtype; notably, CD117 emerged as a potential, additional tool in the differential diagnosis of IPMNs.
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spelling doaj.art-4b83fad6bb66430e9b1a610cd9d0acf02023-11-20T09:56:46ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672020-08-012116579410.3390/ijms21165794CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic SubtypePaola Mattiolo0Seung-Mo Hong1Gaetano Paolino2Borislav C. Rusev3Giovanni Marchegiani4Roberto Salvia5Stefano Andrianello6Paola Capelli7Paola Piccoli8Claudia Parolini9Aldo Scarpa10Rita T. Lawlor11Claudio Luchini12Department of Diagnostics and Public Health, Section of Pathology, University of Verona, 37134 Verona, ItalyDepartment of Pathology, ASAN Medical Center, Seoul 05505, KoreaDepartment of Diagnostics and Public Health, Section of Pathology, University of Verona, 37134 Verona, ItalyDepartment of Diagnostics and Public Health, Section of Pathology, University of Verona, 37134 Verona, ItalyDepartment of Surgery, the Pancreas Institute, University and Hospital Trust of Verona, 37134 Verona, ItalyDepartment of Surgery, the Pancreas Institute, University and Hospital Trust of Verona, 37134 Verona, ItalyDepartment of Surgery, the Pancreas Institute, University and Hospital Trust of Verona, 37134 Verona, ItalyDepartment of Diagnostics and Public Health, Section of Pathology, University of Verona, 37134 Verona, ItalyDepartment of Diagnostics and Public Health, Section of Pathology, University of Verona, 37134 Verona, ItalyDepartment of Diagnostics and Public Health, Section of Pathology, University of Verona, 37134 Verona, ItalyDepartment of Diagnostics and Public Health, Section of Pathology, University of Verona, 37134 Verona, ItalyARC-Net Research Center, University and Hospital Trust of Verona, 37134 Verona, ItalyDepartment of Diagnostics and Public Health, Section of Pathology, University of Verona, 37134 Verona, ItalyThe intraductal oncocytic papillary neoplasm (IOPN) of the pancreas has been recognized by WHO classification as a unique intraductal papillary mucinous neoplasm (IPMN) category. IOPN is composed of oxyphil cells, usually expressing MUC5AC, MUC6, and Hep Par-1, and harboring <i>PRKACA</i>/<i>B</i> fusion genes as their genetic hallmark. Although IOPNs are associated with an infiltrative adenocarcinoma in up to 30% of cases, the survival rate after surgical resection approaches 100%. This highlights the importance of the correct IOPN diagnosis, above all in cases with an associated invasive component. In this study, the immunohistochemical expression of CD117 was investigated in 111 IPMNs, including 17 oncocytic, 45 gastric, 20 pancreatico-biliary, and 29 intestinal IPMNs. We also tested the expression of MUC5AC, MUC6, and Hep Par-1 in the IOPN cohort. CD117 positivity was significantly more frequent in IOPNs compared to the other IPMN subtypes (<i>p</i> < 0.0001). Furthermore, within IOPN, a lower or absent CD117, MUC5AC, MUC6, and Hep Par-1 expression tended to be associated with the presence of an infiltrative component. Our findings shed light into the biology of these complex lesions, which are confirmed to be a distinctive IPMN subtype; notably, CD117 emerged as a potential, additional tool in the differential diagnosis of IPMNs.https://www.mdpi.com/1422-0067/21/16/5794pancreasintraductalIOPNIPMNoncocyticc-kit
spellingShingle Paola Mattiolo
Seung-Mo Hong
Gaetano Paolino
Borislav C. Rusev
Giovanni Marchegiani
Roberto Salvia
Stefano Andrianello
Paola Capelli
Paola Piccoli
Claudia Parolini
Aldo Scarpa
Rita T. Lawlor
Claudio Luchini
CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype
International Journal of Molecular Sciences
pancreas
intraductal
IOPN
IPMN
oncocytic
c-kit
title CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype
title_full CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype
title_fullStr CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype
title_full_unstemmed CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype
title_short CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype
title_sort cd117 is a specific marker of intraductal papillary mucinous neoplasms ipmn of the pancreas oncocytic subtype
topic pancreas
intraductal
IOPN
IPMN
oncocytic
c-kit
url https://www.mdpi.com/1422-0067/21/16/5794
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