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Review

Small Bowel Carcinomas Associated with Immune-Mediated Intestinal Disorders: The Current Knowledge

1
First Department of Internal Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy
2
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy
*
Author to whom correspondence should be addressed.
Cancers 2019, 11(1), 31; https://doi.org/10.3390/cancers11010031
Received: 19 November 2018 / Revised: 10 December 2018 / Accepted: 24 December 2018 / Published: 29 December 2018
(This article belongs to the Special Issue Colorectal Cancers)
Small bowel carcinomas (SBC) are uncommon neoplasms, whose predisposing conditions include hereditary syndromes and immune-mediated intestinal disorders including coeliac disease (CD) and Crohn’s disease (CrD). Although both CD-associated SBC (CD-SBC) and CrD-associated SBC (CrD-SBC) arise from an inflammatory background, they differ substantially in tumour cell phenotype, frequency of microsatellite instability and nuclear β-catenin expression, as well as in prognosis. For these patients, high tumour-infiltrating lymphocyte density and glandular/medullary histotype represent independent positive prognostic factors. Dysplasia adjacent to SBC is rare and characterized by intestinal phenotype and nuclear β-catenin in CD, while it is frequent and typified by gastro-pancreatobiliary marker expression and preserved membranous β-catenin in CrD. Recent evidence suggests that Epstein-Barr virus-positive dysplasia and SBC, albeit exceptional, do exist and are associated with CrD. In this review, we summarize the novel pathological and molecular insights of clinical and therapeutic interest to guide the care of CD-SBC and CrD-SBC. View Full-Text
Keywords: coeliac disease; Crohn’s disease; dysplasia; histotype; overall survival; tumour-infiltrating lymphocyte coeliac disease; Crohn’s disease; dysplasia; histotype; overall survival; tumour-infiltrating lymphocyte
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MDPI and ACS Style

Giuffrida, P.; Vanoli, A.; Arpa, G.; Bonometti, A.; Luinetti, O.; Solcia, E.; Corazza, G.R.; Paulli, M.; Di Sabatino, A. Small Bowel Carcinomas Associated with Immune-Mediated Intestinal Disorders: The Current Knowledge. Cancers 2019, 11, 31. https://doi.org/10.3390/cancers11010031

AMA Style

Giuffrida P, Vanoli A, Arpa G, Bonometti A, Luinetti O, Solcia E, Corazza GR, Paulli M, Di Sabatino A. Small Bowel Carcinomas Associated with Immune-Mediated Intestinal Disorders: The Current Knowledge. Cancers. 2019; 11(1):31. https://doi.org/10.3390/cancers11010031

Chicago/Turabian Style

Giuffrida, Paolo, Alessandro Vanoli, Giovanni Arpa, Arturo Bonometti, Ombretta Luinetti, Enrico Solcia, Gino R. Corazza, Marco Paulli, and Antonio Di Sabatino. 2019. "Small Bowel Carcinomas Associated with Immune-Mediated Intestinal Disorders: The Current Knowledge" Cancers 11, no. 1: 31. https://doi.org/10.3390/cancers11010031

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