Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

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

Paolo Giuffrida
ORCID logo ,
Alessandro Vanoli
ORCID logo ,
Giovanni Arpa
,
Arturo Bonometti
,
Enrico Solcia
,
Gino Roberto Corazza
,
Marco Paulli
,
Antonio Di Sabatino
* ORCID logo
Version 1 : Received: 19 November 2018 / Approved: 20 November 2018 / Online: 20 November 2018 (16:43:14 CET)

A peer-reviewed article of this Preprint also exists.

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. 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.

Abstract

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 tumor-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.

Keywords

coeliac disease; Crohn’s disease; dysplasia; histotype; overall survival; tumor infiltrating lymphocyte.

Subject

Medicine and Pharmacology, Gastroenterology and Hepatology

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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