TY - JOUR
T1 - Small bowel carcinomas associated with immune-mediated intestinal disorders
T2 - The current knowledge
AU - Giuffrida, Paolo
AU - Vanoli, Alessandro
AU - Arpa, Giovanni
AU - Bonometti, Arturo
AU - Luinetti, Ombretta
AU - Solcia, Enrico
AU - Corazza, Gino Roberto
AU - Paulli, Marco
AU - Di Sabatino, Antonio
PY - 2019/1/1
Y1 - 2019/1/1
N2 - 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.
AB - 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.
KW - Coeliac disease
KW - Crohn’s disease
KW - Dysplasia
KW - Histotype
KW - Overall survival
KW - Tumour-infiltrating lymphocyte
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U2 - 10.3390/cancers11010031
DO - 10.3390/cancers11010031
M3 - Review article
AN - SCOPUS:85061966953
VL - 11
JO - Cancers
JF - Cancers
SN - 2072-6694
IS - 1
M1 - 31
ER -