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 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.
Highlights
Small bowel carcinomas (SBC) are remarkably uncommon neoplasms, mostly sporadic.Notwithstanding, several predisposing conditions such as hereditary syndromes –familial adenomatous polyposis, Lynch syndrome, Peutz-Jeghers syndrome, and juvenile polyposis syndromeand chronic immune-mediated intestinal disorders—coeliac disease (CD) and Crohn’s disease (CrD)—are well-known (Table 1) [1]
Small bowel carcinoma associated with coeliac disease (CD-SBC)
Survival is worse in CrD-associated SBC (CrD-SBC) than in colorectal cancer complicating CrD
Summary
Small bowel carcinomas (SBC) are remarkably uncommon neoplasms, mostly sporadic. Notwithstanding, several predisposing conditions such as hereditary syndromes –familial adenomatous polyposis, Lynch syndrome, Peutz-Jeghers syndrome, and juvenile polyposis syndromeand chronic immune-mediated intestinal disorders—coeliac disease (CD) and Crohn’s disease (CrD)—are well-known (Table 1) [1]. The underlying intestinal disorder, i.e., CD or CrD, has been demonstrated to be a stage-independent prognostic factor in patients undergoing surgery for SBC [2]. Both CD and CrD are sustained by similar pathogenic mechanisms, namely T helper 1 and 17 immune responses [3], CD-associated SBC (CD-SBC) and CrD-associated SBC (CrD-SBC). Most CrD-SBC are microsatellite stable, have low TILs and often show a non-glandular histotype coupled with a non-intestinal phenotype [2,7,8,10,11,12]. Crohn’s disease; EBV, Epstein-Barr Virus; MSI, microsatellite instability; spo-SBC, sporadic small bowel carcinoma; yr, year. We here review the current knowledge of CD-SBC and CrD-SBC by highlighting new molecular insights from clinical perspectives, in particular in predicting outcome and response to novel therapeutic options
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