Giuffrida Paolo, Vanoli Alessandro, Arpa Giovanni, Bonometti Arturo, Luinetti Ombretta, Solcia Enrico, Corazza Gino Roberto, Paulli Marco, Di Sabatino Antonio
First Department of Internal Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy.
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy.
Cancers (Basel). 2018 Dec 29;11(1):31. doi: 10.3390/cancers11010031.
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.
小肠癌（SBC）是罕见的肿瘤，其诱发因素包括遗传综合征以及免疫介导的肠道疾病，如乳糜泻（CD）和克罗恩病（CrD）。尽管与CD相关的SBC（CD-SBC）和与CrD相关的SBC（CrD-SBC）均起源于炎症背景，但它们在肿瘤细胞表型、微卫星不稳定性频率、核β-连环蛋白表达以及预后方面存在显著差异。对于这些患者，高肿瘤浸润淋巴细胞密度和腺管/髓样组织学类型是独立的阳性预后因素。SBC附近的发育异常罕见，在CD中表现为肠道表型和核β-连环蛋白，而在CrD中则较为常见，表现为胃肠胰胆管标志物表达以及膜性β-连环蛋白保留。最近的证据表明，尽管罕见，但爱泼斯坦-巴尔病毒阳性的发育异常和SBC确实存在，且与CrD相关。在本综述中，我们总结了具有临床和治疗意义的新的病理学和分子学见解，以指导CD-SBC和CrD-SBC的治疗。
