The Risk of Malignancies in Celiac Disease—A Literature Review

SIMPLE SUMMARY: Celiac disease (CeD) is an multiorgan autoimmune disease precipitated by the ingestion of gluten in genetically predisposed individuals. After the initiation of a gluten-free diet, CeD generally has a benign course, with the complete remission of symptoms and a normal life expectancy; however, robust evidence suggests that subjects with CeD are at increased risk of developing malignancies compared to the general population. Peculiar associations with lymphomas, including enteropathy-associated T-cell lymphoma (EATL), and small bowel carcinoma (SBC), as well as correlations with other cancers, have been thoroughly investigated. In this review, we will examine the risk of developing malignancies in patients with CeD, as well as clinical aspects of and therapeutic options for EATL and SBC. ABSTRACT: Celiac disease (CeD) is an immune-mediated enteropathy precipitated by ingestion of gluten in genetically predisposed individuals. Considering that CeD affects approximately 1% of the Western population, it may be considered a global health problem. In the large majority of cases, CeD has a benign course, characterized by the complete resolution of symptoms and a normal life expectancy after the beginning of a gluten-free-diet (GFD); however, an increased risk of developing malignancies, such as lymphomas and small bowel carcinoma (SBC), has been reported. In particular, enteropathy-associated T-cell lymphoma (EATL), a peculiar type of T-cell lymphoma, is characteristically associated with CeD. Moreover, the possible association between CeD and several other malignancies has been also investigated in a considerable number of studies. In this paper, we aim to provide a comprehensive review of the current knowledge about the associations between CeD and cancer, focusing in particular on EATL and SBC, two rare but aggressive malignancies.