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Review
. 2022 Dec 12;16(1):460.
doi: 10.1186/s13256-022-03682-3.

Malignant complications of celiac disease: a case series and review of the literature

Barbora Packova  1 , Pavel Kohout  2 , Milan Dastych  1 , Jitka Prokesova  1 , Tomas Grolich  3 , Radek Kroupa  4
Affiliations
Review

Malignant complications of celiac disease: a case series and review of the literature

Barbora Packova et al. J Med Case Rep. .

Abstract

Background: Celiac disease is an immune-mediated enteropathy triggered by gluten in genetically susceptible individuals. Diagnosis is based on evaluating specific autoantibodies and histopathologic findings of duodenal biopsy specimens. The only therapy for celiac disease is a gluten-free diet. Celiac disease can be complicated by malnutrition, other autoimmune diseases, refractoriness to treatment, and gastrointestinal tumors. This article presents seven cases of malignancies in patients with celiac disease. Its objective is to raise awareness of the malignant complications of celiac disease, leading to earlier diagnosis and improved outcomes.

Case presentation: Seven cases of malignant complications of celiac disease occurred among 190 patients followed at the Department of Internal Medicine and Gastroenterology, University Hospital Brno from 2014 to 2021. We describe these cases and the presentation, diagnostic process, course, management, and outcomes for each, along with proposed potential risk factors of malignant complications. There was one Caucasian man who was 70 years old and six Caucasian women who were 36, 46, 48, 55, 73, and 82 years old in our cohort. Of the seven cases of malignancies in our cohort, four patients were diagnosed with small bowel adenocarcinoma, one with diffuse large B-cell lymphoma, one with carcinoma of the tongue, and one with colorectal carcinoma.

Conclusions: Malignancies occurred in 3.7% of patients followed up for celiac disease. Awareness of the malignant complications of celiac disease, risk factors, presentation, and disease course could lead to earlier diagnosis and improved outcomes.

Keywords: Carcinoma; Case report; Celiac disease; Complication; Lymphoma; Malignancy.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Gastroscopic view of adenocarcinoma of proximal jejunum. Provided by Radek Kroupa M.D. from archive of Department of Internal Medicine and Gastroenterology, University Hospital Brno
Fig. 2
Fig. 2
Adenocarcinoma of the oral jejunum, intestinal type (right). Intestinal villi without atrophy, but with increased number of intraepithelial lymphocytes (left). Hematoxylin + eosin, 200×. Provided by Leos Kren, Ass. Prof., M.D., Ph.D. from archive of Department of Pathology, University Hospital Brno
Fig. 3
Fig. 3
Small bowel adenocarcinoma in view by video capsule enteroscopy. Provided by Milan Dastych M. D. from archive of Department of Internal Medicine and Gastroenterology, University Hospital Brno
Fig. 4
Fig. 4
Perioperative view of infiltration of jejunum (green arrow) with the lodged capsule (blue arrow). Provided by Tomas Grolich M. D., Department of Surgery, University Hospital Brno
Fig. 5
Fig. 5
Gastroscopic view of diffuse large B-cell lymphoma infiltrating duodenal apex. Provided by Milan Dastych M. D. from archive of Department of Internal Medicine and Gastroenterology, University Hospital Brno
Fig. 6
Fig. 6
Biopsy of the duodenum, villi are flattened with intraepithelial lymphocytes. Regionally there are aggregates of large centrocytes in the lamina propria: infiltration by diffuse large B-cell lymphoma; hematoxylin + eosin, 200× (left). The large centrocytes stain with anti-CD 20 antibody; immunohistochemistry, 200× (right). Provided by Leos Kren, Ass. Prof., M.D., Ph.D. from archive of Department of Pathology, University Hospital Brno
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References

    1. van Gils T, Nijeboer P, Overbeek LI, et al. Risks for lymphoma and gastrointestinal carcinoma in patients with newly diagnosed adult-onset celiac disease: consequences for follow-up. United Eur Gastroenterol J. 2018;6(10):1485–1495. doi: 10.1177/2050640618800540. - DOI - PMC - PubMed
    1. Catassi C, Fabiani E, Corrao G, et al. Risk of non-Hodgkin lymphoma in celiac disease. JAMA. 2002;287(11):1413–1419. doi: 10.1001/jama.287.11.1413. - DOI - PubMed
    1. Smedby KE. Malignant lymphomas in coeliac disease: evidence of increased risks for lymphoma types other than enteropathy-type T cell lymphoma. Gut. 2005;54(1):54–59. doi: 10.1136/gut.2003.032094. - DOI - PMC - PubMed
    1. Halfdanarson TR, Rubio-Tapia A, Ristow KM, Habermann TM, Murray JA, Inwards DJ. Patients with celiac disease and B-cell lymphoma have a better prognosis than those with T-cell lymphoma. Clin Gastroenterol Hepatol. 2010;8(12):1042–1047. doi: 10.1016/j.cgh.2010.09.007. - DOI - PMC - PubMed
    1. Green PHR, Fleischauer AT, Bhagat G, Goyal R, Jabri B, Neugut AI. Risk of malignancy in patients with celiac disease. Am J Med. 2003;115(3):191–195. doi: 10.1016/S0002-9343(03)00302-4. - DOI - PubMed