Malignant Complications of Celiac Disease

A Case Series and Review of the Literature

Barbora Packova; Pavel Kohout; Milan Dastych; Jitka Prokesova; Tomas Grolich; Radek Kroupa

J Med Case Reports. 2022;16(460)

Abstract and Introduction

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.

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Table 1. Summary of the malignancies in celiac disease
Patient	Age at diagnosis of CD, years	Age at diagnosis of malignity, years	Persistent villous atrophy	Anemia	Type of malignity	Stage	Therapy	Outcome
1. Caucasian woman	6	36	Yes	No	SBA	III	Resection, chemotherapy, radiotherapy	Cancer free at 36 months
2. Caucasian woman	82	82	–	Yes	SBA	III	Palliation	Death in 9 months
3. Caucasian man	64	70	No	Yes	SBA	II	Resection, chemotherapy	Cancer free at 50 months
4. Caucasian woman	53	55	No	Yes	SBA	III	Resection, chemotherapy	Death in 22 months
5. Caucasian woman	71	73	Yes	Yes	DLBCL	IVB	Chemotherapy	Cancer free at 45 months
6. Caucasian woman	31	48	No	No	Carcinoma of tongue	IV	Resection, radiotherapy	Cancer free at 20 months
7. Caucasian woman	39	46	No	No	CRCA	III	Resection, chemotherapy	Cancer free at 6 months