Although small intestinal cancer is rare in the general population, certain diseases and conditions can predispose an individual to small intestinal cancers. Celiac disease is an autoimmune disease caused by a reaction to gluten proteins that are present in certain foods like wheat, barley, and oats in genetically predisposed individuals. A question comes in mind of people “ does celiac disease cause cancer?”
A certain number of patients with celiac disease don’t heal completely even after going gluten-free. Healing is not Universal in people with celiac disease, adults people diagnosed with celiac disease don’t heal their intestine for almost about 2 years since started the gluten-free diet.
Potential reasons for no healing or slow healing:
- It might be a low-level gluten condition in the diet
- A slow healing in the intestine
- A condition that affects people who had celiac disease a long time or not diagnosed earlier.
Types of Carcinomas or Cancer associated with Celiac Disease
Three types of carcinomas are mainly associated with celiac disease;
- Adenocarcinoma of the small intestine
- Enteropathy associated T cell lymphoma (EATL).
In addition to these, esophageal, oropharyngeal adenocarcinoma, and some non-gastrointestinal tumors have also been reported. Lymphomas are the most common type, followed by small bowel adenocarcinoma (10). Usually, small bowel adenoma develops most commonly in duodenum 55%, jejunum 30%, and ileum 15%. However, in celiac disease, the most common site is jejunum.
They are characterized by malignant proliferation of lymphocytes, mainly in lymph nodes or lymphatic aggregations in the small bowel wall. They are of two types;
- Hodgkin’s lymphoma, which is characterized by Reed Sternberg cells and inflammatory cells, and comprises about 12 % of cases.
- Non-Hodgkin’s, that is more common lymphoma. Its sub type of T-cell lymphoma is the most aggressive, and the leading cause of death in elderly patients with a two-year survival rate is 15-20%.
Lymphoma associated with celiac disease appears most commonly at extra nodal sites such as small bowel. And it should also be noted that t cell lymphomas outside the small intestine may also be associated with celiac disease. In research conducted under the British society of Gastroenterology research unit, it is clear that the association of the celiac disease with small bowel carcinomas is higher than inflammatory bowel disease. In the same study, the celiac disease was diagnosed before lymphoma in 59% of cases.
It occurs by malignant proliferation of glandular epithelium and generally very aggressive and rapidly spreading. But if removed at an early stage, survival and prognosis are better than compared to those patients suffering from celiac disease(15–17). This type of cancer associated with celiac disease develops by adenoma-carcinoma sequence(18 19). Chronic inflammation leads to malabsorption of nutrients and vitamins, and damage to mucosal barriers causes increased permeability of carcinogens inside the body (20). In addition to small intestinal carcinomas, esophageal and oropharyngeal carcinomas have also been reported in several studies (17,21,22). In a review, 63% of cases of adenocarcinoma had previously been diagnosed with celiac disease (14).
Enteropathy associated T cell lymphoma (EATL) or Other carcinomas
In addition to gastrointestinal cancers, liver, gallbladder, pancreatic, and some non-gastrointestinal tumors, germ cell malignancies, astrocytoma(brain cell tumor), and Wilms tumor(kidney tumor in children), have also been reported in the association of celiac disease (9,23,24).
It is worth mentioning here that not all the patients of the celiac disease develop cancer. The majority of patients don’t develop cancer if diagnosed at an early stage, and there is a recommendation of precautionary measures. Signs and symptoms of cancer are fever, weight loss, fatigue, abdominal pain, enlarged lymph nodes, and gastrointestinal upset (25). Many factors play an essential role in the development of gastrointestinal cancers, among them, diet and environmental factors have as much significance as genetic factors (26,27).
For more information on Celiac Disease Risk Factors
Diet: The food we eat has a significant impact on gastrointestinal cancer development. Here, in this case, gluten, which is present in wheat, rye, and barley, is a crucial component for disease pathophysiology as well as complications (2). It is strictly advised to celiac disease patients to avoid any food containing gluten. It has been shown in many studies if patients have a gluten-free diet, the risk of disease severity as well as malignant complications, can be prevented (28,29).
For more information on What to Eat with Celiac Disease
Early diagnosis: There is a high risk of developing cancer if the disease is diagnosed very late in the course, and it is directly associated with the age of the patient (6). It is evident from studies that two significant factors of developing cancer in celiac disease are delay in diagnosis and no adherence to gluten-free diet (6,30).
For more information on What to Eat with Celiac Disease
No consensus treatment has been documented yet because of the rarity of the disease. So currently, surgery and complete resection is only available treatment with a cure rate of 40-65%. In the case of unresectable tumor or metastasis(spread of cancer to other areas), there is no recommendation of surgery except in emergencies where multidisciplinary evaluation is required30. Adjuvant radiotherapy and chemotherapy are under consideration, and other options are being explored based on available data on colon carcinoma (14,30,31).
How to prevent complications
Small bowel adenocarcinoma associated with celiac disease arises from a premalignant adenoma through adenoma-carcinoma sequence. Unlike other causes of this cancer, celiac disease does not show a typical premalignant mucosal dysplasia or other defects (32–34). So it mostly presents at an advanced stage where it has already metastasized (35). Diagnosis at an initial stage can make it treatable by primary resection (surgery), and a lot of complications don’t occur. Earlier diagnosis has a better prognosis.
The other aspect is disease severity and duration. Currently, no medication is available to prevent complications of celiac disease, and a Gluten-free diet is only an effective management option that is presently available36. Education should be given to the patients to avoid any food containing gluten altogether. Non-adherence to dietary precautions leads to a prolonged inflammatory and autoimmune response, which eventually ends up developing several cancers.
Celiac disease is an immunogenic disease. Delay in diagnosis and non-adherence to a gluten-free diet make the patients of celiac disease predisposed to several types of cancers, most commonly small bowel cancers.
These cancers are diagnosed at late stages with poor outcomes. As there is no cure available, so the importance of prompt diagnosis and strict adherence to a gluten-free diet can not be denied. Only early stages of carcinomas associated with celiac disease are possible further studies are required to manage these conditions.
Celiac disease is a well-established risk factor for small intestinal adenocarcinoma and lymphoma, according to researches (3–5). Delayed diagnosis and prolonged course of the celiac disease are associated with the development of small intestine cancer in celiac patents, so it is imperative to diagnose it at an early age and take precautionary measures to prevent complications6.