coeliac disease ( cd ) , also called gluten - sensitive enteropathy or nontropical sprue , is a unique autoimmune disorder which results from the interaction between gluten and immune , genetic and environmental factors . originally cd was considered as a malabsorption syndrome of childhood , whereas it is now recognized as a disease which may be diagnosed at any age . clinical manifestations of cd differ greatly among patients , varying from vague symptoms such as fatigue or malaise to a classical malabsorption syndrome including diarrhoea and steatorrhoea accompanied by abdominal pain or discomfort . the cornerstone of the treatment of cd is a lifelong gluten - free diet ( gfd ) which leads to noticeable clinical improvement within two weeks in about 70% of patients . several studies have suggested increased mortality from gastrointestinal malignancies in patients with cd compared to the general population [ 1 , 4,5,6 ] . particularly the risk of small bowel malignancies , which is generally an uncommon form of cancer , is increased . in september 2008 , a 70-year - old caucasian male was referred by his general practitioner due to malaise , nausea , vomiting and weight loss of about 8 kg in two months . in his medical history , the patient had undergone a hartmann procedure in 1998 because of a perforated diverticulitis . in 1999 a reversal of the hartmann procedure had occurred . in 2001 the patient had been diagnosed with cd by an esophagogastroduodenoscopy with duodenal biopsies for which he was treated with a gfd on which he responded well clinically and which he followed meticulously . at admission , we performed an esophagogastroduodenoscopy which showed distention of the stomach and duodenum with retention of gastric and enteric fluid ; more than 500 ml was aspirated . at the point of maximal intubation in the pars horizontalis , a distinct obstruction was suggestive with kinking and fixation but without evident pathology of the mucosa . biopsies were taken but probably not at the point of maximum obstruction , which could not be reached because of kinking . histology showed a normal mucosal membrane of the duodenum , without villous atrophy or increased intraepithelial lymphocytes . additionally , a computed tomography scan of the abdomen was performed which showed an expanded stomach and duodenum up to the ligament of treitz . an explorative laparotomy was performed , during which a small tumor was palpated near the ligament of treitz . histological analyses revealed a moderately differentiated , infiltrating adenocarcinoma of 1.5 cm penetrating the muscularis propria and serosa , without invasion of local lymph nodes ( stage t3n0m0 ajcc vi ed 2002 ) ( fig . 1 , fig malignancies involving the small bowel occur very infrequently in the general population . in the netherlands , less than 100 cases a year of small bowel malignancies are revealed while at least 10,000 colorectal carcinomas are diagnosed . despite the fact that the small bowel represents about 75% of the length and 90% of the surface area of the gastrointestinal tract , next to the rarity of these lesions , the signs and symptoms patients present are usually vague and poorly defined , often delaying a correct diagnosis . furthermore , conventional radiographic studies of the upper and lower intestinal tract often appear normal . the association between carcinoma of the small bowel and cd was first reported in 1958 . since then , the association between cd and small bowel carcinoma is persistent although only based on a small number of patients of which the greater part is in the form of case reports . swinson et al . indicated that cd patients have an increased risk of developing small bowel adenocarcinoma ( relative risk 82.6-fold ) . in addition , results of a national survey in the united states revealed similar results ( relative risk 67-fold ) . a large population - based study of malignancy in patients which cd performed in sweden confirmed the increased risk for small bowel cancer , although the risk was less compared to previous studies ( 10-fold ) . possibilities include immunologic disturbance associated with mucosal lymphocyte infiltration , premalignant changes in the damaged surface epithelial cells , increased permeability to oncogenic factors and malabsorption of protective substances such as vitamins a and e [ 10 , 11 ] . another hypothesis which has been postulated is that small bowel adenocarcinoma in cd arises through an adenoma - carcinoma sequence . however , currently there are conflicting results regarding this hypothesis . despite the fact that a lifelong gfd leads to noticeable clinical and histological improvement in about 70% of patients , studies about the protective effect of gfd on malignancy are scarce . most studies suggest that a gfd leads to a decreased incidence of lymphoma , cancer of the mouth , pharynx and oesophagus and a decreased mortality in cd . however , according to our knowledge only one case report was published about the specific role of gfd concerning small bowel malignancy . this report did not show a protective effect of gfd , however , further research is needed to confirm this hypothesis . in conclusion , the present patient is an illustration that cd is a serious disease with possible serious complications such as duodenal adenocarcinoma . despite the association between cd and small bowel malignancy , currently there are no recommendations for screening for this type of malignancy nor specific protocols for the follow - up of patients with cd . in view of our finding and based on the present scientific literature , we recommend that clinicians should take into account the possibility of small bowel malignancy in cd patients with vague symptoms such as fatigue or malaise , especially in combination with weight loss , despite a well - maintained gfd . in these patients