title: Primary small-bowel malignancy in the UK and its association with coeliac disease.
abstract: BACKGROUND
  Malignancies of the small intestine are rare, accounting for <2% of all cancers of the gastrointestinal tract. There is little information about the presentation and prognosis of these tumours, and the frequency of established risk factors.
  
  
  OBJECTIVE
  To estimate the frequency of small-bowel malignancy in the UK, and its relationship to the presence of coeliac disease.
  
  
  METHODS
  Survey of clinicians registered with the British Society of Gastroenterology.
  
  
  METHODS
  Data were collected monthly from June 1998 to May 2000. Clinicians (n=1327) were asked by post to report newly diagnosed cases of primary small-bowel malignancy. A form was sent to reporting clinicians, requesting an anonymous identifier, type of malignancy, and whether coeliac disease was present. A detailed questionnaire followed, requesting further clinical and pathological details.
  
  
  RESULTS
  Clinico-pathological data were ascertained for 395 cases, including 175 adenocarcinomas, 107 lymphomas and 79 carcinoid tumours. In 13% of adenocarcinoma cases and in 39% of lymphomas, there was a diagnosis of coeliac disease. Survival rates at 30 months for adenocarcinomas, lymphomas and carcinoid tumours were 58%, 45% and 78%, respectively. Prognosis of all tumours was inversely related to stage at presentation, and lymphomas associated with coeliac disease were associated with a poorer prognosis.
  
  
  CONCLUSIONS
  This study provides additional evidence that coeliac disease confers susceptibility to adenocarcinoma of the small bowel, as well as lymphoma. The long time from the onset of symptoms to diagnosis of small bowel tumours is of concern, as this delay is reflected in the high proportion that presented with metastatic disease. Although the absolute risk of malignancy is small, coeliac disease complicated by malignancy appears to be poorly controlled.
authors.affiliation: Academic Unit of Medicine, Clinical Sciences Building, St. James's University Hospital, Leeds, UK. P.D.Howdle@leeds.ac.uk
authors.forename: P D
authors.initials: PD
authors.lastname: Howdle
authors.forename: P K
authors.initials: PK
authors.lastname: Jalal
authors.forename: G K T
authors.initials: GK
authors.lastname: Holmes
authors.forename: R S
authors.initials: RS
authors.lastname: Houlston
mesh_terms: D000230:Adenocarcinoma / Q000453:epidemiology / Q000209:etiology* / Q000235:genetics; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002446:Celiac Disease / Q000150:complications* / Q000453:epidemiology; D004198:Disease Susceptibility; D005260:Female; D006306:Health Surveys; D006801:Humans; D007414:Intestinal Neoplasms / Q000453:epidemiology / Q000209:etiology* / Q000235:genetics; D008297:Male; D008875:Middle Aged; D011230:Precancerous Conditions; D011446:Prospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D006113:United Kingdom / Q000453:epidemiology