Small bowel malignancy in coeliac disease

We were interested to read the case report by Rampertab et al on small bowel neoplasia in coeliac disease( Gut 2003;52:1211–14). The findings are very much in accord with ours from the British Society of Gastroenterology (BSG) National UK Survey published earlier this year.¹ Over a two year period (1998–2000), we collected details of 175 cases of primary small intestinal adenocarcinoma, of which 13% were associated with coeliac disease and another 7% with Crohn’s disease. With regard to coeliac associated adenocarcinomas, similar to Rampertab et al, we found a predominance of males (2:1) and an equal distribution between the duodenum and jejunum. Age range was 47–80 years. Fifty five per cent presented acutely, predominantly with obstruction, and 45% chronically with anaemia, weight loss, or abdominal pain. Mean time of symptoms prior to diagnosis was 14 months, which was reflected in a relatively poor 30 month overall survival of 58%. In 63%, coeliac disease had been diagnosed a mean of 8.2 years prior to the diagnosis of adenocarcinoma; in almost all of these patients there had been a good clinical and mucosal response to a gluten free diet. In 37%, coeliac disease was diagnosed at the same time as adenocarcinoma.

Although 13% of small bowel adenocarcinomas being associated with coeliac disease implies that the risk of these cancers in coeliac disease is very high, such an increase translates into a very small absolute life time risk of less than 1%, as these tumours are rare and coeliac disease is very common. Nevertheless, we agree that coeliac patients require long term follow up for this and other complications. However, the best means of surveillance needs to be determined.

Of most concern is the long delay in the diagnosis of small bowel adenocarcinoma, irrespective of whether or not coeliac disease is present. This leads to poor survival as 40% have metastasised by the time the diagnosis is made. A high index of suspicion is required by all gastroenterologists for this rare, but eminently treatable, type of adenocarcinoma.