Intussusception and metastatic colon cancer in an adult with cystic fibrosis

Abstract

Cystic fibrosis (CF) is a true multi-system disease and as life expectancy continues to increase, the spectra of symptoms and diagnoses seen in patients is ever expanding. Some of the conditions that are now seen in CF are specific to the underlying disease process, others are side-effects of long term medications and some are simply related to patients older age. Cancers of the digestive tract were previously extremely rare in patients with CF as they were unlikely to live to an age at which they would present. Over recent years they have been seen more frequently and research has shown that their incidence is higher in patients with CF when compared to the general population.1 In contrast, it has been known for some time that intussusception is more common in children with CF with the peak incidence at 3–12 months of age.2 More recently, in association with patients increased life expectancy, there have been a number of reports of intussusception in older children and adults with CF.3–7 Most of these reports have found the lead point to be inspisated faeces and none have identified a malignant lead point. We present the case of a 35-year-old woman with CF who presented with a sigmoid intussusception caused by 2 malignant polyps. At the time of diagnosis, metastases were identified in 2 local lymph nodes and the liver. As well as being the first reported case of intussusception in a patient with CF in which a malignant lead point has been identified, the case highlights a number of other important clinical, psychological and social issues.