Bowel Adenocarcinoma in a Patient with Cystic Fibrosis

Abstract

Cystic fibrosis (CF) is an autosomal recessive condition affecting one in 2,000 live births in the UK. There are few reports of malignant tumours in this condition probably because, until recently, the majority died before the age of 30 years as a result of recurrent and chronic bronchopulmonary infection with impaired growth and development and resistance to infection due to pancreatic malabsorption. We describe an adult male with CF who died from an adenocarcinoma affecting the ileocaecal region of the bowel.

A cohort study of cystic fibrosis and malignancy

“…Adenocarcinoma of the terminal ileum is a rare tumour which has previously been reported in three patients with cystic fibrosis from other CF centres (Roberts et al, 1986;Siraganian et al, 1987;Swender et al, 1982). The single case which occurred in our cohort was a highly significant finding (P = 0.003).…”

“…Malignancies occurring in cystic fibrosis are rare and we can find only 19 cases reported in the literature; six cases of leukaemia (Miller, 1969;Cole & Pullen, 1970;Biggs et al, 1986), one case of Hodgkins disease (Marra et al, 1990), four cases of adenocarcinoma of the terminal ileum (Roberts et al, 1986;Siraganian et al, 1987;Swender et al, 1982;Redington et al, 1985), two nephroblastomas (Wilms' tumour) (Miller, 1969), three cases of adenocarcinoma of the pancreas (Tedesco et al, 1986;Davis & Sawicka, 1985;McIntosh et al, 1988), a case of retinoblastoma (Gorvoy, 1981) and two cholangiocarcinomas (Abdul-Karim et al, 1982;Stern et al, 1986). While suggestive of a relationship between CF and malignancy, these case reports do not allow the strength of the association to be quantified.…”

“…A cohort study of (Abdul-Karim et al, 1982;Biggs et al, 1986;Cole & Pullen, 1970;Davis & Sawicka, 1985;Gorvoy, 1981;Marra et al, 1990;McIntosh et al, 1988;Miller, 1969;Redington et al, 1985;Roberts et al, 1986;Siraganian et al, 1987;Stern et al, 1986;Swender et al, 1982;Tedesco et al, 1986 (World Health Organisation, 1967;World Health Organisation, 1977). Overall mortality in the cohort was analysed in a similar way to give a standardised mortality ratio (SMR), which compared observed deaths to age-, sex-, and calendar year-specific expectations based on national mortality rates.…”

The Risk of Cancer among Patients with Cystic Fibrosis

“…[2][3][4][5][6][7][8][9][10][11][12][13][14][15] To evaluate this risk, we conducted a retrospective cohort study of the occurrence of cancer in more than 25,000 patients with cystic fibrosis who were followed at centers in the United States or Canada, and we studied the distribution of cancer in more than 18,000 patients with cystic fibrosis from 17 European countries.…”

The risk of gastrointestinal malignancies in cystic fibrosis

“…CF has been clearly recognized as a risk factor for developing small bowel cancer [5]. There have been some reports specifically of ileal cancers in CF patients and unfortunately in these cases the lesion was not noted until autopsy or late in the disease process [6][7][8].…”