Colon Cancer in Cystic Fibrosis patients: Is this a growing problem?

In the last decade, we have found an increased incidence of gastrointestinal (GI) malignancies among Cystic Fibrosis (CF) patients. We discuss the case of a 30-year-old woman of mixed descent, with CF, who was referred to the emergency department for persistent abdominal pain and constipation, complicated by blood-streaked stools. On physical examination, her abdomen was soft, nontender, and without organomegaly or palpable masses. Rectal examination was unrevealing. The patient's initial laboratory evaluation revealed a hemoglobin level of 11.7g/dl. A CT scan of the abdomen was unremarkable. A colonoscopy performed one day after admission showed an intraluminal mass in the proximal Sigmoid Colon, from which biopsies were taken. Pathology reports subsequently revealed invasive Adenocarcinoma of the Colon. The patient underwent tumor resection followed by initiation of chemotherapy. The pathophysiology underlying the increased risk of GI cancers in CF patients is unknown. Possible explanations include genetic mutations or persistent pathologic alterations in digestive tract structures. It is our hope that this case will bring to light the need to further delineate cancer risk among CF patients and to develop screening recommendations tailored to this population.