Gastrointestinal malignancy in cystic fibrosis

Paediatr Respir Rev. 2020 Sep:35:90-92. doi: 10.1016/j.prrv.2020.03.002. Epub 2020 Apr 9.

Abstract

Cystic fibrosis (CF) is a multisystem disease affecting the gastrointestinal (GI) tract as well as the lungs. As survival has increased significantly over the past few decades, complications not seen previously have become apparent. There is an overall increased rate of malignancy in CF, particularly from the GI tract and in the post-transplant population. The most common sites of malignancy are the pancreatico-biliary and digestive tract, as well as an increased rate of testicular cancer. Using an illustrative case of metastatic oesophageal malignancy which initially appeared to be hepatic in origin, we have reviewed the literature surrounding malignancy in CF with a particular focus on the GI tract.

Keywords: Cancer screening; Colon cancer; Cystic fibrosis; Metastases; Oesophageal cancer.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adenocarcinoma, Clear Cell / complications
  • Adenocarcinoma, Clear Cell / diagnosis*
  • Adenocarcinoma, Clear Cell / drug therapy
  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Bone Neoplasms / diagnosis*
  • Bone Neoplasms / secondary
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / epidemiology*
  • Esophageal Neoplasms / complications
  • Esophageal Neoplasms / diagnosis*
  • Esophageal Neoplasms / drug therapy
  • Fatal Outcome
  • Fluorouracil / therapeutic use
  • Gastrointestinal Neoplasms / epidemiology*
  • Humans
  • Leucovorin / therapeutic use
  • Liver Neoplasms / diagnosis*
  • Liver Neoplasms / secondary
  • Male
  • Organoplatinum Compounds / therapeutic use

Substances

  • Organoplatinum Compounds
  • Leucovorin
  • Fluorouracil

Supplementary concepts

  • Folfox protocol