Clinical Characteristics and Specific Management Considerations in Children With Cystic Fibrosis and Childhood Cancer: Case Series and Review of the Literature.

Background: Cystic fibrosis (CF) is an autosomal recessive inherited chronic disorder that predisposes adults to gastrointestinal malignancies. The latter has not been observed in children, perhaps due to the limited documentation of the copresentation of childhood cancer in CF. The following review will provide a comprehensive overview of the relevant literature on this subject.

Methodology: The objective of this study was to identify cases in Belgium over a 25-year period and to review the literature for similar cases. Our secondary objective was to identify areas of attention during oncological management based on the clinical cases and extant literature.

Results: Four cases were diagnosed in Belgium and a further 10 cases were identified in the literature. These fourteen cases exhibited a 1:1 male-to-female ratio. Four cases were hematological malignancies, and 10 cases were solid tumors, predominantly neuroblastomas and osteosarcomas. No specific age group predominated at the time of diagnosis of the malignancy. As the literature indicated that organs were at risk in all systems, at increased risk for treatment toxicities, and a need for increased nutritional and antimicrobial support. The threshold for intervention with supportive care should be substantially lower than in non-CF patients. These patients are predisposed to an increased risk for premature morbidity and mortality.

Conclusion: Short and long-term CF-specific survivorship care plans should be developed based on both disease entities in anticipation of compounded risks for early cardio-respiratory disease and an increased awareness of secondary cancer screening. Research into the copresentation of these diseases should be promoted.