What is Known:

• A wide spectrum of rare syndromes manifesting in childhood is associated with an increased cancer risk.

• For many of these syndromes, expert recommendations for management and tumor surveillance are available, although based on limited evidence.

What is New:

• Evaluating current practice, our data attest significant shortcomings in tumor surveillance of children and adolescents with CPS even in a tertiary-care children’s hospital.

• We clearly advocate a systematic and consistent integration of tumor surveillance into daily practice.