Performance of Tumor Surveillance for Children With Cancer Predisposition.

Area of Science:

• Pediatric Oncology
• Genetics
• Medical Surveillance

Background:

• Pediatric oncology patients increasingly present with underlying cancer predisposition syndromes (CPS).
• Surveillance is crucial for early detection of new tumors in children with CPS.
• Limited data exist on the effectiveness of surveillance for children with CPS.

Purpose of the Study:

• To evaluate the performance of surveillance across a wide spectrum of cancer predisposition syndromes (CPS).

Main Methods:

• A cohort study reviewed surveillance outcomes for 274 children and young adults (birth to 23 years) with CPS.
• Patients underwent standard surveillance regimens at a specialty pediatric oncology center.
• Exclusions included hereditary retinoblastoma and bone marrow failure syndromes.

Main Results:

• Surveillance detected 35 asymptomatic tumors in 27 patients (9.9%), with 28.6% found on initial imaging.
• Tumors detected via surveillance were more localized (83.3%) than those found pre-diagnosis (56.8%).
• Surveillance demonstrated high accuracy: sensitivity (96.4%), specificity (99.6%), PPV (94.3%), NPV (99.6%).

Conclusions:

• Standardized surveillance enables early tumor detection across diverse CPSs.
• Early detection facilitates complete surgical resection and successful treatment for most patients.
• Surveillance is a valuable tool for managing pediatric cancer predisposition syndromes.