NCCN Guideline Concordance Improves Survival in Pediatric and Young Adult Rectal Cancer

Background: Although management guidelines in adult rectal cancer are widely studied, no consensus guidelines exist for the management of pediatric and young adult rectal cancer. Methods: The National Cancer Database (2004-2018) was queried for pediatric (age 0-21) and young adult (age 22-40) patients with rectal cancer. Patients were analyzed for receipt of National Comprehensive Cancer Network (NCCN) guideline -concordant therapy. Impact on survival was evaluated using Cox regression and Kaplan -Meier analysis. Results: 6655 patients (108 pediatric and 6547 young adult patients) with rectal cancer were included. Similar to previously published NCCN quality measures with overall guideline concordance approaching 90 % in adults, 89.6 % of pediatric and 84.6 % of young adult patients were classified as receiving preoperative guideline -concordant therapy. However, pediatric patients were significantly less likely to receive post -operative guideline -concordant therapy than young adult patients (65.3 % verse 76.7 %, respectively, p 1/4 0.008). Risk of death was significantly lower for pediatric patients who received postoperative guideline -concordant therapy (HR, 0.313; CI, 0.168-0.581; p < 0.001). In young adult patients, risk of death was significantly lower for those who received pre -operative guideline -concordant therapy (HR, 0.376, CI 0.338-0.417, p < 0.001), and post -operative guideline -concordant therapy (HR, 0.456; CI 0.413-0.505; p < 0.001). Discussion: NCCN-based guidelines may reasonably guide peri-operative management decisions and improve survival in pediatric and young adult rectal cancer. Given the rarity of this cancer in young patients, employment of an experienced surgical and oncologic multidisciplinary team, along with discussion and involvement of the patient and family, are keys for balancing risks and benefits to offering the best therapeutic strategy. (c) 2023 Elsevier Inc. All rights reserved.