Relapsed Pancreatoblastoma in Children: The Quest for Cure in This Rare Entity

Pancreatoblastoma constitutes the most common malignant pancreatic tumor in children. Pancreatoblastomas are rare and data to generate evidence-based management guidelines are limited. A literature review and pooled data analysis of cases 18 years old or younger with relapsed pancreatoblastoma (RP) was performed to describe their prognosis and management. The 2-year overall survival post-relapse (OS-pr) for patients with RP (n=15) was 54.4% (95% CI: 32.5%-71.6%). On the basis of surgery at relapse, the 2-year OS-pr was 85.7% (95% CI: 59.8%-96.1%) for cases who underwent surgery (n=9) versus 16.7% (95% CI: 1.1%-44.9%) for nonsurgical cases (n=6); P=0.003. This study shows that patients with RP can be salvaged and supports pursuing treatment with curative intent, including maximal safe resection where feasible.