Phase III EuroNet-PHL-LP1 trial: Complete surgical resection or CVP in pediatric patients with nLPHL

Results from the prospective phase III EuroNet-PHL-LP1 trial (EudraCT 2007-004092-19), investigating complete surgical resection (n = 87) or anthracycline-free cyclophosphamide + vinblastine + prednisone (CVP; n = 166) in pediatric patients (aged <18 years) with previously untreated Stage IA/IIA nodular lymphocyte-predominant Hodgkin lymphoma (nLPHL), were published in Blood Advances by Shankar et al. Patients receiving CVP were separated into Cohort 1, who were assessed using 2-deoxy-2-[fluorine-18] fluoro-d-glucose positron emission tomography (18F-FDG PET; n = 82), and Cohort 2, where computed tomography (CT) and/or magnetic resonance imaging (MRI) alone defined response (n = 84). The primary endpoint was 5-year event-free survival (EFS).

Key data: In patients who underwent complete surgical resection, at a median follow-up of 63.3 months, the 5-year progression-free survival (PFS) rate was 79.5% (95% confidence interval [CI], 71.2–88.7). At a median follow-up of 75.1 months, the 5-year EFS, PFS, and overall survival (OS) rates in Cohort 1 were 56.4% (95% CI, 46.5–68.4), 92.2% (95% CI, 86.4–98.4), and 100%, respectively. In Cohort 1, patients with a complete metabolic response (CMR) had a 5-year PFS of 91.3% (95% CI, 83.6–99.9) vs 90.5% (95% CI, 78.8–100) in patients with PET-positive disease who received additional treatment. In Cohort 2, at a median follow-up of 63.9 months, the 5-year PFS and OS rates were 64.7% (95% CI, 55.1–76.0) and 96.8% (95% CI, 92.5–100), respectively. In patients receiving CVP, the most common Grade 3/4 adverse events (AEs) were neutropenia (68.3%), leukopenia (29.3%), vomiting (1.8%), and infections (1.8%). There were two deaths: one patient with unexplained heart failure and one patient with progressive disease. 

Key learning: Results from the EuroNet-PHL-LP1 trial indicate that both complete surgical resection and anthracycline-free CVP chemotherapy represent effective treatment strategies for pediatric patients with early-stage nLPHL.