Background: Isotretinoin (13-cis-retinoic acid; 13-cRA), a differentiation inducer, improves outcome for high-risk neuroblastoma. Published PK studies suggested that many patients achieve 13-cRA levels lower than 5 mM (effective against neuroblastoma in vitro). We showed that 4-oxo-13-cRA, a metabolite of 13-cRA was equally active compared to 13-cRA against neuroblastoma in vitro. We sought to determine the levels of 13-cRA and 4-oxo-13-cRA achieved in patients and their association with clinical outcome.
Methods: Plasma samples (4 hours post-dose, day 14) from COG phase III clinical trials (A-3973, ANBL0532, ANBL0032, and ANBL0931) were obtained for PK analyses. The relationship of PK to clinical outcome was analyzed, limited to patients treated with ch14.18 antibody.
Results: In 629 patients, 370 (60%) achieved median plasma concentrations over 5 mM for 13-cRA+4-oxo-13-cRA combined levels. Plasma levels were higher in patients taking whole capsules relative to open-capsule takers (13-cRA: 1.74 vs 1.03, 4-oxo-13-cRA: 7.22 vs 3.27mM). Both 13-cRA (r=0.29) and 4-oxo-13-cRA (r=0.46) concentrations positively correlated with age (p<0.001). Relationship of PK to overall survival (OS) was analyzed for 524 patients that received both 13-cRA and immunotherapy. In patients ≥18 months old OS at 5 yrs was higher (73 %) for 13-cis-RA plasma levels > 75th percentile (2.5µM) vs. pts with 13-cRA < 25th percentile (0.6µM, 63%) (p=0.039). OS at 5 yrs was also higher for patients ≥18 months-old with 4-oxo-13cRA >5µM vs. <1µM: 76 vs. 66% (p=0.032) and 13-cis-RA + 4-oxo-13-cis-RA >5µM vs. <1µM: 73 vs. 61% (p=0.019).
Conclusion: Combined levels of 13-cRA and the 4-oxo-13-cRA active metabolite were >5 mM in the majority of patients. Age and route of administration influence plasma levels of 13-cRA and 4-oxo-13-cRA. In patients ≥18 months old low plasma levels of 13-cis-RA + 4-oxo-13-cRA were associated with a lower overall survival.