PRIMARY OBJECTIVE:
I. To determine the effect of GM-CSF on anti-GD2 antibody titers among patients who are in first or second (or later) complete response (CR), i.e., have no evidence of neuroblastoma (NB) by standard studies.
SECONDARY OBJECTIVES:
I. To estimate the time-to-relapse ratio of 2nd vaccine over 1st vaccine in patients who are in CR and have received previous vaccine plus beta-glucan.
II. To determine the effect of GM-CSF on anti-GD2 antibody titers among patients who are in first or second (or later) CR, and received a regimen with fewer cycles of beta-glucan.
III. To assess the impact of vaccine plus beta-glucan +/- GM-CSF on event-free survival (EFS) and overall survival (OS) of patients with HR-NB in first or subsequent CR.
IV. To evaluate anti-GD2, anti-GD3 and anti-KLH antibody responses associated with vaccine plus beta-glucan +/- GM-CSF.
V. To assess the safety of vaccine plus beta-glucan and GM-CSF as compared to vaccine plus beta-glucan.
VI. To characterize leukocyte polymorphisms (including C-type lectin single nucleotide polymorphism) and a possible association with outcome or antibody titers.
VII. To determine persistence of anti-NB antibody titers associated with vaccine plus beta-glucan +/- GM-CSF.
VIII. Patients who cannot be enrolled to achieve the primary objective (e.g., because of history of allergy to GM-CSF) (group 3) will undergo exploratory analyses of the above secondary objectives.
EXPLORATORY OBJECTIVES:
I. To assess the utility of monitoring of minimal residual disease (MRD) using blood cell free deoxyribonucleic acid (cfDNA).
OUTLINE: Patients are randomized to 1 of 2 groups. Patients who cannot be randomized are assigned to Group 1.
GROUP 1: Patients receive bivalent vaccine plus OPT-821 subcutaneously (SC) at weeks 1, 2, 3, 8, 20, 32, 52, 65, 78, 90, 104, and 156. Patients receive beta-glucan orally (PO) on week 1, continued as 2 weeks on, 2 weeks off through one cycle after vaccine injection #7, then one cycle with vaccine injections #8-14, and one 2-week cycle with each of the three booster vaccines at months 36, 48, and 60. Additionally, patients undergo blood sample collection, computed tomography (CT), magnetic resonance imaging (MRI), and lobenguane (MIBG) scan or positron emission tomography (PET) on study.
GROUP 2: Patients receive bivalent vaccine plus OPT-821 SC at weeks 1, 2, 3, 8, 20, 32, 52, 65, 78, 90, 104, and 156. Patients receive beta-glucan PO on week 1, continued as 2 weeks on, 2 weeks off through one cycle after vaccine injection #7, then one cycle with vaccine injections #8-14, and one 2-week cycle with each of the three booster vaccines at months 36, 48, and 60. Patients also receive GM-CSF SC for 3 days with vaccinations #1-3, for 7 days (3 days before, the day of, and 3 days after vaccination) for vaccinations #4-11, and for 5 days (1 day before, the day of, and 3 days after vaccination) for vaccinations #12-14. Additionally, patients undergo blood sample collection, CT, MRI, and MIBG scan or PET on study.
