A Study of a Vaccine in Combination With Beta-glucan in People With Neuroblastoma

Inclusion Criteria:

• Diagnosis of NB as defined by international criteria,102 i.e., histopathology (confirmed by the MSK Department of Pathology) or BM metastases plus high urinecatecholamine levels, or positivity in MIBG scan.

• HR-NB as defined by risk-related treatment guidelines and international criteria,102i.e., metastatic/non-localized disease with MYCN amplification (any age), metastaticdisease >18 months old, MYCN-amplified localized disease (any age), or diseaseresistant to standard chemotherapy.

• HR-NB (as defined above) and in 1) first CR at ≥ 6 months from initiation ofimmunotherapy using anti-GD2 antibody, or 2) second or subsequent CR (achieved aftertreatment for PD). CR is defined according to the International NeuroblastomaResponse Criteria. Patients with positive MIBG scan but negative FDG-PET scan, andCR in BM, are eligible.

• Patients with grade 3 toxicities or less using the Common Toxicity Criteria (Version 5.0) developed by the National Cancer Institute of the USA (CTCAE v5.0) related tohematologic, cardiac, neurological, pulmonary, renal, hepatic or gastrointestinalfunction as determined by blood tests or physical exam. Plus:

• Absolute neutrophil count (ANC) ≥ 500/mcl

• Absolute lymphocyte count ≥ 500/mcl

• >21 and <180 days between completion of systemic therapy and 1st vaccination.

• A negative pregnancy test is required for patients with child-bearing capability

• Signed informed consent indicating awareness of the investigational nature of thisprogram.

Exclusion Criteria:

• Patients with grade 4 hematologic, cardiac, neurological, pulmonary, renal, hepaticor gastrointestinal function as determined by blood tests or physical exam, usingthe Common Toxicity Criteria (Version 5.0) developed by the National CancerInstitute of the USA.

• History of allergy to KLH, QS-21, OPT-821, or glucan

• Prior treatment with this vaccine.

• Active life-threatening infection requiring systemic therapy.

• Inability to comply with protocol requirements.