Therapy for Children With Advanced Stage Neuroblastoma

NCT ID: NCT01857934

Last Updated: 2025-11-17

Study Results

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Basic Information

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Recruitment Status

ACTIVE_NOT_RECRUITING

Clinical Phase

PHASE2

Total Enrollment

153 participants

Study Classification

INTERVENTIONAL

Study Start Date

2013-07-05

Study Completion Date

2025-12-31

Brief Summary

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Neuroblastoma is the most common extracranial solid tumor in childhood, with nearly 50% of patients presenting with widespread metastatic disease. The current treatment for this group of high-risk patients includes intensive multi-agent chemotherapy (induction) followed by myeloablative therapy with stem-cell rescue (consolidation) and then treatment of minimal residual disease (MRD) with isotretinoin. Recently a new standard of care was established by enhancing the treatment of MRD with the addition of a monoclonal antibody (ch14.18) which targets a tumor-associated antigen, the disialoganglioside GD2, which is uniformly expressed by neuroblasts. Despite improvement in 2-year event-free survival (EFS) of 20%, more than one-third of children with high-risk neuroblastoma (HR defined in) still cannot be cured by this approach. Therefore, novel therapeutic approaches are needed for this subset of patients. This study will be a pilot Phase II study of a unique anti-disialoganglioside (anti-GD2) monoclonal antibody (mAb) called hu14.18K322A, given with induction chemotherapy.

PRIMARY OBJECTIVE:

* To study the efficacy \[response: complete remission + partial remission (CR+PR)\] to two initial courses of cyclophosphamide and topotecan combined with hu14.18K322A (4 doses/course followed by GM-CSF) in previously untreated children with high-risk neuroblastoma.
* To estimate the event-free survival of patients with newly diagnosed high-risk neuroblastoma treated with the addition of hu14.18K322A to treatment.

SECONDARY OBJECTIVES:

* To study the feasibility of delivering hu14.18K322A to 6 cycles induction chemotherapy and describe the antitumor activity (CR+PR) of this 6 course induction therapy.
* To estimate local control and pattern of failure associated with focal intensity modulated or proton beam radiation therapy dose delivery in high-risk abdominal neuroblastoma.
* To describe the tolerability of four doses of hu14.18K322A with allogeneic natural killer (NK) cells from an acceptable parent, in the immediate post-transplant period \[day +2 - +5 after peripheral blood stem cell (PBSC) infusion\] in consenting participants.
* To describe the tolerability of hu14.18K322A with interleukin-2 and GM-CSF as treatment for minimal residual disease (MRD).

Detailed Description

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The phases of the study are:

1. Screening phase: Tests and evaluations will be done before treatment starts.
2. Induction phase: Includes chemotherapy plus hu14.18K322A mAb. Participants will also have surgery during this part of the study to remove as much tumor as possible.
3. Consolidation/Intensification phase: Includes high doses of chemotherapy and blood stem cell transplantation with additional, experimental "minimal residual disease" (MRD) treatment. Participants will also get radiation treatment to all sites of the tumor(s) after recovery from the stem cell transplant.
4. Maintenance/MRD treatment phase: With immune therapy in addition to the standard treatment with the drug isotretinoin.

Conditions

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Neuroblastoma

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Treatment

Participants receive IV hu14.18K322A with each course of chemotherapy (cyclophosphamide, topotecan, cyclophosphamide, doxorubicin, vincristine, cisplatin, and etoposide). Mesna will be given prior to and after cyclophosphamide infusion. Peripheral blood stem cell harvest (PBSC) and surgical resection of primary tumor will be performed, if feasible. Intensification therapy includes busulfan, melphalan, and levetiracetam with peripheral blood stem cell transplantation. A course of hu14.18K322A with natural killer cell infusion will be given to consenting participants. Radiation therapy will follow PBSC transplant with the exception of any patient requiring emergent radiotherapy. MRD treatment includes hu14.18K322A, G-CSF, GM-CSF, interleukin-2 and isotretinoin.

Cells for infusion are prepared using the CliniMACS System.

Group Type EXPERIMENTAL

cyclophosphamide

Intervention Type DRUG

Given intravenously (IV)

topotecan

Intervention Type DRUG

Given IV

hu14.18K322A

Intervention Type BIOLOGICAL

Given IV

peripheral blood stem cell harvest

Intervention Type PROCEDURE

Following evaluation and approval by a member of the transplant staff and completion of the consent form by the participant, collection of peripheral blood stem cells (PBSC) may take place.

surgical resection

Intervention Type PROCEDURE

The primary tumor will be resected surgically following two initial courses of chemotherapy, if feasible. Patients who are unable to have their primary tumor resected after the initial two courses of induction chemotherapy will undergo surgery for resection of the primary tumor mass and careful lymph node staging.

cisplatin

Intervention Type DRUG

Given IV

etoposide

Intervention Type DRUG

Given IV

doxorubicin

Intervention Type DRUG

Given IV

vincristine

Intervention Type DRUG

Given IV

busulfan

Intervention Type DRUG

Given IV

melphalan

Intervention Type DRUG

Given IV

peripheral blood stem cell transplantation

Intervention Type BIOLOGICAL

Transplantation of previously harvested peripheral blood stem cells.

natural killer cell infusion

Intervention Type BIOLOGICAL

Natural killer (NK) cells obtained from a suitable donor will be given together with hu14.18K322A prior to early hematopoietic cell recovery. In the event there is not a suitable parental donor, consenting participants will receive an additional course of hu14.18K322A.

radiation therapy

Intervention Type RADIATION

Radiation therapy to the primary and metastatic disease sites will follow peripheral blood stem cell transplant with the exception of any patient requiring emergent radiotherapy. External beam radiotherapy will be delivered to the primary site and select metastatic and bulky nodal sites.

GM-CSF

Intervention Type BIOLOGICAL

Given subcutaneously (SQ)

G-CSF

Intervention Type BIOLOGICAL

Given subcutaneously (SQ)

mesna

Intervention Type DRUG

Given IV

levetiracetam

Intervention Type DRUG

Given IV

interleukin-2

Intervention Type BIOLOGICAL

Given by continuous infusion during MRD maintenance, and SQ during induction.

Isotretinoin

Intervention Type DRUG

Given orally (PO)

CliniMACS

Intervention Type DEVICE

The mechanism of action of the CliniMACS Cell Selection System is based on magnetic-activated cell sorting (MACS). The CliniMACS device is a powerful tool for the isolation of many cell types from heterogeneous cell mixtures, (e.g. apheresis products). These can then be separated in a magnetic field using an immunomagnetic label specific for the cell type of interest, such as CD3+ human T cells.

Interventions

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cyclophosphamide

Given intravenously (IV)

Intervention Type DRUG

topotecan

Given IV

Intervention Type DRUG

hu14.18K322A

Given IV

Intervention Type BIOLOGICAL

peripheral blood stem cell harvest

Following evaluation and approval by a member of the transplant staff and completion of the consent form by the participant, collection of peripheral blood stem cells (PBSC) may take place.

Intervention Type PROCEDURE

surgical resection

The primary tumor will be resected surgically following two initial courses of chemotherapy, if feasible. Patients who are unable to have their primary tumor resected after the initial two courses of induction chemotherapy will undergo surgery for resection of the primary tumor mass and careful lymph node staging.

Intervention Type PROCEDURE

cisplatin

Given IV

Intervention Type DRUG

etoposide

Given IV

Intervention Type DRUG

doxorubicin

Given IV

Intervention Type DRUG

vincristine

Given IV

Intervention Type DRUG

busulfan

Given IV

Intervention Type DRUG

melphalan

Given IV

Intervention Type DRUG

peripheral blood stem cell transplantation

Transplantation of previously harvested peripheral blood stem cells.

Intervention Type BIOLOGICAL

natural killer cell infusion

Natural killer (NK) cells obtained from a suitable donor will be given together with hu14.18K322A prior to early hematopoietic cell recovery. In the event there is not a suitable parental donor, consenting participants will receive an additional course of hu14.18K322A.

Intervention Type BIOLOGICAL

radiation therapy

Radiation therapy to the primary and metastatic disease sites will follow peripheral blood stem cell transplant with the exception of any patient requiring emergent radiotherapy. External beam radiotherapy will be delivered to the primary site and select metastatic and bulky nodal sites.

Intervention Type RADIATION

GM-CSF

Given subcutaneously (SQ)

Intervention Type BIOLOGICAL

G-CSF

Given subcutaneously (SQ)

Intervention Type BIOLOGICAL

mesna

Given IV

Intervention Type DRUG

levetiracetam

Given IV

Intervention Type DRUG

interleukin-2

Given by continuous infusion during MRD maintenance, and SQ during induction.

Intervention Type BIOLOGICAL

Isotretinoin

Given orally (PO)

Intervention Type DRUG

CliniMACS

The mechanism of action of the CliniMACS Cell Selection System is based on magnetic-activated cell sorting (MACS). The CliniMACS device is a powerful tool for the isolation of many cell types from heterogeneous cell mixtures, (e.g. apheresis products). These can then be separated in a magnetic field using an immunomagnetic label specific for the cell type of interest, such as CD3+ human T cells.

Intervention Type DEVICE

Other Intervention Names

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Cytoxan(R) Hycamtin(R) humanized anti-GD2 antibody monoclonal antibody dinutuximab PBSCH Platinol-AQ(R) VP16 Vepesid(R) Etopophos(R) Adriamycin(R) Oncovin(R) Busulfex(R) L-phenylalanine mustard Phenylalanine mustard L-PAM L-sarcolysin Alkeran(R) PBSCT NK cell infusion sargramostim Leukine(R) granulocyte macrophage colony stimulating factor Granulocyte colony stimulating factor Neupogen(R) Filgrastim Mesnex(R) Keppra IL-2 aldesleukin Proleukin(R) 13-cis retinoic acid Cell Selection System

Eligibility Criteria

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Inclusion Criteria

* Participants \<19 years of age (eligible until 19th birthday).
* Newly diagnosed, advanced stage, high-risk neuroblastoma defined as one of the following:

* Children \< 1 year with International Neuroblastoma Staging System (INSS) stage 2a, 2b, 3, 4 or 4S disease AND MYCN amplification (\>10 copies, or greater than four-fold increase in MYCN signal as compared to reference signal).
* INSS 2a or 2b disease AND MYCN amplification, regardless of age or additional biologic features
* INSS stage 3 AND:

1. MYCN amplification (\>10 copies, or greater than four-fold increase in MYCN signal as compared to reference signal, regardless of age or additional biologic features
2. Age \> 18 months (\> 547 days) with unfavorable pathology, regardless of MYCN status
* INSS stage 4 and:

1. MYCN amplification, regardless of age or additional biologic features
2. Age \> 18 months (\> 547 days) regardless of biologic features
3. Age 12 - 18 months (365 - 547 days) with any of the following three unfavorable biologic features (MYCN amplification, unfavorable pathology and/or DNA index =1) or any biologic feature that is indeterminant/unknown
* Children at least 365 days initially diagnosed with: INSS stage 1, 2, 4S who progressed to a stage 4 without interval chemotherapy.
* Histologic proof of neuroblastoma or positive bone marrow for tumor cells with increased urine catecholamines.
* Adequate renal and hepatic function (serum creatinine \<3 x upper limit of normal for age, AST\< 3 x upper limit of normal).
* No prior therapy, unless an emergency situation requires local tumor treatment (discuss with principal investigator).
* Written, informed consent according to institutional guidelines.


* Potential donor is a biologic parent
* Potential donor is at least 18 years of age.

Exclusion Criteria

* Any evidence, as judged by the investigator, of severe or uncontrolled systemic disease (e.g., unstable or uncompensated respiratory, cardiac, hepatic, or renal disease).
* Pregnant or breast feeding (female of child-bearing potential).
* Children with INSS 4 disease, age \<18 months with all 3 favorable biologic features (non-amplified MYCN, favorable pathology and DNA index \>1).
Maximum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Cookies for Kids' Cancer

OTHER

Sponsor Role collaborator

CURE Childhood Cancer, Inc.

OTHER

Sponsor Role collaborator

St. Jude Children's Research Hospital

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Sara M. Federico, MD

Role: PRINCIPAL_INVESTIGATOR

St. Jude Children's Research Hospital

Locations

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St. Jude Children's Research Hospital

Memphis, Tennessee, United States

Site Status

Countries

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United States

References

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Furman WL, McCarville B, Shulkin BL, Davidoff A, Krasin M, Hsu CW, Pan H, Wu J, Brennan R, Bishop MW, Helmig S, Stewart E, Navid F, Triplett B, Santana V, Santiago T, Hank JA, Gillies SD, Yu A, Sondel PM, Leung WH, Pappo A, Federico SM. Improved Outcome in Children With Newly Diagnosed High-Risk Neuroblastoma Treated With Chemoimmunotherapy: Updated Results of a Phase II Study Using hu14.18K322A. J Clin Oncol. 2022 Feb 1;40(4):335-344. doi: 10.1200/JCO.21.01375. Epub 2021 Dec 6.

Reference Type DERIVED
PMID: 34871104 (View on PubMed)

Nguyen R, Sahr N, Sykes A, McCarville MB, Federico SM, Sooter A, Cullins D, Rooney B, Janssen WE, Talleur AC, Triplett BM, Anthony G, Dyer MA, Pappo AS, Leung WH, Furman WL. Longitudinal NK cell kinetics and cytotoxicity in children with neuroblastoma enrolled in a clinical phase II trial. J Immunother Cancer. 2020 Mar;8(1):e000176. doi: 10.1136/jitc-2019-000176.

Reference Type DERIVED
PMID: 32221013 (View on PubMed)

Provided Documents

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Document Type: Study Protocol and Statistical Analysis Plan

View Document

Document Type: Informed Consent Form

View Document

Related Links

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http://www.stjude.org

St. Jude Children's Research Hospital

http://www.stjude.org/protocols

Clinical Trials Open at St. Jude

Other Identifiers

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NB2012

Identifier Type: -

Identifier Source: org_study_id

NCI-2013-00034

Identifier Type: REGISTRY

Identifier Source: secondary_id

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