This phase II trial studies if dinutuximab, GM-CSF, isotretinoin in combination with
irinotecan, and temozolomide (chemo-immunotherapy) can be given safely to patients with
high-risk neuroblastoma after Consolidation therapy (which usually consists of two autologous
stem cell transplants and radiation) who have not experienced worsening or recurrence of
their disease. Dinutuximab represents a kind of cancer therapy called immunotherapy. Unlike
chemotherapy and radiation, dinutuximab targets the cancer cells without destroying nearby
healthy cells. Sargramostim helps the body produce normal infection-fighting white blood
cells. Isotretinoin helps the neuroblastoma cells become more mature. These 3 drugs (standard
immunotherapy) are already given to patients with high-risk neuroblastoma after Consolidation
because they have been proven to be beneficial in this setting. Chemotherapy drugs, such as
irinotecan and temozolomide, work in different ways to stop the growth of tumor cells, either
by killing the cells, by stopping them from dividing, or by stopping them from spreading.
They may also effect how well immunotherapy works on neuroblastoma cells. Giving
chemo-immunotherapy after intensive therapy may work better in treating patients with
high-risk neuroblastoma compared to standard immunotherapy.
I. To determine the feasibility of administering dinutuximab, GM-CSF, and isotretinoin in
combination with irinotecan and temozolomide in the frontline Post-Consolidation setting in
patients with high-risk neuroblastoma who have undergone Induction and Consolidation therapy
with tandem high-dose chemotherapy with stem cell rescue (ASCT).
I. To describe the toxicity profile of dinutuximab, GM-CSF, and isotretinoin in combination
with irinotecan and temozolomide in the Post-Consolidation setting.
II. To describe the event-free survival and overall survival of patients who receive
dinutuximab in combination with irinotecan and temozolomide, GM-CSF, and isotretinoinin the
I. To describe the toxicity profiles associated with chemo-immunotherapy in the
Post-Consolidation setting according to the type of prior therapy.
II. To describe response to chemo-immunotherapy in the Post-Consolidation setting using the
revised International Neuroblastoma Risk Classification (INRC) in patients with evaluable or
measurable disease at study entry.
III. To characterize immune and cytokine profiles in patients receiving Post-Consolidation
IV. To bank serial blood samples to investigate the relationship between factors related to
the tumor, host, and immune environment and clinical outcomes in patients treated with
Patients receive temozolomide orally (PO) or via enteral tube daily and irinotecan
intravenously (IV) over 90 minutes daily on days 1-5, dinutuximab IV over 10-20 hours daily
on days 2-5, sargramostim subcutaneously (SC) or IV over 2 hours daily on days 6-12, and
isotretinoin PO twice daily (BID) on days 8-21. Treatment repeats every 28 days for up to 5
cycles (up to 6 cycles for isotretinoin only) in the absence of disease progression or
After completion of study treatment, patients are followed up at 3, 6, 9, 12, 15, 18, 24, 30,
36, 42, 48, 54, and 60 months.
Ganglioneuroblastoma, Nodular, High Risk Neuroblastoma
If you are confirmed eligible after full screening, you will be required to understand and sign the informed consent if you decide to enroll in the study. Once enrolled you may be asked to make scheduled visits over a period of time.
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