Abstract
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 affect 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.

