ACNS1833

Summary

Low-grade gliomas (LGGs) are the most common central nervous system tumors in children. Although surgical resection is the mainstay of therapy, complete resection can be difficult due to tumor location and can lead to adverse effects.

Radiation therapy is an effective treatment for LGGs but can cause serious side effects, such as second cancers. Therefore, most experts agree that the first-line standard of care for LGGs that require treatment is chemotherapy, specifically with classic chemotherapy like carboplatin and vincristine (CV).

Since the vast majority of patients will not succumb to their disease, treatment is often focused on improving progression-free survival and minimizing the impact on areas such as functional/behavioral outcomes and quality of life.

To be included in ACNS1833, participants must be / have 

• Patients must have non-neurofibromatosis type 1 (non-NF1) low-grade glioma (LGG) without a BRAFV600E mutation.
• No prior treatment besides surgery. Note: Patients may be newly-diagnosed or previously diagnosed, and there is no required timeframe between biopsy/surgery and treatment initiation.
• Patients with residual tumor after resection or progressive tumor after initial diagnosis (with or without surgery) who have not received treatment (chemotherapy and/or radiation) are eligible.
• Patients must have two-dimensional measurable tumor ≥ 1 cm² to be eligible.
• Patients must be ≥ 2 years and ≤ 21 years at the time of enrollment.
• Patients must have a body surface area (BSA) of ≥ 0.5 m² at enrollment.
• Patients must have adequate renal, liver, cardiac, and bone marrow function as defined by the protocol.
• Patients with a known seizure disorder should be stable.

To be included in ACNS1833, participants must not be / have

• Patients must not have received any prior tumor-directed therapy, including chemotherapy, radiation therapy, immunotherapy, or bone marrow transplant. Prior surgical intervention is permitted.
• Patients may not be receiving any other investigational agents.

Keywords

Chemotherapy; Low-grade glioma; Tumor