A Study of the Drugs Selumetinib vs. Carboplatin and Vincristine in Patients With Low-Grade Glioma

Exclusion Criteria

• •Eligible histologies will include all tumors considered low-grade glioma or low-grade astrocytoma (World Health Organization \[WHO\] grade I and II) by 5th edition WHO classification of central nervous system (CNS) tumors with the exception of subependymal giant cell astrocytoma
• •Patients with metastatic disease or multiple independent primary LGG are eligible
• •Creatinine clearance or radioisotope glomerular filtration rate (GFR) \>= 70 mL/min/1.73 m\^2 OR a serum creatinine based on age/sex as follows (performed within 7 days prior to enrollment):
• •* Age: Maximum Serum Creatinine (mg/dL)
• •* 2 to \< 6 years: 0.8 mg/dL (male); 0.8 mg/dL (female)
• •* 6 to \< 10 years: 1 mg/dL (male); 1 mg/dL (female)
• •* 10 to \< 13 years: 1.2 mg/dL (male); 1.2 mg/dL (female)
• •* 13 to \< 16 years: 1.5 mg/dL (male); 1.4 mg/dL (female)
• •* \>= 16 years: 1.7 mg/dL (male); 1.4 mg/dL (female)
• •Total bilirubin =\< 1.5 x upper limit of normal (ULN) for age (performed within 7 days prior to enrollment) (children with a diagnosis of Gilbert's syndrome will be allowed on study regardless of their total and indirect \[unconjugated\] bilirubin levels as long as their direct \[conjugated\] bilirubin is \< 3.1 mg/dL)
• •Serum glutamic pyruvic transaminase (SGPT) (alanine aminotransferase \[ALT\]) =\< 135 U/L (performed within 7 days prior to enrollment). For the purpose of this study, the ULN for SGPT is 45 U/L
• •Albumin \>= 2 g/dL (performed within 7 days prior to enrollment)
• •Left ventricular ejection fraction (LVEF) \>= 53% (or institutional normal; if the LVEF result is given as a range of values, then the upper value of the range will be used) by echocardiogram (performed within 4 weeks prior to enrollment)
• •Corrected QT (QTc) interval =\< 450 msec by electrocardiography (EKG) (performed within 4 weeks prior to enrollment)
• •Absolute neutrophil count \>= 1,000/uL (unsupported) (performed within 7 days prior to enrollment)
• •Platelets \>= 100,000/uL (unsupported) (performed within 7 days prior to enrollment)
• •Hemoglobin \>= 8 g/dL (may be supported) (performed within 7 days prior to enrollment)
• •Patients with a known seizure disorder must be stable and must not have experienced a significant increase in seizure frequency within 2 weeks prior to enrollment
• •Patients 2-17 years of age must have a blood pressure that is =\< 95th percentile for age, height, and sex at the time of enrollment (with or without the use of anti-hypertensive medications)
• •Patients \>= 18 years of age must have a blood pressure =\< 130/80 mmHg at the time of enrollment (with or without the use of anti-hypertensive medications)
• •Note for patients of all ages: Adequate blood pressure can be achieved using medication for the treatment of hypertension
• •All patients must have ophthalmology toxicity assessments performed within 8 weeks prior to enrollment
• •For all patients, an MRI of the brain (with orbital cuts for optic pathway tumors) and/or spine (depending on the site(s) of primary disease) with and without contrast must be performed within 8 weeks prior to enrollment
• •Patients must have a performance status corresponding to Eastern Cooperative Oncology Group (ECOG) scores of 0, 1, or 2. Use Karnofsky for patients \> 16 years of age and Lansky for patients =\< 16 years of age
• •Patients must have the ability to swallow whole capsules
• •All patients and/or their parents or legal guardians must sign a written informed consent
• •All institutional, Food and Drug Administration (FDA), and National Cancer Institute (NCI) requirements for human studies must be met
• •All patients have signed an appropriate consent form and Health Insurance Portability and Accountability Act (HIPAA) authorization form (if applicable)
• •All patients have been consented and enrolled on APEC14B1 (NCT02402244) Part A for Pre-Enrollment Eligibility Screening for ACNS1833
• •Patients must not have received any prior tumor-directed therapy including chemotherapy, radiation therapy, immunotherapy, or bone marrow transplant. Prior surgical intervention (with the exclusion of laser interstitial thermal therapy \[LITT\]) is permitted
• •Patients with a concurrent malignancy or history of treatment (other than surgery) for another tumor within the last year are ineligible
• •Patients may not be receiving any other investigational agents
• •Patients with any serious medical or psychiatric illness/condition, including substance use disorders or ophthalmological conditions, likely in the judgment of the investigator to interfere or limit compliance with study requirements/treatment
• •* Note: Women study participants of child-bearing potential must use acceptable contraception during the study and for 1 week (7 days) after the last dose of selumetinib. Men study participants with sexual partners who are pregnant or who are of child-bearing potential must use acceptable contraception during the study and for 1 week (7 days) after the last dose of study agent. Acceptable contraception includes implants, injectables, or oral contraceptives (all combined with barrier methods), some intrauterine devices (IUDs), vasectomy or abstinence
• •Known genetic disorder that increases risk for coronary artery disease. Note: The presence of dyslipidemia in a family with a history of myocardial infarction is not in itself an exclusion unless there is a known genetic disorder documented
• •Symptomatic heart failure
• •New York Health Association (NYHA) class II-IV prior or current cardiomyopathy
• •Severe valvular heart disease
• •History of atrial fibrillation
• •Current or past history of central serous retinopathy
• •Current or past history of retinal vein occlusion or retinal detachment
• •Patients with uncontrolled glaucoma
• •Supplementation with vitamin E greater than 100% of the daily recommended dose. Any multivitamin containing vitamin E must be stopped prior to study enrollment even if less than 100% of the daily recommended dosing for vitamin E
• •Surgery within 2 weeks prior to enrollment, with the exception of surgical biopsy, placement of a vascular access device or cerebral spinal fluid (CSF) diverting procedure such as endoscopic third ventriculostomy (ETV) and ventriculoperitoneal (VP) shunt.
• •* Note: Patients must have healed from any prior surgery