Selumetinib in Treating Young Patients With Recurrent or Refractory Low Grade Glioma

This phase I/II trial studies the side effects and the best dose of selumetinib and how well it works in treating or re-treating young patients with low grade glioma that has come back (recurrent) or does not respond to treatment (refractory). Selumetinib may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth.

Inclusion Criteria: * Imaging evaluations necessary to establish eligibility for study entry must be done within three (3) weeks prior to registration * All other evaluations necessary to establish eligibility for study entry must be done within two (2) weeks prior to registration * Patients must start therapy within 7 calendar days of registration * Laboratory values must be no older than seven (7) days prior to the start of therapy; if a test that is repeated after registration and prior to therapy is outside the limits for eligibility, it must be rechecked within 48 hours prior to the start of therapy; if laboratory values still fail to meet eligibility criteria, the patient may not receive protocol therapy * All patients must meet the following inclusion and exclusion criteria; NO EXCEPTIONS WILL BE GIVEN * ELIGIBILITY CRITERIA FOR SCREENING ON PHASE II STUDY: Participant is willing to sign a screening consent and provide adequate pre-trial tumor material for BRAF testing (both for BRAF V\^600E mutation and BRAF KIAA1549 fusion assessments) * All patients who are candidates for enrollment in stratum 5 based on their tumor histology must be pre-screened * Screening may be applied to potential stratum 1 and 2 patients * ELIGIBILITY CRITERIA FOR SCREENING ON PHASE II STUDY: Patients whose prior BRAF testing was performed at another lab (Clinical Laboratory Improvement Amendments \[CLIA\]/College of American Pathologist \[CAP\] certified or otherwise) must send additional tumor material to Brigham and Women's Hospital (BWH) for confirmation; however, to preserve available tumor material, patients whose tumor material has previously undergone BRAF analysis at the Lindeman and Ligon Labs at Brigham and Women's Hospital using the same procedures as described in this protocol, will not be required to submit additional tumor material for analysis; these patients must have both the BRAFV600E mutation and BRAF KIAA1549 fusion assessments done and if only one test was previously conducted; additional tissue will be required for the second test * ELIGIBILITY CRITERIA FOR SCREENING ON PHASE II STUDY: Patient must be \>= 3 but =\< 21 years of age at registration * ELIGIBILITY CRITERIA FOR SCREENING ON PHASE II STUDY: Patient must have one of the following: * For stratum 5: non NF-1 associated low grade glioma (LGG) (other than pilocytic astrocytoma or optic pathway glioma) * For stratum 1 or 2: non NF-1, non-optic pathway pilocytic astrocytoma; note: all patients with non NF-1 associated optic pathway glioma with or without tissue must be enrolled on stratum 4 * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patients with sporadic (non NF-1 associated), histologically diagnosed progressive, recurrent or refractory non-optic pathway pilocytic astrocytoma who have pre- treatment tumor tissue available for BRAF analysis * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: NF-1 patients with radiographic evidence of a progressive, recurrent or refractory low grade glioma, with or without pre-treatment tumor tissue * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patients with progressive, recurrent or refractory optic pathway glioma, with or without pre-treatment tumor tissue * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patients with histologically diagnosed progressive, recurrent or refractory non NF-1 associated LGG (other than pilocytic astrocytoma or optic pathway glioma); these patients must have BRAF aberrations as documented by the Lindeman and Ligon Labs at Brigham and Women's Hospital using the same procedures * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patients will be assigned to one of 6 strata prior to enrollment; all BRAF assessments used for stratification below must be done at the Lindeman and Ligon Labs at Brigham and Women's Hospital using the same procedures as described in this protocol; assessments for both BRAF V\^600E mutation and BRAF KIAA1549 fusion are required for patients who will enroll on strata 1, 2 and 5 * Stratum 1: patients with non NF-1 associated progressive, recurrent or refractory pilocytic astrocytoma with pre-trial tumor material available and with a BRAF aberration i.e. BRAFV\^600E mutation and/or BRAF KIAA1549 fusion as determined by IHC and FISH, respectively; patients with optic pathway glioma are excluded * Stratum 2: patients with non NF-1 associated progressive, recurrent or refractory pilocytic astrocytoma with pre-trial tumor material available and without a BRAF aberration i.e. BRAF\^V600E mutation and/or BRAF KIAA1549 fusion as determined by IHC and FISH, respectively; patients with optic pathway glioma are excluded * Stratum 3: patients with neuro-fibromatosis 1 (NF-1) associated progressive, recurrent or refractory low grade glioma (World Health Organization \[WHO\] grade I \& II), with or without tissue * Stratum 4\*: patients with non-NF1 associated progressive, recurrent or refractory optic pathway glioma with or without tissue available for BRAF evaluation * Stratum 5: patients with non NF-1 associated progressive, recurrent or refractory low grade glioma other than pilocytic astrocytoma or optic pathway glioma with a documented BRAF aberration identified in pre-trial tumor material * Stratum 6: patients with non-NF-1 associated progressive, recurrent or refractory low grade glioma (other than optic pathway glioma \[OPG\]) with tissue available for BRAF analyses who cannot be classified into stratum 1, 2 or 5 due to inadequate tissue quality, assay failure, etc * Clarification: Stratum 4 was specifically designed for patients with hypothalamic/optic pathway gliomas; the intent is that if there is any optic chiasm invasion regardless of where the tumor is originating from (chiasm vs. hypothalamus vs. other location), the patient should be enrolled on Stratum 4, regardless of whether the tumor has been biopsied or not; obviously, there are some tumors that include part of the hypothalamus and clearly do NOT include the chiasm at all; in these situations, and if the tumor is a biopsy proven pilocytic astrocytoma, these patients should be enrolled on Stratum 1 or 2 (depending upon BRAF status) * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patients must have bi-dimensionally measurable disease defined as at least one lesion that can be accurately measured in at least two planes in order to be eligible for this study * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patients must have received prior therapy other than surgery and must have fully recovered from the acute toxic effects of all prior chemotherapy, immunotherapy, biologic therapy or radiotherapy prior to study entry * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Myelosuppressive chemotherapy: Patients must have received their last dose of known myelosuppressive anticancer chemotherapy at least three weeks prior to study registration or at least six weeks if nitrosourea * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Biologic agent: Patient must have received their last dose of the biologic agent \>= 7 days prior to study registration * For biologic agents that have a prolonged half-life, at least three half-lives must have elapsed prior to registration * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Monoclonal antibody treatment: at least three half-lives must have elapsed prior to registration * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Radiation: patients must have: * Had their last fraction of local irradiation to primary tumor \>= 12 months prior to registration; investigators are reminded to review potentially eligible cases to avoid confusion with pseudo-progression * Had their last fraction of craniospinal irradiation (\> 24 Gy) \> 3 months prior to registration * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Corticosteroids: patients who are receiving dexamethasone must be on a stable or decreasing dose for at least 1 week prior to registration * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Growth factors: Patients must be off all colony-forming growth factor(s) for at least 1 week prior to registration (filgrastim, sargramostim, erythropoietin) and at least 2 weeks for long-acting formulations * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patient must be \>= 3 but =\< 21 years of age at registration * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patients must have a body surface area (BSA) \>= 0.55 m\^2 * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patients with neurological deficits should have deficits that are stable for a minimum of 1 week prior to registration * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Patients must be able to swallow capsules * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Karnofsky performance scale (KPS for \> 16 years \[yrs.\] of age) or Lansky performance score (LPS for =\< 16 years of age) \>= 60 assessed within two weeks prior to registration * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Absolute neutrophil count \>= 1,000/uL (unsupported) (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Platelets \>= 100,000/L (unsupported) (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Hemoglobin \>= 8 g/dL (may be supported) (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Aspartate aminotransferase (AST) (serum glutamic oxaloacetic transaminase \[SGOT\])/alanine aminotransferase (ALT) (serum glutamate pyruvate transaminase \[SGPT\]) =\< 2.5 X institutional upper limit of normal for age (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Total bilirubin \< 1.5 times upper limit of normal for age (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Albumin \>= 3 g/dL (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Serum sodium and potassium within the institutional limits of normal (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Serum calcium and magnesium above the institutional lower limit of normal (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Creatinine clearance or radioisotope glomerular filtration rate (GFR) \>= 70 ml/min/1.73m\^2 or a serum creatinine based on age as follows (within 14 days of registration and within 7 days of the start of treatment): * =\< 5 years: 0.8 mg/dL * \> 5 years but =\< 10 years: 1 mg/dL * \> 10 years but =\< 15 years: 1.2 mg/dL * \> 15 years: 1.5 mg/dL * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Left ventricular ejection fraction (LVEF) \>= 55% * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Corrected QT (QTc) interval =\< 450 msecs * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Hypertension: * Patients, 3-17 years of age must have a blood pressure that is =\< 95th percentile for age, height and sex at the time of registration * The normal blood pressure by height, age and sex tables can be accessed in the Generic Forms section of the Pediatric Brain Tumor Consortium (PBTC) members' webpage * Patients who are \>= 18 years of age must have a blood pressure that is \< 140/90 mm of Hg at the time of registration * Note: if a blood pressure (BP) reading prior to registration is above the 95th percentile for age, height and sex it must be rechecked and documented to be =\< the 95th percentile for age, height and sex prior to patient registration * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Female patients of childbearing potential must not be pregnant or breast-feeding; female patients of childbearing potential must have a negative serum or urine pregnancy test * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: The effects of AZD6244 on the developing human fetus are unknown; for this reason, women of child-bearing potential and men must agree to use adequate contraception (hormonal or barrier method of birth control; abstinence) prior to study entry and for the duration of study participation, and for four weeks after dosing with AZD6244 ceases; women of child-bearing potential must have a negative pregnancy test prior to entry; should a woman become pregnant or suspect she is pregnant while she or her partner is participating in this study, she should inform her treating physician immediately; please note that the AZD6244 manufacturer recommends that adequate contraception for male patients should be used for 16 weeks post-last dose due to sperm life cycle * ELIGIBILITY FOR TREATMENT CRITERIA FOR PHASE II STUDY: Ability to understand and the willingness to sign a written informed consent document according to institutional guidelines * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Patients must have recurrence or progression of their low-grade glioma after coming off treatment with AZD6244 on PBTC-029 or PBTC-029B, with or without having received additional anti-tumor therapy following discontinuation of AZD6244; the progression must be unequivocal and sufficient to warrant re-treatment in the opinion of the investigator; progression will be defined as either progressive disease (PD) that meets the study definitions of progressive disease by MRI or vision deterioration thought to be related to tumor in patients with optic pathway tumors * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Patients must have received treatment on PBTC-029 or PBTC-029B for a minimum of 12 courses with at least stable disease, or had a sustained response (partial response \[PR\]/ complete response \[CR\]) but remained on treatment \< 12 courses * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Patients must have bi-dimensionally measurable disease defined as at least one lesion that can be accurately measured in at least two planes * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Patients must have fully recovered from the acute toxic effects of all prior chemotherapy, immunotherapy, biologic therapy or radiotherapy prior to study entry * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Myelosuppressive chemotherapy: Patients must have received their last dose of known myelosuppressive anticancer chemotherapy at least three weeks prior to study registration or at least six weeks if nitrosourea * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Biologic agent: Patient must have received their last dose of the biologic agent \>= 7 days prior to study registration * For biologic agents that have a prolonged half-life, at least three half-lives must have elapsed prior to registration * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Other Investigational Agents (not fitting into one of the above specified categories): * Patients must have received their last dose of any other investigational agent greater than 28 days prior to enrollment * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Radiation: patients must have: * Had their last fraction of local irradiation to primary tumor \>= 12 months prior to registration; investigators are reminded to review potentially eligible cases to avoid confusion with pseudo-progression * Had their last fraction of craniospinal irradiation (\> 24 Gy) \> 3 months prior to registration * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Corticosteroids: patients who are receiving dexamethasone must be on a stable or decreasing dose for at least 1 week prior to registration * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Growth factors: Patients must be off all colony-forming growth factor(s) for at least 1 week prior to registration (filgrastim, sargramostim, erythropoietin) and at least 2 weeks for long-acting formulations * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Patients with neurological deficits should have deficits that are stable for a minimum of 1 week prior to registration * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Patients must be able to swallow capsules * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Karnofsky performance scale (KPS for \> 16 yrs. of age) or Lansky performance score (LPS for =\< 16 years of age) \>= 60 assessed within two weeks prior to registration * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Absolute neutrophil count \>= 1,000/uL (unsupported) (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Platelets \>= 100,000/L (unsupported) (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Hemoglobin \>= 8 g/dL (may be supported) (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: AST (SGOT)/ALT (SGPT) =\< 2.5 X institutional upper limit of normal for age (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Total bilirubin \< 1.5 times upper limit of normal for age (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Albumin \>= 3 g/dL (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Serum sodium and potassium within the institutional limits of normal (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Serum calcium and magnesium above the institutional lower limit of normal (within 14 days of registration and within 7 days of the start of treatment) * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Creatinine clearance or radioisotope GFR \>= 70 ml/min/1.73m\^2 or a serum creatinine based on age as follows (within 14 days of registration and within 7 days of the start of treatment): * =\< 5 years: 0.8 mg/dL * \> 5 years but =\< 10 years: 1 mg/dL * \> 10 years but =\< 15 years: 1.2 mg/dL * \> 15 years: 1.5 mg/dL * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: LVEF \>= 55% * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: QTc interval =\< 450 msecs * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: Female patients of childbearing potential must not be pregnant or breast-feeding; female patients of childbearing potential must have a negative serum or urine pregnancy test * ELIGIBILITY CRITERIA FOR ENROLLMENT ON THE RE-TREATMENT STUDY: The effects of AZD6244 on the developing human fetus are unknown; for this reason, women of child-bearing potential and men must agree to use adequate contraception (hormonal or barrier method of birth control; abstinence) prior to study entry and for the duration of study participation, and for four weeks after dosing with AZD6244 ceases; women of child-bearing potential must have a negative pregnancy test prior to entry; should a woman become pregnant or suspect she is