Vemurafenib and Cobimetinib in Treating Patients with BRAF V600E Mutation Positive Craniopharyngioma

Inclusion Criteria

• Pre-registration: Patients must have local diagnosis of papillary craniopharyngioma and have tissue slides available for submission to central pathology review; central pathology review will include immunohistochemistry (IHC) testing for BRAF V600E mutation (VE1 clone) and beta-catenin IHC (membranous, non-nuclear pattern) if needed to confirm diagnosis of papillary craniopharyngioma
• Histologically proven papillary craniopharyngioma as documented by central pathology review with positive BRAF V600E mutation by IHC
• Measurable disease and/or non-measurable disease * Measurable disease, defined as bidimensionally measurable lesions with clearly defined margins by magnetic resonance imaging (MRI) scans, with a minimum diameter of 10 mm in both dimensions * Progressive disease required in cohort B, defined as any progressive measurable disease after surgery and radiation; progressive or recurrent disease is not required in cohort A, but is allowed provided it is a new diagnosis and patient has not received prior treatment
• Prior treatment * Cohort A: No prior therapy received other than surgery * Cohort B: Prior radiation therapy required (any type of prior radiation is allowed) ** For patients treated with external beam radiation therapy, interstitial brachytherapy or radiosurgery, an interval of >= 3 months must have elapsed from completion of radiation therapy to registration ** Recovered to Common Terminology Criteria for Adverse Events (CTCAE) grade 1 or less toxicity attributed to radiation with exception of alopecia and fatigue * For patients enrolling on Cohort A or Cohort B: ** For patients treated with surgery, an interval of >= 21 days must have elapsed prior to registration ** No prior treatment with BRAF or MEK inhibitors ** Steroid dosing stable for at least 4 days prior to registration
• Not pregnant and not nursing, because this study involves an agent that has known genotoxic, mutagenic and teratogenic effects; therefore, for women of childbearing potential only, a negative pregnancy test done =< 7 days prior to registration is required
• Age >= 18 years
• Eastern Cooperative Oncology Group (ECOG) performance status =< 2
• Comorbid conditions * No evidence of active bleeding, bleeding diathesis, or hemoptysis (>= 1/2 teaspoon of red blood) =< 8 weeks prior to registration * No evidence of intracranial hemorrhage =< 4 weeks prior to registration * Patients who have experienced thromboembolic event within 6 months prior to registration must be on stable therapeutic anticoagulation for at least 4 weeks prior to registration * No symptomatic congestive heart failure (New York Heart Association class II, III, or IV) within 6 months prior to registration * No current unstable angina or uncontrolled arrhythmia * No uncontrolled hypertension at time of registration (blood pressure [BP] > 150/95 despite antihypertensive therapy) * No known history of prolonged QT syndrome * No known history of ventricular arrhythmia within 6 months of registration * No known history of uveitis or iritis =< 4 weeks prior to registration * No evidence of retinal pathology that is considered a risk factor for neurosensory retinal detachment, retinal vein occlusion (RVO), or neovascular macular degeneration within 12 months of registration
• Concomitant medications * Chronic concomitant treatment with strong CYP3A4 inducers or CYP3A4 inhibitors is not allowed; patients must discontinue the drug at least 14 days prior to study registration * Chronic concomitant treatment with CYP1A2 substrate is not allowed; patients must discontinue the drug at least 14 days prior to study registration
• Absolute neutrophil count >= 1500/mm^3
• Platelets >= 100,000/mm^3
• Creatinine =< 1.5 mg/dL OR creatinine clearance >= 45 mL/min
• Bilirubin =< 1.5 upper limit of normal (ULN)
• Aspartate aminotransferase (AST)/alanine aminotransferase (ALT) =< 2.5 ULN