Erivedge (Vismodegib) in the Treatment of Pediatric Patients With Refractory Pontine Glioma

Inclusion Criteria: * Subjects must have radiographically proven diffuse intrinsic pontine glioma and confirmation of residual disease after initial therapy or at the time of recurrence/progression as confirmed by MRI of the brain * Subjects must be age ≥3 years and ≤ 18 years * Diffuse intrinsic pontine glioma with measurable disease after receiving radiotherapy either concurrent with or followed by ≤ 2 prior courses of chemotherapy * Measurable disease as defined by: Measurable tumor \>10mm by MRI * Karnofsky performance status (PS) 60-100% (for patients \> 16 years of age) OR Lansky PS 60-100% (for patients ≤ 16 years of age) * Body surface area \> 0.67 m2 and ≤ 2.21 m2 * Life expectancy of at least 2 months * A negative urine pregnancy test is required for female participants of child bearing potential (≥13 years of age or after onset of menses) * Acceptable liver function as defined by: 1. Bilirubin ≤ 1.5 times upper limit of normal 2. AST (SGOT), ALT (SGPT) and Alkaline phosphatase ≤ 2.5 times upper limit of normal * Creatinine clearance or radioisotope GFR ≥ 70 mL/min OR serum creatinine based on age as follows: * 0.8 mg/dL (for patients ≤ 5 years of age) * 1.0 mg/dL (for patients 6 to 10 years of age) * 1.2 mg/dL (for patients 11 to 15 years of age) * 1.5 mg/dL (for patients \> 15 years of age) * Acceptable hematologic status as defined by: 1. Granulocyte ≥ 1500 cells/mm3 2. Platelet count ≥ 100,000 (plt/mm3) 3. Serum albumin ≥ 2.5 g/dL * Urinalysis: a. No clinically significant abnormalities * Acceptable coagulation status as defined by: 1. PT/INR less than 1.5 2. PTT within normal limits * Subjects must be able to swallow and retain oral medication * Female post-pubertal study subjects need to agree to use one of the more effective birth control methods during treatment and for 7 (seven) months after treatment is stopped. These methods include total abstinence (no sex), oral contraceptives ("the pill"), an intrauterine device (IUD), levonorgestrol implants (Norplant), or medroxyprogesterone acetate injections (Depo-provera shots). * Male post-pubertal study subjects need to agree to use condoms with spermicide, even after a vasectomy, during sexual intercourse with female partners while being treated with Erivedge capsule and for 2 months after the last dose to avoid exposing an embryo or fetus to Vismodegib. * Voluntarily signed and dated a written IRB-approved informed consent by parent or legal guardian of subject Exclusion Criteria: * Concurrent anti-cancer therapy (chemotherapy, radiation therapy, surgery, immunotherapy, hormonal therapy, biologic therapy) other than the ones specified in the protocol. Patients must have discontinued the above cancer therapies for generally about 3 weeks (8 weeks for radiotherapy) prior to the first dose of study medication, as well as recovered from toxicity (to ≤ than grade 2 except for alopecia) induced by previous treatments. * Currently receiving another investigational medicinal product. * Uncontrolled concurrent illness including, but not limited to: 1. Active, uncontrolled bacterial, viral, or fungal infections requiring systemic therapy 2. Diarrhea of any cause ≥ CTCAE grade 2 3. Psychiatric illness/social situations that would compromise patient safety or limit compliance with study requirements including maintenance of a compliance/pill diary 4. Any kind of malabsorption syndrome significantly affecting gastrointestinal function * Pregnant or nursing female patients. NOTE: If a female patient becomes pregnant or suspects that she is pregnant while participating in this study, she should stop taking study drug and immediately inform her treating physician immediately. * Prior therapy with a Hedgehog inhibitor * Unwillingness or inability to comply with procedures required in this protocol * Serious nonmalignant disease (e.g., hydronephrosis, liver failure, or other conditions) that could compromise protocol objectives in the opinion of the investigator and/or the sponsor. * History of Congestive Heart Failure (CHF) or ventricular arrhythmia requiring medication.