Stereotactic Biopsy Split-Course Radiation Therapy in Diffuse Midline Glioma, SPORT-DMG Study

This phase II trial studies the clinical outcomes of hypofractionated radiation therapy in patients with diffuse midline gliomas. This study aims to change the way radiation is delivered, from giving 6 weeks of radiation all at once to giving 2 weeks of radiation. This may determine if there is a difference in the outcome of the treatment, and most importantly, the patients' quality of life.

PRIMARY OBJECTIVE: I. To estimate the time to progression after the second hypofractionated radiation course of 25 Gy in 10 fractions in patients with diffuse midline glioma of the pons, calculated from date of diagnosis. SECONDARY OBJECTIVES: I. To evaluate the quality of life outcomes for patients with diffuse midline gliomas of the pons. II. To estimate progression free survival intervals for patients after each hypofractionated radiation treatment course. III. To evaluate the quality of life outcomes for parents of patients =< 18 years with the Functional Assessment of Cancer Therapy General (FACT-G) Family/Caregiver Questionnaire. IV. To estimate the overall survival for patients with diffuse midline gliomas of the pons treated with planned multi-course hypofractionated radiation courses. V. To report toxicities associated with hypofractionated, planned, multi-course radiation treatment for diffuse midline glioma of the pons. OUTLINE: Patients undergo hypofractionated radiation therapy (RT) over 10 fractions. Patients who achieve progression undergo up to 2 retreatment courses. After completion of study treatment, patients are followed up at 1 month, every 2 months for year 1, every 3 months for year 2, then every 6 months for year 3.

Patients undergo hypofractionated RT over 10 fractions. Patients who achieve progression undergo up to 2 retreatment courses.

From date of diagnosis to the date of radiographic or clinical progression following second course of hypofractionated radiation therapy, assessed up to 3 years

Results from Pediatric Quality of Life Inventory Brain Tumor Module will be used to evaluate the quality of life outcomes in patients up to 18 years old and National Comprehensive Cancer Network-Functional Assessment of Cancer Therapy (FACT)-Brain Symptom Index-24 in patients more than 18 years old. Changes in raw score from baseline to each time-point will be assessed, in each of the physical and mental health domains. The Wilcoxon signed-rank test will be utilized to assess changes in raw scores. Mean change, along with standard deviation will be reported.

From registration date to the earliest date of documentation of progression after each course of radiation therapy (RT) or death due to any cause, assessed up to 3 years

Changes in raw score from baseline to each time-point will be assessed, in each of the physical and mental health domains. The Wilcoxon signed-rank test will be utilized to assess changes in raw scores. Mean change, along with standard deviation will be reported.

From registration date to death due to any cause, censoring patients alive at the date of last contact, assessed up to 3 years

The maximum grade for each type of adverse event will be recorded for each patient, and frequency tables will be reviewed to determine patterns. Additionally, the relationship of the adverse event(s) to the study treatment will be taken into consideration.

Inclusion Criteria: Age >= 1 year(s) old (no maximum age) Radiologic appearance of diffuse midline glioma of the pons, including diffuse infiltration of >= 50% of the pons on MRI, with or without extension to the midbrain and/or medulla oblongata with at least 1 of the 3 brainstem symptoms (cranial nerve deficit, long tract sign, or cerebellar sign) If all features of this clinicoradiologic criteria are met, then patients can continue on protocol with or without a biopsy If all features of this clinicoradiologic criteria are not met, patients must receive a brainstem lesion biopsy to be treated on protocol. If this cannot be completed, patients will be withdrawn from the study If biopsy has already been completed at an outside institution, the outside pathology report will be reviewed ahead of trial enrollment. The pathology specimen will then be sent to Mayo Clinic for further review, but will not delay study enrollment Able to undergo MRI Brain Negative urine pregnancy test completed =< 7 days prior to registration, for women of childbearing potential only Primary language of English or Spanish for patients and their caregiver Patient or caregiver willing and able to provide written informed consent Caregiver able to complete questionnaires by themselves or with assistance Willing to return to enrolling institution for follow-up during the active monitoring phase of the study Exclusion Criteria: Any patient who has received previous radiation to the brain Any patient who has received previous chemotherapy Any patient with a diagnosis of neurofibromatosis type 1 or 2 (NF1 or NF2) Any of the following: Pregnant women Nursing women Women of childbearing potential who are unwilling to employ adequate contraception Co-morbid systemic illnesses or other severe concurrent disease which, in the judgment of the investigator, would make the patient inappropriate for entry into this study or interfere significantly with the proper assessment of safety and toxicity of the prescribed regimens Uncontrolled intercurrent illness including, but not limited to, ongoing or active infection, symptomatic congestive heart failure, unstable angina pectoris, cardiac arrhythmia, or psychiatric illness/social situations that would limit compliance with study requirements Other active malignancy =< 5 years prior to registration. EXCEPTIONS: Non-melanotic skin cancer, breast cancer, prostate cancer, well-differentiated thyroid cancer, carcinoma-in-situ of the cervix NOTE: If there is a history or prior malignancy, they must not be receiving other specific treatment for their cancer Patients > 16 years with an Eastern Cooperative Oncology Group (ECOG) score >= 4 and patients =< 16 years with a Lansky play scale =< 20