This study will evaluate BCB-276, an investigational B7-H3-targeted Chimeric Antigen Receptor (CAR) T cell therapy, in children and young adults with diffuse intrinsic pontine glioma (DIPG). DIPG is a rare and aggressive brain tumor with limited treatment options. CAR T cell therapy uses a patient's own immune cells that are changed in a laboratory to recognize and attack cancer cells. The purpose of this study is to determine whether BCB-276, when given after completion of standard radiation therapy, is safe and can improve survival for patients with DIPG.
To participate, individuals must be between 1 and 26 years of age when they join the study, have a diagnosis of DIPG, and enroll for treatment within 6 weeks of completing initial radiation therapy. Participants must not have received prior anti-cancer therapy beyond radiation with or without temozolomide prior to joining this study.
BCB-276 is administered intraventricularly (into the fluid around the brain), which requires placement of a catheter for treatment. BCB-276 is given every 2 weeks at a research center over a period of several months (approximately 7-8 months). Participation includes travel to a study site, procedures to support treatment administration, sample collection, and ongoing monitoring for safety and effectiveness, with follow-up visits lasting up to about 2 years.
Participants must be aged 1 and ≤ 26 years and weigh ≥10kg.
Diagnosis of Diffuse Intrinsic Pontine Glioma (DIPG) based on imaging, with or without biopsy confirmation consistent with high-grade glioma or diffuse midline glioma
Able to tolerate leukapheresis and other study procedures in the opinion of the Investigator.
Central Nervous System (CNS) reservoir catheter present prior to first dose of study drug.
Previous tumor-directed therapy or treatment-directed clinical study other than standard radiation with or without temozolamide.
Evidence of metastatic disease.
Requirement of high or increasing doses of corticosteroids prior to participation.
Severe swallowing difficulties or other significant clinical conditions that may interfere with participation.
Single group
BCB-276
Biological/VaccineFollowing completion of standard radiation therapy, eligible participants with DIPG will undergo leukapheresis, a procedure to collect white blood cells used to manufacture BCB-276, an autologous CAR T cell therapy made from the participant's own immune cells designed to target B7-H3. All eligible participants will receive up to 15 intraventricular administrations of BCB-276 every 2 weeks for an approximate total duration of 30 weeks.
Overall Survival
Up to 24 months from date of enrollment
Safety and Tolerability of BCB-276
Incidence of adverse events, including serious adverse events, and adverse events leading to discontinuation of study treatment.
Radiographic Response to BCB-276
Measure the frequency of disease response (progressive disease, stable disease, partial response, complete response) based on MRI assessment per RANO criteria.
Presence of BCB-276 in Cerebrospinal Fluid (CSF)
Quantify the presence and amount of BCB-276 in CSF.
Progression-Free Survival (PFS)
PFS as measured by time in months, from date of diagnosis to first incidence of clinical or radiographically confirmed progression.
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