Infant-Type Hemispheric Gliomas: A Review of Clinical, Radiologic, Histopathologic, and Molecular Features

Area of Science:

• Pediatric neuro-oncology
• Tumorigenesis and molecular pathology
• Clinical trial design

Background:

• Infant-type hemispheric gliomas (IHGs) are rare, large, hemorrhagic cerebral tumors diagnosed in infancy.
• Standard treatment involves surgery and chemotherapy, adapted from high-grade glioma trials.
• While survival rates are good, survivors face significant long-term neurologic and neurocognitive deficits.

Purpose of the Study:

• To review historical clinical trials for IHGs.
• To delineate the histopathologic and molecular characteristics of IHGs.
• To identify current therapeutic gaps and the need for standardized approaches.

Main Methods:

• Literature review of historical clinical trials.
• Analysis of histopathologic and molecular data for IHGs.
• Synthesis of current treatment outcomes and molecular drivers.

Main Results:

• IHGs are molecularly distinct, driven by RTK fusions and sharing features with desmoplastic infantile tumors.
• Current treatment regimens result in high rates of morbidity, hemorrhage, and progression.
• Tyrosine kinase inhibitors (TKIs) show potential but are primarily used in recurrent settings.

Conclusions:

• Standard treatment for IHGs yields poor long-term neurodevelopmental outcomes.
• Molecular insights reveal IHGs are targetable by TKIs, though their primary role is undefined.
• Collaborative research is crucial to establish standardized, improved treatment protocols for IHGs.