Gliomatosis cerebri

Gliomatosis cerebri (GC) is a rare and aggressive primary brain tumor characterized by diffuse, widespread infiltration of glial tumor cells throughout the brain, involving at least three cerebral lobes. Unlike more localized brain tumors, gliomatosis cerebri spreads extensively through the brain parenchyma, often affecting both cerebral hemispheres and sometimes extending into the brainstem, cerebellum, and spinal cord. The World Health Organization previously classified it as a distinct entity, but in the 2016 WHO classification of CNS tumors, it was reclassified as a growth pattern rather than a separate tumor type, typically representing a diffuse astrocytic or oligodendroglial tumor. Clinical presentation is variable and depends on the regions of the brain affected. Key symptoms include progressive cognitive decline, personality and behavioral changes, headaches, seizures, corticospinal tract deficits (such as weakness on one side of the body), visual disturbances, and increased intracranial pressure. Because the tumor infiltrates diffusely rather than forming a discrete mass, symptoms may develop insidiously over weeks to months before diagnosis. MRI imaging typically reveals widespread T2/FLAIR signal abnormality across multiple brain regions, often without significant contrast enhancement or mass effect. Treatment of gliomatosis cerebri remains challenging due to its diffuse nature, which precludes complete surgical resection. Management typically involves radiation therapy and chemotherapy, with temozolomide being the most commonly used chemotherapeutic agent. Whole-brain radiation therapy or involved-field radiation may be employed depending on the extent of disease. Despite treatment, the prognosis is generally poor, with median survival ranging from approximately 12 to 24 months, though outcomes vary based on molecular features, patient age, and performance status. Molecular profiling, including IDH mutation status and 1p/19q codeletion testing, is increasingly important for guiding treatment decisions and prognostication.

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