Gliomatosis cerebri

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Gliomatosis cerebri
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Headache, seizures, cognitive dysfunction, personality changes
Complications	Increased intracranial pressure, hydrocephalus
Onset	Typically in adulthood
Duration	Progressive
Types	Type 1 (diffuse), Type 2 (focal)
Causes	Unknown, possibly genetic mutations
Risks	Genetic predisposition, radiation exposure
Diagnosis	MRI, biopsy
Differential diagnosis	Diffuse intrinsic pontine glioma, astrocytoma, oligodendroglioma
Prevention	N/A
Treatment	Chemotherapy, radiation therapy, palliative care
Medication	N/A
Prognosis	Poor, median survival 12-24 months
Frequency	Rare
Deaths	N/A

A rare type of brain tumor

Gliomatosis cerebri is a rare and aggressive form of brain tumor characterized by the diffuse infiltration of glial cells across multiple regions of the central nervous system. Unlike other types of glioma, gliomatosis cerebri does not form a distinct mass but rather spreads extensively, often involving at least three cerebral lobes.

Pathophysiology

Gliomatosis cerebri is primarily composed of astrocytes, a type of glial cell, and is classified as a World Health Organization (WHO) grade III tumor. The diffuse nature of the tumor makes it challenging to diagnose and treat. The tumor cells infiltrate the white matter and gray matter, disrupting normal neural pathways and leading to a variety of neurological symptoms.

Symptoms

The symptoms of gliomatosis cerebri are varied and depend on the regions of the brain affected. Common symptoms include:

• Headache
• Seizures
• Cognitive impairment
• Personality changes
• Motor dysfunction
• Visual disturbances

Diagnosis

Magnetic resonance imaging (MRI) is the primary tool for diagnosing gliomatosis cerebri. The MRI typically shows diffuse hyperintensity on T2-weighted images, with extensive involvement of the brain parenchyma. Biopsy may be performed to confirm the diagnosis and to differentiate it from other types of diffuse gliomas.

Treatment

Treatment options for gliomatosis cerebri are limited due to its diffuse nature. Standard treatment approaches include:

• Radiation therapy
• Chemotherapy with agents such as temozolomide
• Corticosteroids to reduce cerebral edema

Surgical resection is generally not feasible due to the widespread infiltration of the tumor.

Prognosis

The prognosis for patients with gliomatosis cerebri is generally poor, with a median survival of approximately 12 to 24 months after diagnosis. The aggressive nature of the tumor and its resistance to conventional therapies contribute to the poor outcome.

See also

• Glioma
• Astrocytoma
• Brain tumor
• Central nervous system