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Brainstem Gliomas

Practice Essentials

As the name implies, brainstem gliomas occur in the region of the brain stem. Approximately 60% of the time they are centered within the pons, but can arise from the midbrain or medulla, and can infiltrate beyond the brainstem. They account for about 20% of all pediatric primary brain tumors, but constitute less than 2% of all adult gliomas. 

Axial T2 FLAIR MRI image showing a mass in the bra

Axial T2 FLAIR MRI image showing a mass in the brainstem consistent with brainstem glioma.

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Signs and symptoms

Common presenting signs and symptoms include the following:

Double vision

Weakness

Unsteady gait

Difficulty in swallowing

Dysarthria

Headache (most common presenting symptom in adults)

Drowsiness

Nausea

Vomiting

Behavioral changes or seizures in children (rare)

Deterioration of handwriting and speech, in older children

Common clinical findings on physical examination can be summarized as constituting a triad of cranial nerve deficits, long tract signs, and ataxia (of trunk and limbs). Papilledema may be seen. Sixth and seventh cranial nerves are involved commonly. Facial sensory loss and a primary-position, upbeating nystagmus may be seen. Crossed deficits (facial signs and symptoms contralateral to arm/leg signs and symptoms) are also characteristic of brainstem gliomas.

Certain manifestations suggest specific tumor locations, as follows:

Infants and children with failure to thrive – Pontine gliomas

Involvement of cranial nerve III or IV – A mesencephalic component

Hydrocephalus – Tumors in periaqueductal or fourth ventricle outflow locations

Patients with tectal lesions may present with the following:

Headache, nausea, and vomiting

Diplopia

Parinaud syndrome

Patients with cervicomedullary lesions may present with the following:

Dysphagia, unsteadiness, nasal speech, vomiting, and weakness

Sensory loss in the face (involvement of the trigeminal nucleus)

Dysphagia and/or dysphonia from lower cranial nerve involvement (commonly IX and X)

Long tract signs

Ataxia

Downbeating nystagmus and oculomyoclonus (medullary involvement)

See Clinical Presentation for more detail.

Diagnosis

The workup for brainstem gliomas may include the following:

MRI of the head – Diagnostic test of choice

CT scan – Less accurate than MRI, but appropriate when MRI is not available

CSF examination – Often used to evaluate for other diagnoses affecting the brainstem

Arteriography – Occasionally useful in differentiating vascular lesions, including tumors, from gliomas

Lab studies of blood chemistry and related body fluids are not helpful as a rule. Tissue confirmation is recommended, but sometimes not feasible.

See Workup for more detail.

Management

Treatment of brainstem gliomas may comprise the following:

Focal radiotherapy

Chemotherapy

Surgical resection/biopsy

Observation alone may be considered for some adult patients with the following:

A tectal lesion

A cervicomedullary lesion

Mild symptoms of long duration

Focal radiotherapy

Remains the cornerstone of treatment for brainstem gliomas

Can improve or stabilize the patient’s condition

Should be administered to any patient with significant and progressive neurologic symptoms

Conventional doses of radiotherapy ranges from 54 to 60 Gy

Reported survival rates with radiation therapy are better in patients with exophytic tumors

Chemotherapy

Chemotherapy with agents such as temozolomide may benefit some patients with high-grade histology (glioblastoma)

Chemotherapy at relapse may benefit some patients

Chemotherapy may include conventional agents such as temozolomide and carboplatin/vincristine

Antiangiogenesis agents (eg, thalidomide, bevacizumab) have been used with success in supratentorial gliomas, but with variable success in brainstem gliomas

Patients should be encouraged to enroll in clinical trials (if available)

Surgical resection

Surgical therapy is performed in conjunction with radiation therapy, chemotherapy, or both. While it is not required for diagnosis or treatment of brainstem glioma, judicious use of biopsy/resection is recommended when safe. Surgery may improve symptomatic control and provide tissue for molecular testing for both prognosis and potential therapeutic implications. It should particularly be considered in the following situations:

Tumors of the cervicomedullary junction

Dorsal exophytic tumors protruding into the fourth ventricle

Cystic tumors

Enhancing tumors with clear margins that exert a space-occupying effect

Benign tumors (ie, those with slow clinical progression)

See Treatment and Medication for more detail.

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