Ganglionic glioma

Summarize

The body's central nervous system consists of the brain and spinal cord. The brain and spinal cord contain many neurons and other supporting cells (such as glial cells). Neurons are specialized nerve cells that send messages from the central nervous system to the rest of the body. These neurons are interconnected by the release and recognition of chemicals, and by neuroelectric signals.

A ganglion is anatomically a clump of biological tissue, usually a collection of nerve cell bodies. The cells within a ganglion are called ganglion cells, which are nodular formations of functionally identical collections of neuronal cell bodies.

Neuronal tumors are a rare class of tumors that occur in the brain or spinal cord. Many of these types of tumors are benign and do not spread to other parts of the body. However, these tumors can still cause seizures or other problems by pressing on the surrounding nerve tissue.

Ganglionic gliomas are a rare type of neuronal tumor and are low-grade malignant mixed cell tumors containing glial cells (responsible for providing structural support to the central nervous system) and neuronal cells (functional components of the central nervous system). Clinical treatment options for neuronal tumors are generally surgical removal, and in some cases, chemotherapy and radiation therapy are also optional treatments.

Epidemiological

Ganglion gliomas occur primarily in young children and adolescents. Children with certain genetic syndromes, including neurofibromatosis and tuberous sclerosis, have a higher risk of developing ganglionic gliomas. However, most ganglion gliomas are spontaneous and have little or no cause.

The temporal lobe of the brain is the most common site of development, with ganglion cell tumors of the brainstem and spinal cord being less common. Within the spinal cord, ganglioneuronal gliomas most commonly involve the cervical cord, although cases involving the thoracic and lumbar medullas and the full length of the spinal cord have been reported. Spinal ganglion cell tumors occur in a wide age range from newborns to the elderly, but most often occur in children or young adults. No differences in incidence have been found by sex, race, or ethnicity.

Etiolog & Risk factors

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Classification & Stage

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Clinical manifestations

Because ganglion gliomas grow relatively slowly, children may have symptoms for months or longer before diagnosis. Many children with ganglion gliomas first develop seizures, and other symptoms associated with increased intracranial pressure include: headaches (usually upon waking in the morning), occasional nausea and vomiting, fatigue, and weakness on one side of the body. Symptoms of ganglion glioma may be confused with symptoms of other neurological conditions and require detailed consultation with a medical professional for diagnosis.

Clinical Department

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Examination & Diagnosis

In addition to physical examination, taking a history, and neurological examination (testing reflexes, muscle strength, eye and mouth movements, coordination, and alertness), other tests include imaging and neurophysiologic examinations, as referenced below:

health checkup

Patients may exhibit a range of symptoms of elevated intracranial pressure.

Computed tomography (also known as CT scan)

A diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce horizontal and vertical cross-sectional images (often called slices) of the body.CT scans are more detailed than plain x-rays. For suspected ganglion gliomas, a CT scan of the brain is usually performed.

Magnetic resonance imaging (MRI)

A combination of magnetic fields, radiofrequency, and computers are used to generate detailed images of organs and structures within the body. For the diagnosis of ganglion gliomas, MRIs of the brain and spinal cord are often necessary.

electroencephalogram (EEG)

For children who have had seizures, the test records continuous electrical activity in the brain through electrodes attached to the scalp and helps identify and localize the specific part of the brain where the seizures are occurring.

histological biopsy

Tissue samples of the tumor are removed through a biopsy needle during surgery performed by the surgeon, and the diagnosis is confirmed by tissue staining and microscopic examination.

Clinical Management

Treatment of ganglionic gliomas generally includes the following tools:

1. Neurosurgery

It may be possible to safely remove tumors through this procedure, but surgery is often limited because these tumors are often located in deep central locations in the brain.

2. Chemotherapy

Tumor growth is controlled by stopping or slowing the growth and division of tumor cells through injections or oral administration of chemotherapy drugs.

3. Radiotherapy

The use of high-energy X-rays or other rays to control the local growth of tumors. Because of the side effects associated with long-term use of this therapy, it is generally only recommended for patients whose tumors have recurred. Meanwhile, there is still no clear conclusion as to whether radiation therapy can improve the long-term survival of ganglion gliomas.

4. Targeted therapy

Mutations in genes that promote cell division (e.g., the BRAF gene) are present in some children's tumors, and recent clinical reports have shown that targeted drugs against these mutations are effective in controlling tumor growth in some patients.

Specific treatments will be considered by the specialist based on a combination of factors such as the patient's age, general health and medical history, the type, location and size of the tumor, the extent of the disease, tolerance to specific medications, treatments or therapies, and the doctor's expectations for disease progression. The recommended treatment for progressive or recurrent ganglionic gliomas is radiation therapy. Clinical trials and experimental therapies may also be used for recurrent tumors that do not respond to standard therapies.

Prognosis

Patients with ganglion gliomas have a five-year survival rate of 80 to 90 percent after diagnosis. The extent of resection at initial surgery is the most significant factor affecting patient survival.

Follow-up & Review

The recurrence rate of ganglion gliomas ranges from 20% to 30%, and patients with less differentiated tumors and who are younger at the time of diagnosis are less likely to recur after surgery. Due to the large time span of recurrence for this type of tumor, it is recommended to maintain regular follow-up under the supervision of a physician.

Routine

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Cutting-edge Therapeutic & Clinical Research

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References

1. https://www.saintlukeskc.org/health-library/neuronal-and-mixed-neuronal-glial-tumors
        2. https://www.stjude.org/disease/ ganglioglioma.html
        3. http://www.danafarberbostonchildrens.org/conditions/brain-tumor/ganglioglioma.aspx
        4. Oppenheimer DC, et al. Ganglioglioma of the Spinal Cord. J Clin Imaging Sci 2015, 5:53.
        5. Pekmezci M, et al. The genetic landscape of ganglioglioma. Acta Neuropathol Commun. 2018 Jun 7;6(1):47;
        6. Beland B , et al. Unprecedented response to combination BRAF and MEK inhibitors in adult anaplastic ganglioglioma. J Neurooncol. 2018 May;137(3):667 -669;
        7. marks AM , et al. Response to the BRAF/MEK inhibitors dabrafenib/trametinib in an adolescent with a BRAF V600E mutated anaplastic ganglioglioma intolerant to vemurafenib. Pediatr Blood Cancer. 2018 May;65(5):e26969.
        8. Yust-Katz S et al. Clinical and prognostic features of adult patients with gangliogliomas. Neuro Oncol. 2014 Mar;16(3):409-413.