Ganglioglioma

What is Ganglioglioma?

Ganglioglioma is a slow growing tumor develops in the central nervous system. The nature of the tumor is benign and main clinical presentation is an epileptic seizure. The location of the tumor is not specific, as it found in any place of central nervous system, but commonly found in the temporal lobe.

Ganglioglioma is usually considered as a low-grade tumor with the negligible prospect to spread to other parts, but in the rare instance, it can transform to high-grade tumor having to expandability. Cerebrum, the motor and sensory action controlling brain part mostly affected with Ganglioglioma^1,2.

Ganglioglioma predominance

The onset of ganglioglioma usually occurs at the early age of life, therefore, children and adolescents are most vulnerable. The predominance of ganglioglioma is same for both the gender. The incidence rate is almost equal to 2% of all primary intracranial tumors, whereas primary cerebral tumors account up to 10% in children. The incidence of ganglioglioma is rare².

Causes of Development

Transformation of neuronal and glial elements causes the development of ganglioglioma. The underlying cause of this transformation is usually sporadic. But, children have definite genetic syndromes such as tuberous sclerosis and neurofibromatosis 1, have a high risk of generating glial tumors, including gangliogliomas⁵.

Signs & symptoms

Ganglioglioma is relatively slow growing a tumor and may symptoms are not prominent and cause delayed diagnosis. The prime symptom of Ganglioglioma is episodic epileptic seizure attack and others are as follows:

• Headaches
• High intracranial pressure
• Nausea
• Vomiting
• Blurry vision
• Paresthesia or one-sided body weakness

The above mentioned clinical presentations are not specific, therefore different diagnostic tests are performed for analyzing the characteristic features or signs of the gangliogliomas. The following signs may find out:

• Papilloedema, without any central or localized neurological deficits
• Acute deterioration
• Decreased level of consciousness
• Progressive hemiparesis that may need urgent neurosurgical admission^2,3,4

Ganglioglioma Diagnosis

The following are the different diagnostic tests are available to detecting the gangliogliomas.

Initially, clinician prefers to conduct a physical examination and symptomatic discussion. Depending upon the findings of physical examination and analyzing the medical history the following neurological examining are conducted like

• Reflex testing
• Muscle strength
• Eye movement
• Mouth movement
• Body coordination
• Body balancing
• Alertness

After conducting the initial examination the following confirmatory tests are conducted, like

• Magnetic resonance imaging (MRI)
• Electroencephalogram
• Computerized tomography (CT or CAT) scan
• Biopsy

Doctor select the treatment plan for an individual patient depending upon the test results of the above-mentioned tests^2,3,4.

Ganglioglioma Treatment

The following treatment options are available to treat gangliogliomas. These treatments include:

• Neurosurgery

The surgical intervention assists in taking out the possible portion of lump. The complete removal of the tumor is difficult as the tumor is deep sited within the brain. Different neurosurgery program is launched to provide training to a neurosurgeon for successful removal of the tumor. Recently Intra-operative MRI develops to allow the most complete and safest method for removal of a tumor from the brain.

• Chemotherapy

If tumor become high graded or malignant or size tumor is large, then usually chemotherapy is applied to shrink the size of the tumor or to destroy malignant cells.

• Radiation therapy

Targeted radiation therapy is applied to restrict the growth of the tumors. Usually, radiation therapy is not recommended for children as other surrounded tissue become necrosis and other due to another long-term side effect. Radiation therapy is only applied to a child when the tumor re-growth occurs.

A seizure is one of the primary clinical presentation of the gangliogliomas. The management of the seizure attack is usually difficult. The following are the treatment management options available for gangliogliomas.

• The specialized neurologist may require treating epilepsy, as usual, anti-epileptic drugs do not provide sufficient positive result.
• The tumor removal through surgical intervention is provided best treatment outcome to control seizure^2,3,4,5.

Ganglioglioma Prognosis

There is less chance to recur the tumor, as most of the gangliogliomas are benign is nature. In the case of progressive tumor or recurrence of the condition, then radiotherapy is applied to get the best outcome. Different experimental and clinical trials are conducted to develop a novel therapy to treat a recurrence of the lump, as many patients are not responsive to standard therapies. Therefore, patients can enroll in these types of trial program to explore themselves in novel therapies ⁵.

Ganglioglioma Survival rate

Early detection and complete removal of the gangliogliomas have high survival rate. But this rate is lower in the case of complete removal of the tumor is not possible or come back the condition.

The average survival rate is 5.7 years in almost 93% cases, but this is not constant for all the cases, as WHO grading the gangliogliomas into three categories Grade I, Grade II and Grade III. The prognosis of ganglioglioma depends upon this grading. In the case of Grade I, the survival rate is usually 10 years for 84-93%, but it becomes decrease with increasing the grade of the ganglioglioma^5,6.

References

1. Dr. Yuranga Weerakkody, A.Prof Frank Gaillard. et al.; Ganglioglioma; Radiopaedia.org; Online available at https://radiopaedia.org/articles/ganglioglioma
2. Jye Young Song, Jeong Hoon Kim, Young Hyun Cho, Chang Jin Kim, Eun Jung Lee; Treatment and Outcomes for Gangliogliomas: A Single-Center Review of 16 Patients; Brain Tumor Res Treat. 2014 Oct; 2(2): 49–55.
3. Published online 2014 Oct 31. doi:  10.14791/btrt.2014.2.2.49; available at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4231627/
4. Ajay Pandita, Anandh Balasubramaniam, Richard Perrin, Patrick Shannon, Abhijit Guha; Malignant and benign ganglioglioma: A pathological and molecular study; Neuro Oncol (April 2007) 9 (2):124-134.doi: 10.1215/15228517-2006-029; available at http://neuro-oncology.oxfordjournals.org/content/9/2/124.full
5. Ganglioglioma; UCLA Health; http://neurosurgery.ucla.edu/ganglioglioma
6. Ganglioglioma Overview; Dana Farber Boston Children’s Cancer And Blood Disorders Center; http://www.danafarberbostonchildrens.org/conditions/brain-tumor/ganglioglioma.aspx
7. Luyken C, Blümcke I, Fimmers R, Urbach H, Wiestler OD, Schramm J. Supratentorial gangliogliomas: histopathologic grading and tumor recurrence in 184 patients with a median follow-up of 8 years. Cancer.2004;101:146–155.

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