Ganglioglioma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Ganglioglioma is a tumor that arises from ganglion cells in the central nervous system. On gross pathology, ganglioglioma varies from partially cystic mass with a mural nodule to a solid mass expanding the overlying gyrus. On microscopic pathology, ganglioglioma is composed of ganglion cells and neoplastic glial cells with positive staining for synaptophysin, neuronal specific enolase, and GFAP. Ganglioglioma must be differentiated from dysembryoplastic neuroepithelial tumors, pleomorphic xanthoastrocytoma, oligodendroglioma, desmoplastic infantile ganglioglioma, astrocytoma, ependymoma, and transverse myelitis. Patients with ganglioglioma may progress to develop local recurrence. Complications that can develop as a result of ganglioglioma are metastasis and local recurrence. Prognosis is generally good with treatment.^[1] The hallmark of cerebral ganglioglioma is temporal lobe epilepsy.^[1] Common symptoms of spinal cord ganglioglioma include back pain, neck pain, radicular pain, weakness, paresthesia, gait disturbance, and bowel and bladder dysfunction. Less common symptoms of spinal cord ganglioglioma include Brown-Sequard syndrome, acute headache due to subarachnoid hemmorhage, progressive scoliosis, loss of motor function, and frequent falls.^[2] Common physical examination findings of ganglioglioma include weak irregular pulse, hypertension, wide pulse pressure, unilateral pupillary dilatation, abnormal ophthalmic examination, and focal neurological deficits.^[3] On x-ray, spinal cord ganglioglioma is characterized by scoliosis, bony remodelling, posterior vertebral body scalloping, and remodelling of the pedicle or posterior arch.^[2] On CT scan of the head, ganglioglioma is characterized by iso- or hypodense, calcification, bone remodelling, and enhancement of solid non-calcified component. On MRI of the brain, ganglioglioma is characterized by iso- to hypointense solid component on T1, variable contrast enhancement of solid component on T1 C+ (Gd), hyperintense solid component and variable signal in cystic component on T2, and calcification on T2 (GE/SWI).^[1] On MRI scan of spinal cord, ganglioglioma is characterized by mixed signal intensity on T1-weighted images, high intensity on T2, patchy enhancement on T1 C+ (Gd), and calcification with low signal blooming on gradient echo.^[2] The mainstay of therapy for ganglioglioma is surgery. In the brain, a reasonable resection margin can be achieved with surgery.^[1] Radiation therapy is usually reserved for patients when either incomplete resection is achievable or tumor recurrence occurs.^[1]

Ganglioglioma was first reported by CB Courville in 1930.^[4]

Ganglioglioma arises from neuronal glial cells, which are cells of the central nervous system. It is a rare disease that tends to affect children, adolescents, and young adults. Ganglioglioma affects male and female equally. On gross pathology, ganglioglioma varies from partially cystic mass with a mural nodule to a solid mass expanding the overlying gyrus. On microscopic pathology, ganglioglioma is composed of ganglion cells and neoplastic glial cells with positive staining for synaptophysin, neuronal specific enolase, and GFAP.

There are no established causes for ganglioglioma.

Ganglioglioma must be differentiated from dysembryoplastic neuroepithelial tumors, pleomorphic xanthoastrocytoma, oligodendroglioma, desmoplastic infantile ganglioglioma, astrocytoma, ependymoma, and transverse myelitis.

Ganglioglioma is the most frequent neuronal-glial CNS neoplasm. It is a rare disease that tends to affect children, adolescents, and young adults. Ganglioglioma affects male and female equally.

There are no established risk factors for ganglioglioma.

If left untreated, patients with ganglioglioma may progress to develop local recurrence. Complications that can develop as a result of ganglioglioma are metastasis and local recurrence. Prognosis is generally good with treatment.^[1]

There is no established system for the staging of ganglioglioma.

The hallmark of cerebral ganglioglioma is temporal lobe epilepsy.^[1] Most common symptoms of spinal cord ganglioglioma include back pain, neck pain, radicular pain, weakness, paresthesia, gait disturbance, and bowel and bladder dysfunction. Less common symptoms of spinal cord ganglioglioma include Brown-Sequard syndrome, acute headache due to subarachnoid hemmorhage, progressive scoliosis, loss of motor function, and frequent falls.^[2]

Common physical examination findings of ganglioglioma include weak irregular pulse, hypertension, wide pulse pressure, unilateral pupillary dilatation, abnormal ophthalmic examination, and focal neurological deficits.^[3]

There are no diagnostic lab findings associated with ganglioglioma.

There are no x-ray findings associated with cerebral ganglioglioma. On x-ray, spinal cord ganglioglioma is characterized by scoliosis, bony remodelling, posterior vertebral body scalloping, and remodelling of the pedicle or posterior arch.^[2]

On head CT scan, ganglioglioma is characterized by iso- or hypodense, calcification, bone remodelling, and enhancement of solid non-calcified component.

On MRI brain, ganglioglioma is characterized by iso- to hypointense solid component on T1, variable contrast enhancement of solid component on T1 C+ (Gd), hyperintense solid component and variable signal in cystic component on T2, and calcification on T2 (GE/SWI).^[1] On MRI scan of spinal cord, ganglioglioma is characterized by mixed signal intensity on T1-weighted images, high intensity on T2, patchy enhancement on T1 C+ (Gd), and calcification with low signal blooming on gradient echo.^[2]

There are no ultrasound findings associated with ganglioglioma.

There are no other imaging findings associated with ganglioglioma.

There are no other diagnostic studies associated with ganglioglioma.

Radiotherapy or chemotherapy is not the first-line treatment option for patients with gangliogliomas. Radiation therapy is usually reserved for patients when either incomplete resection is achievable or tumor recurrence occurs.^[1]

The mainstay of therapy for ganglioglioma is surgery. In the brain, a reasonable resection margin can be achieved with surgery.^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]Mandana Chitsazan, M.D. [3]

Ganglioglioma was first reported by CB Courville in 1930.

Ganglioglioma was first reported by CB Courville in 1930.^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mandana Chitsazan, M.D. [2]

Ganglioglioma may be classified according to the 2016 World Health Organization (WHO) Classification of Tumors of the Central Nervous System into grades I and III.

According to the 2016 edition of the World Health Organization (WHO) classification of Tumors of the Central Nervous System, ganglioglioma is classified into 2 grades:^[1]

• Grade I : lesions with low proliferative potential
• grade III: anaplastic ganglioglioma

In 2016 edition of the WHO classification of CNS tumors, Criteria for grade II ganglioglioma have not been defined.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mandana Chitsazan, M.D. [2]

A ganglioglioma arises from neuronal glial cells, which are cells of the central nervous system. On gross pathology, a ganglioglioma varies from being a partially cystic mass with a mural nodule to a solid mass expanding the overlying gyrus. On microscopic pathology, a ganglioglioma is composed of ganglion cells and neoplastic glial cells with positive staining for synaptophysin, neuronal specific enolase, and GFAP.

Mutation of BRAF V600E gene has been detected in ganglioglioma. The mutant BRAF protein is expressed predominantly in neural cells and to a lesser degree, in the glial component. ^{[1] [2]}

Different pathways may be involved in the molecular pathogenesis of the ganglioglioma. Activation of the PI3K-Akt-mTOR pathway has been noted in ganglioglioma.

Gangliogliomas are composed of two cell populations:^[3]

• Ganglion cells (large mature neuronal elements): ganglio-
• Neoplastic glial elements (astrocytic): -glioma

The glial component determines the biological behaviour of ganglioglioma. Dedifferentiation into high grade tumors may occur, and usually involves the glial component.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

There are no established causes for ganglioglioma.

There are no established causes for ganglioglioma.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Ganglioglioma is the most frequent neuronal-glial CNS neoplasm. It is a rare disease that tends to affect children, adolescents, and young adults. Ganglioglioma affects male and female equally.

Ganglioglioma accounts for around 2% of all primary intracranial tumors, and up to 10% of primary cerebral tumors in children.^[1]

Ganglioglioma commonly affects children, adolescents, and young adults between the age of 10 to 30 years.^[1]

Ganglioglioma affects men and women equally.^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

There are no established risk factors for ganglioglioma.

There are no established risk factors for ganglioglioma.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Screening for ganglioglioma is not recommended.

Screening for ganglioglioma is not recommended.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Ganglioglioma must be differentiated from dysembryoplastic neuroepithelial tumors, pleomorphic xanthoastrocytoma, oligodendroglioma, desmoplastic infantile ganglioglioma, astrocytoma, ependymoma, and transverse myelitis.

Ganglioglioma must be differentiated from:^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Patients with ganglioglioma may progress to develop local recurrence. Complications that can develop as a result of ganglioglioma are metastasis and local recurrence. Prognosis is generally good with treatment.^[1]

Patients with ganglioglioma may progress to develop local recurrence. Symptoms are usually slowly progressive. Due to their non-specific nature, the diagnosis is often delayed.

Common complications of ganglioglioma include:

• Metastasis
• Local recurrence

The prognosis of ganglioglioma is good with treatment, and the 10-year survival rate of children with ganglioglioma is approximately 90%.^[2]

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