Ganglioglioma overview
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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]
Overview
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]
Historical Perspective
Ganglioglioma was first reported by CB Courville in 1930.[4]
Classification
Pathophysiology
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.
Causes
There are no established causes for ganglioglioma.
Differentiating brain tumors from other diseases
Ganglioglioma must be differentiated from dysembryoplastic neuroepithelial tumors, pleomorphic xanthoastrocytoma, oligodendroglioma, desmoplastic infantile ganglioglioma, astrocytoma, ependymoma, and transverse myelitis.
Epidemiology and Demographics
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.
Risk factors
There are no established risk factors for ganglioglioma.
Natural History, Complications and Prognosis
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]
Diagnosis
Staging
There is no established system for the staging of ganglioglioma.
History and Symptoms
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]
Physical examination
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]
Laboratory Findings
There are no diagnostic lab findings associated with ganglioglioma.
X Ray
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]
CT
On head CT scan, ganglioglioma is characterized by iso- or hypodense, calcification, bone remodelling, and enhancement of solid non-calcified component.
MRI
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]
Ultrasound
There are no ultrasound findings associated with ganglioglioma.
Other imaging findings
There are no other imaging findings associated with ganglioglioma.
Other Diagnostic Studies
There are no other diagnostic studies associated with ganglioglioma.
Treatment
Medical Therapy
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]
Surgery
The mainstay of therapy for ganglioglioma is surgery. In the brain, a reasonable resection margin can be achieved with surgery.[1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 1.9 Features of ganglioglioma. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioglioma
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Presentation of spinal cord ganglioglioma. Dr Ayush Goel and Dr Sara Wein et al. Radiopaedia 2015. http://radiopaedia.org/articles/spinal-ganglioglioma
- ↑ 3.0 3.1 Presentation of increased ICP. Patient.info. http://patient.info/doctor/raised-intracranial-pressure
- ↑ Courville, CB (1930). "Ganglioglioma, tumor of the central nervous system: review of the literature and report of two cases". Arch Neurol Psychiatry. 24: 439–91. doi:10.1001/archneurpsyc.1930.022201500020.
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