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
Historical Perspective
Classification
Pathophysiology
Ganglioglioma arises from neuronal glial cells, which are cells of 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.
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 of the neuronal-glial CNS neoplasms. Ganglioglioma 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
The complications of brain tumors are brain herniation, loss of brain function and recurrence. The prognosis in primary brain tumors depends on the type of tumor, age, functional status of the patient, the extent of surgical tumor removal, spread of metastasis and biogenetic markers.[1]. Patients with benign gliomas may survive for many years[2][3] while survival in most cases of glioblastoma multiforme is limited to a few months after diagnosis. The 5-year survival rate is 33.3%.
Diagnosis
Staging and Grading
The subtypes of brain tumors have different staging systems based on the grade, extent of spread, size and presence of distant metastases. The grades of brain tumors are subdivided into Grades 1,2,3 and 4. The lower the grade, better the prognosis.
History and Symptoms
The most common symptoms of brain tumors are headache, seizures, visual changes and changes in personality, mood and concentration.
Physical examination
Common physical examination findings of brain tumors include altered sense of consciousness, anisocoria, papilledema and focal neurological deficits.
CT
Imaging plays a central role in the diagnosis of brain tumors. On head CT scan, brain cancer is characterized by hypodense mass lesions.
MRI
Imaging plays a central role in the diagnosis of brain tumors. On MRI brain, brain tumor is characterized by hypointense or isointense on T1-weighted scans, or hyperintense on T2-weighted MRI.
Other imaging findings
The other imaging modalities that can be used for brain tumors are single-photon emission computed tomography, positron emission tomography and whole bone scan.
Other Diagnostic Studies
Electrophysiological exams, such as electroencephalography play a marginal role in the diagnosis of brain tumors. The definitive diagnosis of brain tumor can only be confirmed by histological examination of tumor tissue samples obtained either by means of brain biopsy or open surgery. The histologic examination is essential for determining the appropriate treatment and the correct prognosis.
Treatment
Medical Therapy
The predominant therapy for brain tumor is surgical resection. Adjunctive chemotherapy and radiation may be required.
Surgery
Meningiomas, with the exception of some tumors located at the skull base, can be successfully removed surgically. In more difficult cases, stereotactic radiosurgery, such as Gamma Knife radiosurgery, remains a viable option. Most pituitary adenomas can be removed surgically using a minimally invasive approach through the nasal cavity and skull base (trans-nasal, trans-sphenoidal approach). Larger pituitary adenomas require a craniotomy (opening of the skull) for their removal. Radiotherapy, including stereotactic approaches, is reserved for inoperable cases.
References
- ↑ Nicolato A, Gerosa MA, Fina P, Iuzzolino P, Giorgiutti F, Bricolo A. Prognostic factors in low-grade supratentorial astrocytomas: a uni-multivariate statistical analysis in 76 surgically treated adult patients. Surg Neurol 1995;44:208-21; discussion 221-3. PMID 8545771.
- ↑ Janny P, Cure H, Mohr M, Heldt N, Kwiatkowski F, Lemaire JJ, Plagne R, Rozan R. Low grade supratentorial astrocytomas. Management and prognostic factors. Cancer 1994;73:1937-45. PMID 8137221.
- ↑ Piepmeier J, Christopher S, Spencer D, Byrne T, Kim J, Knisel JP, Lacy J, Tsukerman L, Makuch R. Variations in the natural history and survival of patients with supratentorial low-grade astrocytomas. Neurosurgery 1996;38:872-8; discussion 878-9. PMID 8727811.