Oligodendroglioma overview: Difference between revisions
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* There is quite a wide range of treatments to meet the individual needs of each patient which includes standard therapies, precision medicine and clinical trials | * There is quite a wide range of treatments to meet the individual needs of each patient which includes standard therapies, precision medicine and clinical trials | ||
* Some of them are listed below: | * Some of them are listed below: | ||
**'''Brachytherapy:''' | **'''Brachytherapy:''' | ||
*** destroys tumors by implanting radioactive medicine directly to or near the treatment site | *** destroys tumors by implanting radioactive medicine directly to or near the treatment site |
Revision as of 14:40, 10 May 2019
Oligodendroglioma Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]Sujit Routray, M.D. [3]
Overview
Oligodendrogliomas are a type of glioma that are believed to originate from the tripotential glial precursor cells and not from the bipotential oligodendrocytes. The term "oligodendroglioma" was first coined by Bailey and Cushing in 1926 following the observation that the tumor cells are morphologically similar to oligodendrocytes. Oligodendroglioma may be classified according to the WHO classification of the central nervous system tumors into five subtypes: oligodendroglioma (OII), anaplastic oligodendroglioma (OIII), oligoastrocytoma (OAII), anaplastic oligoastrocytoma (OAIII), and glioblastoma with oligodendroglioma component (GBMo). Genes associated with the pathogenesis of oligodendroglioma include t(1;19)(q10;p10), NJDS, IDH1, IDH2, CIC, FUBP1, p53, Leu-7, TCF-12, MGMT, TP73, EGFR, and PTEN. On gross pathology, oligodendroglioma is characterized by a well-circumscribed, gelatinous, calcified, gray mass which may expand a gyrus and remodel the skull. On microscopic histopathological analysis, oligodendroglioma is characterized by diffuse growth pattern of highly cellular lesion with rounded nucleus with atypia and perinuclear halo resembling fried eggs, distinct cell borders, clear cytoplasm, and abundant calcification. Common causes of oligodendroglioma include genetic mutations. Oligodendroglioma must be differentiated from pilocytic astrocytoma, central neurocytoma, ependymoma, dysembryoplastic neuroepithelial tumor, and meningioma. Oligodendroglioma is a disease that tends to affect the middle-aged adult population. Oligodendroglioma most commonly occurs in the 4th and 5th decade of life. Males are more commonly affected with oligodendroglioma than females. The male to female ratio is approximately 2 to 1. Oligodendroglioma usually affects individuals of the Caucasian race. African American, Latin American, and Asian individuals are less likely to develop oligodendroglioma. The most potent risk factor in the development of oligodendroglioma is family history of brain tumors. If left untreated, patients with oligodendroglioma may progress to develop seizures, focal neurological deficits, hydrocephalus, brain herniation, intracranial hemorrhage, and ultimately death. When evaluating a patient for oligodendroglioma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough past medical history review. Other specific areas of focus when obtaining the history include review of common risk factors such as family history of brain tumors. Symptoms associated with oligodendroglioma include seizure, headache, nausea, vomiting, vertigo, visual loss, diplopia, strabismus, muscle weakness, numbness, speech difficulties, mood disturbances, and personality changes. Common physical examination findings of oligodendroglioma include nystagmus, papilledema, esotropia, visual field loss, altered mental status, aphasia, ataxia, hemiparesis, tremors, and focal neurological deficits. Findings on CT scan suggestive of oligodendroglioma are round or oval, marginated, hypo- to isodense mass with hemorrhage, calcification, and ill-defined enhancement following intravenous contrast administration. On brain MRI, oligodendroglioma is characterized by a mass which is typically hypointense on T1-weighted images and hyperintense on T2-weighted images. Calcification is observed on T2* decay component of MRI. Other imaging studies for oligodendroglioma include MR spectroscopy (dominant N-acetyl aspartate peak, increased choline levels and decreased NAA levels with a myo-inositol peak), MR perfusion (increased "chicken wire" network of vascularity, which results in elevated relative cerebral blood volume), PET scan (to differentiate between oligodendroglioma from anaplastic oligodendroglioma and tumor recurrence from tumor necrosis), and bone scan (bone metastasis). The predominant therapy for oligodendroglioma is surgical resection. Adjunctive chemotherapy and radiation are required. Supportive therapy for oligodendroglioma includes anticonvulsants and corticosteroids.
Historical Perspective
The term "oligodendroglioma" was first coined by Bailey and Cushing in 1926 following the observation that the tumor cells are morphologically similar to oligodendrocytes. Oligodendroglioma was first described and published by W. E. Carnegie Dickson in 1926. In 2009, NJDS mutation was first identified in the pathogenesis of oligodendroglioma by Kevin Smith. Irradiation of pituitary adenoma was also discovered to be associated with oligodendroglioma by Kevin Smith et al.
Classification
Oligodendroglioma may be classified according to the WHO classification of the central nervous system tumors into five subtypes: oligodendroglioma (OII), anaplastic oligodendroglioma (OIII), oligoastrocytoma (OAII), anaplastic oligoastrocytoma (OAIII), and glioblastoma with oligodendroglioma component (GBMo).
Pathophysiology
Oligodendroglioma arises from the tripotential glial precursor cells and not from the bipotential oligodendrocytes. Genes associated with the pathogenesis of oligodendroglioma include t(1;19)(q10;p10), NJDS, IDH1, IDH2, CIC, FUBP1, p53, Leu-7, TCF-12, MGMT, TP73, EGFR, and PTEN. On gross pathology, oligodendroglioma is characterized by a well-circumscribed, gelatinous, calcified, gray mass which may expand a gyrus and remodel the skull. On microscopic histopathological analysis, oligodendroglioma is characterized by diffuse growth pattern of highly cellular lesion with rounded nucleus with atypia and perinuclear halo resembling fried eggs, distinct cell borders, clear cytoplasm, and abundant calcification. Oligodendroglioma is demonstrated by positivity to tumor markers such as MAP2, GFAP, S-100, EMA, IDH1-R132H, ATRX, Ki-67, NSE , Synaptophysin, OLIG1, and OLIG2.
Causes
Common causes of oligodendroglioma include genetic mutations. Common genetic mutations involved in the development of oligodendroglioma can be found here.
Differentiating Oligodendroglioma from other diseases
Oligodendroglioma must be differentiated from pilocytic astrocytoma, central neurocytoma, ependymoma, dysembryoplastic neuroepithelial tumor, and meningioma.
Epidemiology and Demographics
Oligodendroglioma, although rare, is the third most common glioma. The incidence of oligodendroglioma and anaplastic oligodendroglioma is estimated to be 0.32 and 0.17 cases per 100,000 individuals in the United States, respectively. Oligodendroglioma is a disease that tends to affect the middle-aged adult population. Oligodendroglioma most commonly occurs in the 4th and 5th decade of life. Males are more commonly affected with oligodendroglioma than females. The male to female ratio is approximately 2 to 1. Oligodendroglioma usually affects individuals of the Caucasian race. African American, Latin American, and Asian individuals are less likely to develop oligodendroglioma.
Risk factors
The most potent risk factor in the development of oligodendroglioma is family history of brain tumors.
Screening
There is insufficient evidence to recommend routine screening for oligodendroglioma.
Natural History, Complications and Prognosis
If left untreated, patients with oligodendroglioma may progress to develop seizures, focal neurological deficits, hydrocephalus, brain herniation, intracranial hemorrhage, and ultimately death. Common complications associated with oligodendroglioma include hydrocephalus, intracranial hemorrhage, coma, bone marrow metastasis, recurrence, venous thromboembolism, parkinsonism, and side effects of chemotherapy and radiotherapy. Depending on the extent and grade of the tumor at the time of diagnosis, the prognosis of oligodendroglioma may vary. However, the prognosis is generally regarded as good. The median survival time for oligodendroglioma is 11.6 years for grade II and 3.5 years for grade III.
Diagnosis
- CT or MRI scan is necessary to characterize the anatomy of oligodendroglial tumors such as:
- Tumor size
- Tumor location
- Heter/homogeneity
- However, the confirmation of final diagnosis is dependent on histopathologic examination of the biopsy specimen
Staging
There is no established system for the staging of oligodendroglioma.
History and Symptoms
When evaluating a patient for oligodendroglioma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough past medical history review. Other specific areas of focus when obtaining the history include review of common risk factors such as family history of brain tumors. Symptoms associated with oligodendroglioma include seizure, headache, nausea, vomiting, vertigo, visual loss, diplopia, strabismus, muscle weakness, numbness, speech difficulties, mood disturbances, and personality changes.
Physical examination
Common physical examination findings of oligodendroglioma include nystagmus, papilledema, esotropia, visual field loss, altered mental status, and focal neurological deficits.
Laboratory Findings
Some patients with oligodendroglioma may have elevated protein and cell count with normal glucose and lactate on CSF analysis, which is usually suggestive of hydrocephalus.
Chest X Ray
Chest x-ray may be performed to detect metastases of anaplastic oligodendroglioma to the lungs.
CT
Head CT scan may be helpful in the diagnosis of oligodendroglioma. Findings on CT scan suggestive of oligodendroglioma are round or oval, marginated, hypo- to isodense mass with hemorrhage, calcification, and ill-defined enhancement following intravenous contrast administration.
MRI
Brain MRI is helpful in the diagnosis of oligodendroglioma. On brain MRI, oligodendroglioma is characterized by a mass which is typically hypointense on T1-weighted images and hyperintense on T2-weighted images. Calcification is observed on T2* decay component of MRI.
Ultrasound
There are no ultrasound findings associated with oligodendroglioma.
Other Imaging Findings
Other imaging studies for oligodendroglioma include MR spectroscopy (dominant N-acetyl aspartate peak, increased choline levels and decreased NAA levels with a myo-inositol peak), MR perfusion (increased "chicken wire" network of vascularity, which results in elevated relative cerebral blood volume), PET scan (to differentiate between oligodendroglioma from anaplastic oligodendroglioma and tumor recurrence from tumor necrosis), and bone scan (bone metastasis).
Other Diagnostic Studies
Other diagnostic studies for oligodendroglioma include biopsy (homogeneous, compact, rounded cells with distinct borders and clear cytoplasm surrounding a dense central nucleus and perinuclear halo) and fluorescent in-situ hybridization (FISH) technique (deletions of chromosome 1p and 19q).
Treatment
Innovative treatment options:
- Brain tumors can be complex and require a combination of treatments for the best outcome
- There is quite a wide range of treatments to meet the individual needs of each patient which includes standard therapies, precision medicine and clinical trials
- Some of them are listed below:
- Brachytherapy:
- destroys tumors by implanting radioactive medicine directly to or near the treatment site
- Brachytherapy:
- Chemotherapy:
- reaches cancer that may have spread, even microscopically, throughout the body
- Chemotherapy:
- Craniotomy:
- surgical procedure that removes a bone flap, a section of the skull, to access the brain
- Craniotomy:
- Intensity-modulated radiation therapy:
- uses computer technology to deliver radiation treatment that precisely fits the size and shape of the tumor
- Intensity-modulated radiation therapy:
- Minimally invasive cranial base surgery:
- uses smaller incisions and specially designed instruments to eliminate a tumor while saving surrounding tissue from damage
- Minimally invasive cranial base surgery:
- Stereotactic radiosurgery & radiotherapy:
- a noninvasive procedure that applies large doses of radiation directly to a tumor
- Stereotactic radiosurgery & radiotherapy:
Medical Therapy
The predominant therapy for oligodendroglioma is surgical resection. Adjunctive chemotherapy and radiation are required. Supportive therapy for oligodendroglioma includes anticonvulsants and corticosteroids.
Surgery
Surgery is the first-line treatment option for patients with oligodendroglioma. CSF shunting is usually reserved for patients with hydrocephalus.
Primary Prevention
There is no established method for prevention of oligodendroglioma.
Secondary Prevention
There are no secondary preventive measures available for oligodendroglioma.