Oligodendroglioma overview

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Oligodendroglioma from other Diseases

Epidemiology & Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Diagnosis

Staging

History & Symptoms

Physical Examination

Laboratory Findings

Chest X Ray

CT

MRI

Ultrasound

Other Imaging Findings

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Overview

Oligodendrogliomas are a type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell. They occur primarily in adults (9.4% of all primary brain and central nervous system tumors) and are only rarely found in children (4% of all primary brain tumors). The median age of diagnosis for oligodendroglioma is 41 years of age.

Overview

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.[1] Oligodendroglioma was first described and published by W. E. Carnegie Dickson in 1926.[2] 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.[3]

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).[4]

Pathophysiology

Oligodendroglioma arises from the tripotential glial precursor cells and not from the bipotential oligodendrocytes.[5] 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.[6][7][8][9][10][11][12][13][14] On gross pathology, oligodendroglioma is characterized by a well-circumscribed, gelatinous, calcified, gray mass which may expand a gyrus and remodel the skull.[15] 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.[14][16][17][18] 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.[19][20][14]

Causes

Common causes of oligodendroglioma include genetic mutations. The genes associated with the etiology of oligodendroglioma include t[1;19][q10;p10], NJDS, IDH1, IDH2, CIC, FUBP1, p53, Leu-7, TCF-12, MGMT, TP73, EGFR, and PTEN.[6][7][8][9][10][11][12][13][14]

Differentiating Oligodendroglioma from other diseases

Oligodendroglioma must be differentiated from pilocytic astrocytoma, central neurocytoma, ependymoma, dysembryoplastic neuroepithelial tumor, meningioma, cerebral metastasis, brain abscess, tuberculoma, sarcoidosis, neurocysticercosis, multiple myeloma, primary CNS lymphoma, cerebral toxoplasmosis, germ cell tumor, ganglioglioma, pleomorphic xanthoastrocytoma, herpes simplex encephalitis, stroke, cerebral arteriovenous malformation, and epilepsy.[21][22][23][24]

Epidemiology and Demographics

Oligodendroglioma, although rare, is the third most common glioma.[25] 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.[26] Oligodendroglioma is a disease that tends to affect the middle-aged adult population.[25] 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.[27] Oligodendroglioma usually affects individuals of the Caucasian race. African American, Latin American, and Asian individuals are less likely to develop oligodendroglioma.[28]

Risk factors

The most potent risk factor in the development of oligodendroglioma is family history of brain tumors.[26]

Screening

There is insufficient evidence to recommend routine screening for oligodendroglioma.[29]

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.[30] Common complications associated with oligodendroglioma include hydrocephalus, intracranial hemorrhage, coma, bone marrow metastasis, recurrence, venous thromboembolism, parkinsonism, and side effects of chemotherapy and radiotherapy.[27][31][32][33][34][35][36] 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.[37]

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.[26] Symptoms associated with oligodendroglioma include seizure, headache, nausea, vomiting, vertigo, changes in speech, mood, and personality, visual loss, diplopia, strabismus, muscle weakness, and numbness.[24][38][39][40]

Physical examination

Common physical examination findings of oligodendroglioma include nystagmus, papilledema, esotropia, visual field loss, altered mental status, aphasia, ataxia, hemiparesis, tremors, and focal neurological deficits.[40][24][18][41][36]

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.[42]

Chest X Ray

Chest x-ray may be performed to detect metastases of anaplastic oligodendroglioma to the lungs.[43]

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 hemmorhage, calcification, and ill-defined enhancement following intravenous contrast administration.[44][42]

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.[45][42]

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).[18][46][47][48][49][50][51][52][53]

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).[54][18][17]

Medical Therapy

The predominant therapy for oligodendroglioma is surgical resection. Adjunctive chemotherapy and radiation are required.[55][56][57] Supportive therapy for oligodendroglioma includes anticonvulsants and corticosteroids.[55]

Surgery

Surgery is the first-line treatment option for patients with oligodendroglioma.[55] CSF shunting is usually reserved for patients with hydrocephalus.[42]

Primary Prevention

There is no established method for prevention of oligodendroglioma.

Secondary Prevention

There are no secondary preventive measures available for oligodendroglioma.

References

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