Brain tumor classification
Brain tumor Microchapters |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Brain tumors can be classified into two main categories: primary and secondary tumors. Primary tumors originate in astrocytes, oligodendrocytes and ependymal cells. Secondary tumors originate in malignant cancers located primarily in other organs.
Classification
Primary tumors
Tumors occurring in the brain include: astrocytoma, pilocytic astrocytoma, dysembryoplastic neuroepithelial tumor, oligodendrogliomas, ependymoma, glioblastoma multiforme, mixed gliomas, oligoastrocytomas, medulloblastoma, retinoblastoma, neuroblastoma, germinoma and teratoma.
Most primary brain tumors originate from glia (gliomas) such as astrocytes (astrocytomas), oligodendrocytes (oligodendrogliomas), or ependymal cells (ependymoma). There are also mixed forms, with both an astrocytic and an oligodendroglial cell component. These are called mixed gliomas or oligoastrocytomas. Plus, mixed glio-neuronal tumors (tumors displaying a neuronal, as well as a glial component, e.g. gangliogliomas, disembryoplastic neuroepithelial tumors) and tumors originating from neuronal cells (e.g. gangliocytoma, central gangliocytoma) can also be encountered.
Other varieties of primary brain tumors include: primitive neuroectodermal tumors (PNET, e.g. medulloblastoma]], medulloepithelioma, neuroblastoma, retinoblastoma, ependymoblastoma), tumors of the pineal parenchyma (e.g. pineocytoma, pineoblastoma), ependymal cell tumors, choroid plexus tumors, neuroepithelial tumors of uncertain origin (e.g. gliomatosis cerebri, astroblastoma), etc.
Secondary tumors and non-tumor lesions
Secondary or metastatic brain tumors originate from malignant tumors (cancers) located primarily in other organs. Their incidence is higher than that of primary brain tumors. The most frequent types of metastatic brain tumors originate in the lung, skin (malignant melanoma), kidney (hypernephroma), breast (breast carcinoma), and colon (colon carcinoma). These tumor cells reach the brain via the blood-stream.
Some non-tumoral masses and lesions can mimic tumors of the central nervous system. These include tuberculosis of the brain, cerebral abscess (commonly in toxoplasmosis), and hamartomas (for example, in tuberous sclerosis and von Recklinghausen neurofibromatosis).
Symptoms of brain tumors may depend on two factors: tumor size (volume) and tumor location. The time point of symptom onset in the course of disease correlates in many cases with the nature of the tumor ("benign", i.e. slow-growing/late symptom onset, or malignant (fast growing/early symptom onset).
Many low-grade (benign) tumors can remain asymptomatic (symptom-free) for years and they may accidentally be discovered by imaging exams for unrelated reasons (such as a minor trauma).
New onset of epilepsy[1] is a frequent reason for seeking medical attention in brain tumor cases.
Large tumors or tumors with extensive perifocal swelling edema inevitably lead to elevated intracranial pressure (intracranial hypertension), which translates clinically into headaches, vomiting (sometimes without nausea), altered state of consciousness (somnolence, coma), dilatation of the pupil on the side of the lesion (anisocoria), papilledema (prominent optic disc at the funduscopic examination). However, even small tumors obstructing the passage of cerebrospinal fluid (CSF) may cause early signs of increased intracranial pressure. Increased intracranial pressure may result in herniation (i.e. displacement) of certain parts of the brain, such as the cerebellar tonsils or the temporal uncus, resulting in lethal brainstem compression. In young children, elevated intracranial pressure may cause an increase in the diameter of the skull and bulging of the fontanelles.
Depending on the tumor location and the damage it may have caused to surrounding brain structures, either through compression or infiltration, any type of focal neurologic symptoms may occur, such as cognitive and behavioral impairment, personality changes, hemiparesis, (hemi) hypesthesia, aphasia, ataxia, visual field impairment, facial paralysis, double vision, tremor etc. These symptoms are not specific for brain tumors - they may be caused by a large variety of neurologic conditions (e.g. stroke, traumatic brain injury). What counts, however, is the location of the lesion and the functional systems (e.g. motor, sensory, visual, etc.) it affects.
A bilateral temporal visual field defect (bitemporal hemianopia—due to compression of the optic chiasm), often associated with endocrine disfunction—either hypopituitarism or hyperproduction of pituitary hormones and hyperprolactinemia is suggestive of a pituitary tumor.
SPECIFIC TUMOR TYPES
Brain tumors are classified depending on:
- Location of the tumor
- Type of tissue involved
- Whether they are noncancerous (benign) or cancerous (malignant)
- Other factors
Sometimes, tumors that start out less aggressive can change their biologic behavior and become more aggressive.
Tumors can occur at any age, but many types are most common in a certain age group. In adults, gliomas and meningiomas are the most common.
Gliomas come from glial cells such as astrocytes, oligodendrocytes, and ependymal cells. Gliomas are divided into three types:
- Astrocytic tumors include astrocytomas (can be noncancerous), anaplastic astrocytomas, and glioblastomas.
- Oligodendroglial tumors. Some primary brain tumors are made up of both astrocytic and oligodendrocytic tumors. These are called mixed gliomas.
- Glioblastomas are the most aggressive type of primary brain tumor.
Meningiomas and schwannomas are two other types of brain tumors. These tumors:
- Occur most often between ages 40 and 70.
- Are usually noncancerous, but can still cause serious complications and death from their size or location. Some are cancerous and aggressive.
Other primary brain tumors in adults are rare. These include:
- Ependymomas
- Craniopharyngiomas
- Pituitary tumors
- Primary (central nervous system - CNS) lymphoma
- Pineal gland tumors
- Primary germ cell tumors of the brain
References
- ↑ Lopez MBS, Laws ER Jr. Neurosurgical Focus 12(2), Article 1, 2002.