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meningioma

Meningioma

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What is Meningioma?

 A tumor begins when normal cells change and grow uncontrollably, forming a mass. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

The brain and spinal column make up the central nervous system (CNS), where all vital functions of the body are controlled. When a tumor forms in the CNS, it can be especially problematic and challenging to treat because a person's thought processes and movements can be affected.

This guide focuses on meningioma, which is a slow-growing tumor that usually forms on the surface of the brain and may cause significant symptoms if it grows and presses on the brain or spinal cord. Specifically, meningioma is a type of tumor that occurs in the meninges, which are the thin membranes that surround and protect the brain and spinal cord. There are three meningeal layers called the dura mater, arachnoid, and pia mater. The cerebrospinal fluid (CSF) is made near the center of the brain, in the lateral ventricles, and circulates around the brain and spinal cord between the arachnoid and pia layers.

Approximately 80% of meningiomas are benign. The remaining 20% are either atypical (a type of slow-growing tumor that could become cancerous) or, in 1 - 2% of meningiomas, malignant.

Subtypes of meningioma

Meningioma is classified into subtypes based on the location of the tumor.

Falx and parasagittal meningioma (accounts for 25% of meningiomas). The falx is a membrane sitting in a groove that runs between the two sides of the brain. It contains a large blood vessel. Parasagittal meningioma occurs near the falx.

Convexity meningioma (20%). This type of meningioma occurs on the upper surface of the brain.

Sphenoid meningioma (20%). The sphenoidal ridge is located behind the eyes. Sphenoid meningioma occurs mostly in women.

Olfactory groove meningioma (10%). This type of meningioma occurs along the nerves that connect the brain to the nose.

Posterior fossa meningioma (10%). Posterior fossa meningioma develops at the back of the brain, on the underside.

Suprasellar meningioma (10%). Suprasellar meningioma occurs next to the sella turcica, an area at the base of the skull where the pituitary gland sits.

Spinal meningioma (less than 10%). Most common in women between the ages of 40 and 70, a spinal meningioma usually occurs in the spine at chest level and may push against the spinal cord. Spinal meningioma may cause pain radiating around the chest wall, bladder trouble, and/or weakness or numbness in the legs.

Intraorbital meningioma (less than 10%). This type of meningioma develops in or around the eye sockets.

Intraventricular meningioma (2%). Intraventricular meningioma occurs in the chambers that carry fluid throughout the brain.

Symptoms and Signs

People with meningioma may experience the following symptoms or signs. Sometimes, people with meningioma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a tumor. If you are concerned about a symptom or sign on this list, please talk with your doctor.

Symptoms of meningioma can be general (caused by the pressure of the tumor on the brain or spinal cord), or specific (caused by the tumor stopping the normal functioning of a specific part of the brain or by pressure on nerves or blood vessels). Generally, meningioma is not diagnosed until symptoms begin.

General symptoms include:

1) Seizures. Motor seizures, also called convulsions, are sudden involuntary movements of a person's muscles. People may experience different types of seizures, including myoclonic, tonic-clonic (grand mal), sensory, and complex partial. Certain drugs can help prevent or control them. The differences between these types of seizures can be found below:

2) Myclonic

Single or multiple muscle twitches, jerks, spasms

3) Tonic-Clonic (Grand Mal)

(a) Loss of consciousness and body tone, followed by twitching and relaxing muscle contractions

(b) Loss of control of body functions

(c) May be short period of no breathing (30 seconds) and person may turn a shade of blue

(d) After this type of seizure a person may be sleepy and experience a headache, confusion, weakness, numbness, and sore muscles.

4) Sensory

Change in sensation, vision, smell, and/or hearing without losing consciousness

5) Complex partial

May cause a loss of awareness or a partial or total loss of consciousness

May be associated with repetitive, unintentional movements, such as twitching

6) Headaches, which may be severe and may worsen with activity or in the early morning

7) Personality or memory changes

8) Nausea or vomiting

9) Blurred vision

Symptoms that may be specific to the location of the tumor include:

Falx and parasagittal meningioma

Leg weakness

Convexity meningioma

Seizures

Headaches

Focal neurological deficits, which are problems with nerve function that affect either a specific location, such as the left side of the face, right side of the face, left arm, right arm, left leg, right leg, or a small area, such as the tongue. The tumor can also affect a specific function. For example, speech may be affected, but not the ability to write. It also may cause a loss of movement or sensation.

Personality or memory changes

Sphenoid meningioma

Loss of sensation or numbness in the face

Loss of patches of sight within field of vision, blindness, double vision

Olfactory groove meningioma

Loss of sense of smell

Loss of patches of sight within field of vision, blindness, double vision

Posterior fossa meningioma

Sharp pains in the face, facial numbness, and spasms of the facial muscles

Loss of hearing

Difficulty swallowing

Trouble walking

Suprasellar meningioma

Swelling of the optic disk, which is in the retina of the eye where nerve fibers come together to form part of the optic nerve.

Loss of patches of sight within field of vision, blindness, double vision

Spinal meningioma

Back pain

Pain in the limbs or chest

Intraorbital meningioma

Bulging of the eye

Loss of vision

Intraventricular meningioma

Personality or memory changes

Headaches

Dizziness

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you've been experiencing the symptom(s) and how often.

If a tumor is diagnosed, relieving symptoms and side effects remains an important part of care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

Risk Factors and Prevention

A risk factor is anything that increases a person's chance of developing a tumor. Although risk factors can influence the development of a tumor, most do not directly cause a tumor. Some people with several risk factors never develop tumors, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor can help you make more informed lifestyle and health care choices.

The risk factors for meningioma include:

Age. Adults between 30 and 70 are most likely to be diagnosed with meningioma. Children rarely develop meningioma.

Gender. Women are more than twice as likely as men to develop meningioma; however, men are three times as likely as women to be diagnosed with malignant meningioma.

Radiation exposure. High-dose radiation to the head may increase a person's risk of developing meningioma. Also, low-dose radiation in the treatment of tinea capitis (ringworm of the scalp) may increase a person's risk of developing meningioma decades after treatment. Genetic disorders. People with neurofibromatosis type 2(NF2) have a higher risk of developing meningioma. People with NF2 are also more likely to develop malignant or multiple meningioma (more than one tumor).

Race/Ethnicity.In the United States, black people have higher rates of meningioma than white people. Meningioma is more common in Africa than in North America or Europe.

At this time, there are no known ways to prevent meningioma.

Diagnosis

Doctors use many tests to diagnose a tumor and find out if it is cancerous and, if so, if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether a cancerous tumor has metastasized.

Your doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of tumor suspected

Severity of symptoms

Previous test results

Sometimes, meningioma is found accidently while having a procedure for another reason. More commonly, meningioma is often not diagnosed until a person starts having symptoms. The neuro-oncologist (a doctor who specializes in diagnosing and treating brain tumors and other tumors of the nervous system) can use the patient's symptoms as clues to the location of the tumor. In addition to the patient's detailed medical history and physical examination, the following tests may help the doctor diagnose meningioma and find out where it is located:

Neurological, vision, and hearing tests. These tests help find out how a possible tumor is affecting the brain. An eye examination can find changes to the optic nerve caused by pressure from a meningioma.

Imaging tests

Imaging tests are most useful when the results are combined with the patient's medical history, physical examination, and neurological tests. This combination helps to more accurately find out where the tumor began, and whether or where it has spread. The most common imaging tests used for diagnosing meningioma include:

Computed tomography (CT or CAT) scan. A CT scan takes x-rays of the head from many different angles. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient's vein to provide better detail. A CT scan is best for finding changes in the skull that can be caused by meningioma, such as hardening of the area near the tumor, which can mean that the tumor has been there for a long time.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient's vein to create a clearer picture. MRIs may create more detailed pictures than CT scans and often show changes in the brain caused by the tumor, such as swelling or areas where the tumor has spread. MRI is the preferred method of diagnosing meningioma.

X-ray. An x-ray is a way to create a picture of the structures inside of the body using a small amount of radiation. An x-ray of the head can sometimes help doctors determine the presence and location of meningioma, but is not sufficient to diagnose the type of tumor.

Cerebral angiogram. A cerebral angiogram is a type of x-ray, or series of x-rays, of the head that shows the arteries and veins in the brain. X-rays are taken after a contrast medium is injected into the main arteries of the head. Because a meningioma can block important veins that drain blood from the brain, it is sometimes important to get an angiogram to plan surgery. In addition, there may be abnormal blood vessels that feed the tumor and these can be seen with the angiogram. Sometimes, material is injected into these tumors before surgery to reduce bleeding during surgery.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient's body. This substance is absorbed mainly by organs and tissues that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body. A PET scan is rarely used for meningioma.

Lumbar puncture (spinal tap). A lumbar puncture is a procedure in which a doctor uses a needle to take a sample of cerebrospinal fluid (CSF) to look for tumor cells, blood, or tumor markers (substances found in higher than normal amounts in the blood, urine, or body tissues of people with certain types of tumors). CSF is the fluid that flows around the brain and the spinal cord. Doctors generally give an anesthetic to numb the lower back before the procedure. A lumbar puncture is rarely needed in patients with meningioma.

Electroencephalography (EEG). An EEG is a noninvasive test in which electrodes are attached to the outside of a person's head to measure electrical activity of the brain. Specifically, EEGs are used to detect seizures. Because meningiomas can cause seizures in some patients, EEGs are occasionally needed for patients with this tumor.

Sometimes, meningioma is diagnosed using only the imaging tests above because the location of the tumor may make a biopsy (see below) risky.

Stereotactic neurosurgery/Biopsy. A biopsy can be performed by using a needle guided to the tumor with computers and imaging tests (called a stereotactic technique), or it can be done during surgery when the surgeon can look at the tumor directly. Most meningiomas are removed rather than biopsied so surgery for meningioma is usually done by an open craniotomy (surgery where part of the skull is removed to provide access to the brain) instead of with stereotactic techniques.

Stages and Grades

Staging is a way of describing where the tumor is located, if it is cancerous if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of tumors.

After meningioma has been diagnosed, additional tests will be done to learn more about the tumor. As outlined in the Risk Factorssection, people with neurofibromatosis type 2 are more likely to have multiple meningioma. If this is the case, the patient will have a variety of tests, including an MRI, to determine the how much the tumor has spread within the brain and spine.

There is no formal staging system for meningioma because CNS tumors cannot be staged the same way as other types of tumors. For meningioma, doctors use seven factors to determine the treatment options and prognosis:

Tumor histology/Grading. How a tumor looks under a microscope is called tumor histology. A sample of the tumor is removed during surgery for a biopsy. When surgery is not possible, a biopsy alone is done to get the sample.

Using the histology, the doctor can determine the type of tumor and its grade. Grading describes how closely the tumor cells resemble normal tissue under a microscope. To decide on a treatment, both the type and grade of the tumor must be identified.

Normal tissue is usually differentiated, meaning it is made up of different types of cells grouped together. Tumor tissue that is cancerous is usually made up of cells that look more alike. In general, the more differentiated the tissue and the lower the grade, the better the prognosis.

To determine the histology of a tumor, doctors also look at:

Mitosis (the number of cells dividing)

Hypercellularity (the tumor contains large numbers of cells)

Vascular proliferation (blood vessels in the tumor are growing)

Necrosis (dead tissue in the tumor)

In general, a meningioma is classified into one of three grades:

A grade I tumor does not have mitosis or necrosis.

A grade II tumor is hypercellular and has mitosis and may have a small amount of necrosis, but does not invade the nearby brain. This is usually called “atypical.”

A grade III tumor has necrosis and often has invaded the brain. This is usually called “anaplastic.”

Labeling Index using MIB-1 test. This test identifies the percentage of cells in the tumor that are in the process of dividing, called the S phase of division. The more cells that are in that phase, the more aggressive the tumor is. Generally, a slow-growing meningioma has a MIB-1 of less than 5%.

Age of patient. In adults, the age of the patient when diagnosed is one of the best ways to predict prognosis. In general, the younger the adult, the better the prognosis.

Extent of tumor residual. This is how much of the tumor was left behind after surgery. It includes three classifications:

Gross total. The entire tumor was removed (microscopic cells may remain).

Subtotal. Only part of the tumor was removed.

Biopsy only. Only a small portion, used for a biopsy, was removed.

The prognosis is better when all of the tumor can be surgically removed.

Tumor location. Tumors can form in any part of the CNS. A tumor can cause greater damage to some areas than others, and some tumors are harder to completely remove than others because of where they are located.

Functional neurologic status. The doctor will test how well a patient's CNS is working by using an assessment called the Karnofsky Performance Scale. A higher score indicates a better prognosis.

Metastatic spread. Meningioma rarely metastasizes to other parts of the body. One reason for this is that a meningioma is more self-contained than a tumor that forms elsewhere in the body. Another reason metastasis does not occur often with brain tumors is because the brain does not have a well-formed lymph system to carry tumor cells elsewhere in the body. Cerebrospinal fluid can spread tumor cells, but this is rare with meningioma.

Recurrent: A recurrent tumor is one that comes back after treatment. If there is a recurrence, the tumor may need to be graded again using the system above.

Biogenetic markers

The factors listed above are the best way to determine the prognosis for a person with meningioma. Researchers are currently looking for tumor markers (substances in the blood that are found in higher levels in a person with a tumor) that could make meningioma easier to diagnose and allow the staging of adult CNS tumors. These tools may someday make it possible for doctors to determine how quickly a brain tumor will grow and spread, develop more effective treatments, and more accurately predict prognosis.

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