Wednesday, March 31, 2010

Types of Brain Tumor

A brain tumor is an abnormal growth that involves the brain itself or its surrounding structures. This distinction helps us to divide nervous system tumors into two general classes:

  1. Intra-axial tumors: These are tumors which grow within the substance of the brain itself
  2. Extra-axial tumors: These are tumors that originate outside of the actual tissues of the brain, but can involve the brain because of their close proximity to it.

In addition, pineal region tumors, originating from the area of the pineal gland, while technically part of the brain are often considered separately. Tumors vary in location as well as other characteristics such as their typical presenting symptoms, their speed of growth and aggressiveness (whether they are benign or malignant) and their typical locations.


Follow the links below to obtain more information about each type of tumor:

Intra-Axial Tumors:

Glioma:
The Most Common Type of Primary Brain Tumor

What Is It?

A glioma is a tumor of the brain that arises from glial cells. Glial cells can be thought of as the "helper" cells in the nervous system. They aid the nerve cells so that they can perform their functions normally. When these glial cells give rise to a tumor, it is called a glioma.

These are the most common general type of tumor that arises from the brain itself. The class includes several sub-types of tumors, including astrocytomas,ependymomas, oligodendrogliomas and glioblastoma multiforme. They can occur just about anywhere in the brain and spinal cord.

Gliomas can vary in their aggressiveness pathologically. They can vary from more benign tumors ("low grade") to more malignant ("high grade"). Generally, benign tumors are more slow growing and less invasive and destructive to normal brain tissue while more malignant tumors grow quickly, invade tissue and are more destructive. All of these tumors are graded on a 4-point scale as either grade I, grade II, grade III or grade IV. Grade I tumors are the most benign and Grade IV tumors are the most malignant. Some types of grade IV tumors are also known as glioblastoma multiforme.


What Types of Symptoms Are Typical?

Symptoms can vary widely depending on the specific type of tumor, its size and location. Neurological symptoms occur depending on where the tumor is. The tumor can be destructive of normal brain tissue so symptoms may arise from the dysfunction of the normal brain in the area of the tumor. This can include just about any neurological symptom but common ones include weakness or numbness, visual changes, memory loss, etc. Again, this varies significantly from patient to patient depending on the specific tumor.

In addition, any mass within the head can lead to increased pressure which can cause headaches, nausea and vomiting, all of which are common symptoms of neurological diseases. Another possible symptom is the new onset of seizures in a person who previously did not have a seizure disorder.


How Is The Diagnosis Typically Made?

After a thorough neurological examination, typically a CT scan or MRI scanwith contrast dye is ordered. These scans can provide a "window into the head", allowing doctors to see tumors or other pathology. This is done both to determine if the patient has a tumor and to determine the characteristics of that tumor (size, shape, location, etc.).

While the appearance of the tumor can help narrow the possibilities, a definitive diagnosis usually requires some tumor tissue to be obtained so that a pathologist can examine it. This is often done with either a biopsy, to remove just a small piece of tumor, or surgery to remove a significant portion of the tumor.

Once tissue is obtained, the pathologist can generally tell exactly what type of tumor it is and grade it in terms of severity. A glioma can be considered more benign or more malignant, depending on the specific pathology. While some are slow growing and less aggressive, some, such as glioblastoma multiforme, are very malignant and can spread rapidly.


What Are Some Common Treatments?

Once tissue is obtained, the pathologist can generally tell exactly what type of tumor it is and grade it in terms of severity. Further treatment, such as further surgery, radiation therapy or chemotherapy all depend on the specific type and severity of the tumor. These treatment decisions can only be made by the patient's physician along with the patient and cannot be generalized.

Generally, these treatment decisions will often be related to how malignant the tumor is and its specific pathology.

Astrocytoma;

What Is It?

An astrocytoma is a type of brain tumor which arises from cells within the brain itself. These cells, called astrocytes, are part of the family of glial cells. Glial cells can be thought of as "helper" cells in the brain. They help keep the internal environment steady so that the nerve cells can function properly. Therefore, these tumors are considered a form of glioma.

Astrocytes occur throughout the brain and spinal cord so these tumors can arise in almost any part of the central nervous system. Generally, there is no obvious cause for the tumor in any given patient, although a small number of these tumors arise in some patients with specific familial neurological conditions such as neurofibromatosis.

They vary considerably from one to the next. In addition to varying in location, the tumors can have various levels of aggressiveness. They vary from more benign ("low grade") to more malignant ("high grade"). Like other gliomas, they are generally graded on a 4-point scale (I, II, III, or IV), with grade I being the most benign and grade IV being the most malignant. Grade IV astrocytomas are also known as glioblastoma multiforme.


What Types of Symptoms Are Typical?

As with most brain tumors, symptoms can vary widely depending on the tumor grade, its size and location. The tumor can cause dysfunction of surrounding or adjacent brain, so neurological symptoms can occur and depend on where the tumor is located. This varies significantly from patient to patient. Symptoms such as weakness or numbness, visual changes, memory loss and others are not uncommon.

Any mass inside the head can increase the pressure and lead to headaches as well as nausea and vomiting, which are all common symptoms of brain tumors. New onset of seizures in a person who does not previously have a history of a seizure disorder is another common way that these tumors present.


How Is The Diagnosis Typically Made?

In addition to a thorough neurological examination, generally an imaging study, such as a CT scan or MRI scan with contrast dye, is ordered to visualize the tumor. This helps to confirm the presence of the tumor, determine its size and location and give some clues as to the type of tumor.

As with most tumors, some tissue of the tumor is required to make the ultimate diagnosis and confirm that it is an astrocytoma. This allows the pathologist to study the tissue and make a definitive diagnosis and to grade the tumor.


What Are Some Common Treatments?

Treatments can vary considerably but are generally guided by the specifics of each tumor, including its grade, its size and its location. Common treatments include surgery to remove part or all of the tumor, radiation therapy and chemotherapy, but these vary widely depending on the specific tumor type and characteristics. These treatment decisions should be made on a case by case basis with the patient's physician and clinical team.

Ependymoma

What Is It?

An ependymoma is a type of brain tumor that arises from cells that line the ventricles of the brain and the central canal of the spinal cord. The ventricles are fluid filled spaces inside the brain. There are four ventricles, two lateral ventricles, the 3rd ventricle and the 4th ventricle. An ependymoma can arise in any of these areas since all of them are lined with ependymal cells (the name for the cells lining the venricles).

These tumors are in the class of brain tumors collectively called Gliomas, because they arise from glial cells. Glial cells can be thought of as "helper" cells in the brain, helping the nerve cells to do their job properly. Generally, no clear cause for these tumors has been found. They can occur in any of the ventricles or in the spinal cord. Occasionally, after originating in one place, they spread to other areas through the cerebrospinal fluid, the liquid that is inside and surrounding the brain and spinal cord. Both adults and children can develop an ependymoma.


What Types of Symptoms Are Typical?

Symptoms vary considerably depending on the location of the tumor and its size. However, because of their common location within the ventricles, as these tumors enlarge they can block the normal flow of the fluid in the ventricles. This leads to a back up of fluid pressure which is termed hydrocephalus. If a patient does have hydrocephalus it can cause headaches, nausea and vomiting. More severe or prolonged pressure increases can lead to problems with vision and even lethargy and coma in severe cases. Other symptoms vary depending on the site of the tumor.


How Is The Diagnosis Typically Made?

Generally, after a general neurological examination, additional testing is required to diagnose ependymoma. Some form of imaging, either CT scanor MRI scan with contrast dye, is used to get a picture inside the head and brain. These studies are generally very effective in detecting a brain tumor. Sometimes a spinal MRI is also needed to look for any tumor in the spinal canal.

However, while the appearance on these scans can suggest an ependymoma, the diagnosis cannot be definitively made without a sample of tumor tissue which the pathologist examines. This can be accomplished through a biopsy or a full surgical removal of the tumor depending on what is indicated in the specific case.

Occasionally, a lumbar puncture will be performed. This test, also known as a spinal tap, includes numbing the skin of the back and inserting a needle into the spinal canal to remove a small amount of fluid. Because these tumors can sometimes spread through this fluid, the sample can be analyzed for tumor cells to help make the diagnosis.


What Are Some Common Treatments?

The treatment of any brain tumor depends on many factors. Generalizations cannot be made. the neurosurgeon treating the patient will typically discuss the treatment options with other physicians and the patient to determine what treatments are best in each case.

However, some of the common options include medications, surgery to biopsy or remove the tumor, radiation treatment and chemotherapy. Which are appropriate depends completely on the specifics of each case. In the case of hydrocephalus, some patients will require a shunt, which is a treatment for hydrocephalus to decrease the pressure of liquid in the head.

Glioblastoma Multiforme (GBM)

What Is It?

Glioblastoma multiforme, also known as malignant glioma or a grade IV glioma, is a tumor that arises within the brain or spinal cord and is derived from glial cells. Glial cells can be though of as "helper" cells in the nervous system. They help keep conditions normal so that the nerve cells can function properly. However, when they give rise to a tumor, these tumors are termed Gliomas. GBM is unfortunately one of the most malignant and aggressive tumors that occur in the human body.

GBM, rather than being a completely different type of glioma, is actually just a severe, aggressive form of other glioma tumors. For example, botholigodendroglioma and astrocytomas, if very malignant, can be considered a glioblastoma multiforme. They can occur throughout the brain and spinal cord. They generally have no known cause. However, while some GBMs develop from previously diagnosed lower-grade tumors, some appear to occur spontaneously.

Gliomas are generally graded depending on their level of aggressiveness. They can vary from more benign tumors ("low grade") to more malignant ("high grade"). Generally, benign tumors are more slow growing and less invasive and destructive to normal brain tissue while more malignant tumors grow quickly, invade tissue and are more destructive. All of these tumors are graded on a 4-point scale as either grade I, grade II, grade III or grade IV. Grade I tumors are the most benign and Grade IV tumors are the most malignant.

Grade IV tumors, the most malignant variety of gliomas, are also known as glioblastoma multiforme. Some astrocytomas or oligodendrogliomas which were previously a lower grade, can become more malignant and eventually become a GBM. Some GBMs appear sporadically and seem to first present as a GBM without going through a lower grade tumor stage first.


What Types of Symptoms Are Typical?

As for any glioma, the presenting symptoms can vary greatly depending on its size and location. While glioblastomas are generally very quickly growing, they can present with very different symptoms depending on where they occur. Just about any neurological symptom is possible including, but not limited to, weakness, numbness, visual changes, memory loss, etc. This varies considerably from patient to patient.

Any mass in the head can cause pressure that can lead to headaches, nausea and vomiting. Another common symptom is seizure.


How Is The Diagnosis Typically Made?

Generally, after a general neurological examination, additional testing is required to diagnose glioblastoma. Some form of imaging, either CT scan orMRI scan with contrast dye, is used to get a picture inside the head and brain. These studies are generally very effective in detecting a brain tumor and defining its size and location.

However, the definitive diagnosis of GBM cannot be made until some tissue is collected to be studied by a pathologist. This tissue is usually either obtained by a biopsy or a larger operation.


What Are Some Common Treatments?

As with all gliomas, the treatment plan varies considerably from patient to patient depending on the specifics of each tumor. In general, because glioblastoma is so aggressive and malignant, aggressive treatment strategies, often using surgery, radiation treatments and/or chemotherapy are employed. However, the treatment plan, of course, is determined by the patient and their personal physician and varies depending on many factors.

Oligodendroglioma

What Is It?

Oligodendroglioma is a brain tumor that arises from a type of glial cell in the brain itself, called an oligodendrocyte. Oligodendrocytes are cells that help to insulate the long processes of nerve cells in the central nervous system. This insulation helps electrical impulses move quickly down the nerve cell. These cells are part of a class of cells known as glial cells, including astrocytes, ependymal cells and microglia. These cells can all be thought of as "helper" cells that help the nerve cells function properly and optimally. For this reason, oligos are considered part of the glioma family of brain tumors.

Like other gliomas, oligodendrogliomas can vary in aggressiveness and are graded from I to IV from more benign to more malignant. Like astrocytomas, over time some oligo's can become more malignant. If these tumors get to grade IV, like astrocytomas, they can be considered glioblastoma multiforme.

The cause of these tumors is unknown. They can occur in many areas of the brain but most commonly in the frontal and temporal lobes. They are very uncommon in the spinal cord. They largely occur in adults with only a small percentage found in children.


What Types of Symptoms Are Typical?

Seizures is a common presenting symptom of oligodendroglioma. However, depending on their location, many different neurological symptoms are possible. As they enlarge, headaches, nausea and vomiting can occur as is common with many types of brain tumors.


How Is The Diagnosis Typically Made?

In addition to a neurological examination, most presenting patients will undergo a CT scan or MRI scan to determine if they have a brain tumor. These tumors can be generally seen on these imaging studies and their size and location can be determined. However, like other tumors, a definitive diagnosis is made only when tumor tissue can be sampled so that a pathologist can determine if it is in fact an oligo and if so, what is its grade.


What Are Some Common Treatments?

Treatment varies considerably but is generally guided by the pathologist. Different tumor types and grades will be approached differently. Some common options include surgery to remove part or all of the tumor, radiation therapy and/or chemotherapy. The decision about which treatments are most suitable to each patient should be discussed with the patient's treating doctor or doctors.

Hemangioblastoma

What Is It?

Hemangioblastoma is a tumor thought to be derived from cells that are precursors to blood vessels and/or blood cells. While they can occur in several places, these brain tumors most frequently occur in the cerebellum, a part of the brain that is important for motor coordination and balance, and the spinal cord. As might be expected from their blood vessel roots, these tumors tend to be very vascular; in other words, they have abundant blood vessels and therefore can have the tendency to bleed.

In addition to their vascular nature, hemangioblastoma can be associated with a tumor cyst, a fluid filled cavity that grows alongside the tumor itself. While most of these tumors are low grade, more benign, they can be somewhat aggressive and can occasionally be more malignant.

Overall, this type of tumor is rare. They can occur sporadically, without any obvious known cause. However, some hemangioblastoma occur in association with a familial, hereditary disorder called von Hippel-Lindau (VHL) disease. VHL is a genetic disorder which is autosomal dominant. In other words, if a person receives just one of the abnormal genes from either parent, they can develop the disease. von Hippel-Lindau leads primarily to several different types of tumors that develop in numerous tissues and organs. Hemangioblastoma is just one of these tumors which occur frequently in these patients.


What Types of Symptoms Are Typical?

While symptoms are variable from patient to patient, because of their typical location in the cerebellum and spinal cord, there are some common presenting symptoms. The symptoms can often be slow to progress but with sudden exacerbations which can be due to bleeding, cyst growth or other injury to surrounding tissues.

Tumors in the cerebellum can lead to difficulty with coordination and gait. If the tumor or cyst involves the 4th ventricle, a fluid-filled space in the area of the cerebellum and brain stem, it can lead to hydrocephalus, which is a blockage of the normal flow of this fluid which leads to increased pressure. It can be associated with headaches, nausea and vomiting. Rarely, the tumor can involve the brain stem itself.

For tumors that grow in the spinal cord, pain, arm and/or leg weakness and numbness can all occur. In severe cases this can lead to complete paralysis.

In patients who have a hemangioblastoma due to von Hippel-Lindau disease, patients may have symptoms due to tumors in other organs as well as polycythemia.


How Is The Diagnosis Typically Made?

Like most brain tumors, after a thorough neurological examination, an imaging study such as an MRI scan is usually performed. These studies can identify the tumor, its size and location. There are certain features of these tumors, such as their location and the presence of large blood vessels or hemorrhage, which make diagnosis by MRI possible. However, like most tumors, definitive diagnosis relies on obtaining tumor tissue for evaluation by a pathologist.


What Are Some Common Treatments?

Patients who present with hydrocephalus will often require a procedure to treat this condition, such as a ventriculoperitoneal shunt. For the tumor itself, surgery is often a primary part of the treatment plan. The goal of surgery is generally to remove the entire tumor. Because these are generally benign tumors, a complete removal is one of the best chances for a cure.

Because of the vascularity of hemangioblastoma, some tumors are embolized prior to surgery. This means that an radiologist injects glue or other materials into the large blood vessels to attempt to cut off blood flow. This can sometimes make surgery easier and safer by preventing some bleeding at the time of subsequent surgery.

Radiation therapy is another option for the tumor, particularly for residual tumor or an unresectable tumor.

The specifics of treatment is highly individualized depending on specific patient and tumor characteristics and therefore cannot be generalized. Each patient must discuss the specific treatment plan with their own physicians.

Medulloblastoma and PNETs

What Is It?

Medulloblastoma is a brain tumor which occurs most frequently in children, accounting for about 30% of all pediatric brain tumors. It is named for its site of origin, in an area called the superior medullary velum, which is associated with the midline of the cerebellum, a large part of the brain that is important for motor coordination and balance. However, the tumor can spread from this origin to involve other parts of the central nervous system. Spread outside of the nervous system is also possible, but rare.

Most cases of medulloblastoma occur sporadically without a known cause. It is by far most common in young children, but can rarely occur in adults as well. However, a small number are associated with hereditary disorders such as Gorlin syndrome, blue rubber-bleb nevus syndrome, Turcot syndrome and Rubinstein-Taybi syndrome.

Medulloblastoma is in a family of related brain tumors called primitive neuroectodermal tumors (PNET). PNETs can occur throughout the brain but this location in the cerebellum is the most common. Other examples of tumors considered to be in this family of brain tumor include pineoblastoma, ependymoblastoma, retinoblastoma, central neuroblastoma, and peripheral neuroblastoma.


What Types of Symptoms Are Typical?

Because of its location in the cerebellum and near the 4th ventricle, a fluid-filled space in the brain, the most common presenting symptoms are cerebellar dysfunction and hydrocephalus.

Cerebellar dysfunction typically causes difficulty with motor coordination, particularly for gait and balance. Less commonly, poor coordination of the arms can occur.

Obstructive hydrocephalus, which results from the tumor blocking the normal flow of cerebrospinal fluid in the ventricles, leads to increased pressure in the ventricles which can lead to other neurological deterioration as well as headache, nausea and vomiting. In very young infants, the hydrocephalus can lead to enlargement of the head.

Later in the course of disease, if tumor spreads to other parts of the nervous system, neurological symptoms can occur, including cranial nerve dysfunction and spinal cord symptoms.


How Is The Diagnosis Typically Made?

After a neurological examination which suggests a tumor, a CT scan or MRI scan of the brain is generally performed. These imaging studies can help identify the tumor, its size and location. While the appearance and location of medulloblastoma typically clues doctors in to this diagnosis, any tumor needs tumor tissue examination to make the definitive diagnosis. Patients will often get imaging of the spine as well to determine if the tumor has spread elsewhere in the nervous system.


What Are Some Common Treatments?

Surgery is often recommended for medulloblastoma. The goal of surgery is to obtain tissue for diagnosis and usually to attempt to remove as much of the tumor as possible. Better long-term survival can occur with complete tumor removal but this is not always possible depending on the size of the tumor and other factors.

Other options which are considered in each case are radiation treatment, although this can be contraindicated in very young children due to the injury it can do to the developing nervous system, and chemotherapy. The specifics of the treatment for each case is variable and should be discussed with the patient with their own physician team.

Patients who present with hydrocephalus will sometimes require a separate procedure, such as a shunt or endoscopic third ventriculostomy, to treat the hydrocephalus. However, this condition will sometimes be cured simply by removal of the tumor itself.

Metastatic Brain Tumor

What Is It?

A metastatic brain tumor is a cancerous tumor which originates in another organ or tissue and then spreads by way of the blood stream to the brain (it metastasizes). Therefore, it is not a primary brain tumor which started growing in the brain. Of all the tumors that can occur in the brain, a metastatic tumor is by far the most common. Primary brain tumors that originate in the brain itself are far less common.

Malignant tumors from anywhere in the body have some propensity to spread outside of their site of origin, either via blood vessels or lymphatic vessels. While many different types of tumor can metastasize to the brain, some of the most common include lung cancer, breast cancer, melanoma (malignant tumor of the skin), kidney cancer and bladder cancer, followed by some sarcomas, testicular and germ cell tumors and others. Some common cancers are very rare to spread to the brain, such as colon cancer or prostate cancer.

Metastatic brain tumors can be solitary (only one visible tumor) or multiple. In severe cases, literally hundreds of small tumors can occur in the brain in a patient with widely metastatic disease.


What Types of Symptoms Are Typical?

When a metastatic brain tumor begins growing in the brain, it can cause injury to the brain in that area, either by destroying cells, causing inflammation or causing pressure on normal brain tissue. In addition to any symptoms associated with the primary tumor in other organs, this nervous system injury and inflammation can lead to neurological symptoms. Initially, when the tumor is small, it may be asymptomatic. If symptoms do occur, these symptoms depend on many factors, including the size and location of the tumor. Some common symptoms include new onset of seizures, headache, nausea and vomiting, personality changes, memory loss, visual changes, speech difficulty, weakness of a part of the body or impaired coordination and balance, to name just a few. Every patient is different.


How Is The Diagnosis Typically Made?

Metastatic brain tumors are sometimes diagnosed in a patient with a known cancer elsewhere upon further work-up but can also be the first sign of disease in a patient who is not aware they have a tumor elsewhere. In the former case, the brain tumor can sometimes be found on a metastatic work-up, meaning that they received a CT scan or MRI scanto actively look for evidence of spread of their primary tumor. These patients may be asymtpomatic. Alternately, these patients may begin having neurological symptoms which prompt a work-up for a metastatic brain tumor.

In the later case, a patient without a known primary tumor may first present with neurological symptoms. When the diagnosis of the metastatic tumor is made, a work-up to find the primary tumor usually follows.

As with any tumor, to make a definitive diagnosis and to distinguish a metastatic tumor from a primary brain tumor, tumor tissue is needed to be evaluated by a pathologist.


What Are Some Common Treatments?

Treatment of metastatic varies dramatically depending on patient and tumor characteristics. Treatment will depend on the type of tumor, how many metastases are present and what the state of the primary tumor elsewhere in the body is. In general, patients in good condition who have only a solitary tumor may be offered surgery to remove the brain tumor. Alternatively, or as an adjunct, radiation therapy and/or chemotherapy may be offered. Again, these treatment decisions depend heavily on the specifics of each case and should be discussed with the specific patient's physicians.

Colloid Cyst

What Is It?

A colloid cyst is a benign brain cyst which typically occurs in the middle of the brain in an area called the 3rd ventricle, one of the fluid filled spaces within the brain. These cysts are not really tumors in the sense that they do not tend to continue to grow in size. They do not spread to other parts of the body. They consist of a thin cyst wall filled with soft material.

It is not known what causes a colloid cyst. They may be congenital malformations which are present from birth.


What Types of Symptoms Are Typical?

Most commonly patients with a colloid cyst are asymptomatic and do not know they have the cyst until it is found incidentally. In other words, they receive an MRI or CT scan for another reason, revealing the cyst.

In many patients, a history of headache may present. Because there are many causes of headache, most benign, it is not always clear if these headaches are caused directly by the colloid cyst.

Because of the location of the cyst, at the front, top of the 3rd ventricle, it is in a position that can obstruct the normal flow of fluid from other fluid filled spaces in the brain, the lateral ventricles. Large cysts sometimes can be associated with obstruction which can lead to increased pressure inside the brain and enlargement of the ventricles, a condition know as hydrocephalus. If a patient presents with hydrocephalus they may complain of headache, and nausea and vomiting. If severe, it can lead to a deterioration in the level of consciousness and eventually coma.

These cysts are sometimes blamed for sudden death. While this appears to be a very rare complication of a colloid cyst, it happens very infrequently, presumably from acute obstruction and acute hydrocephalus.


How Is The Diagnosis Typically Made?

Most commonly, whether a patient presents with symptoms or is asymptomatic, the cyst is demonstrated on an imaging study of the brain, either a CT scan or MRI scan.

While most masses in the brain should be confirmed by pathology before making a definitive diagnosis, to distinguish it from other types of tumors, in the case of colloid cysts the appearance on MRI is generally very typical and there is not much question as to the correct diagnosis. If a patient does undergo surgery, pieces of the cyst will generally be sent to a pathologist to confirm that it is consistent with a colloid brain cyst and that there is no evidence of another type of tumor.


What Are Some Common Treatments?

Some patients will opt for conservative treatment without surgery and will simply follow-up with MRI's to be sure it is not growing or changing. However, some patients, particularly if they are symptomatic, will undergo surgery to remove the cyst. The goal is to drain the cyst and remove as much of the wall of the cyst as possible so that it does not recur. This type of procedure is done in several ways. Traditionally it is done with an open procedure which opens the ventricles to access the cyst. More recently some surgeons have used an endoscope to access the ventricles through only small bur holes in the skull. They then remove the cyst through the endoscope with special instruments for this purpose.

Each patient is different and therefore each should consult their own treating physicians regarding the most appropriate treatment options for their specific case.


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