tumortypes

Brain tumors are classified into tumor types. This describes the location of the tumor and it also speaks to whether the found tumor is benign or malignant.

Benign or Malignant?

greybarA recently released report from the Centers for Disease Control and Prevention now lists brain cancer as the deadliest childhood cancer.

The brain is an incredible organ. Housed in a relatively small space – the skull – it has more folds and gyrations than intestines doing yoga. That is why brain tumors are so difficult to “hunt down”, because it must be determined whether the tumor is a primary tumor or a metastasis (spread) from a tumor in other parts of the body.

Benign tumors  have no cellular physical appearances of cancer and are a growth with no “appendages” to other sources of blood vessels which the tumor could use to spread.

Malignant tumors are full-on aggressive and “take over” by tapping into the immune and blood systems of the patient’s body.

Both types of tumors in certain areas of the brain can render much damage. This is why the doctors do CAT scans, PET scans, MRIs and MRAs.

An MRI (Magnetic Resonance Imaging) uses radio waves, a magnetic field, and a computer to create a scan of your body parts to look for health issues.

Another testing procedure now being used is an MRA (Magnetic Resonance Angiography/Angiogram). This type of test looks specifically at the body’s blood vessels – including blood vessels in the brain and spinal cord. Unlike a traditional angiogram, which requires inserting a catheter into the body, an MRA is a less invasive and less painful procedure. Your doctor may suggest an MRA in order to rule out an aneurysm or weakness in the wall of an artery, or to look at the areas of your brain and/or spine. During an MRA, the patient lies flat inside the scanner, much like the large, tunnel-like tube of an MRI that takes pictures of your body. In some cases, a dye – known as contrast – may be added to the patient’s bloodstream to make blood vessels easier to see. When needed, the contrast is administered intravenously.

In this section we have outlined the tumors for your benefit. Like any other cancer, they have levels and there are differences. We hope this listing helps you understand. Either way, you are in a battle against brain tumors – and we are with you all the way.

Benign

LOW-GRADE ASTROCYTOMAS

Description
The Astrocytoma is derived from a normal supporting cell in the brain called the astrocyte. In a patient with one of these tumors, the cells in the Astrocytoma tumor are no longer normal in appearance. The degree of this abnormality is used to determine the tumor’s grade, and the tumor’s grade determines the prognosis of the tumor. Astrocytomas are graded from 1 to 4, with Grade 1 being the slowest growing and Grade 4 being the most rapidly growing and malignant lesions.

Benign or low-grade Astrocytomas  (Grade 2 Astrocytoma)  are uncommon tumors, accounting for about 10 percent to 15 percent of Gliomas. These tumors also have a more favorable prognosis compared to Anaplastic Astrocytomas and Glioblastomas.

Symptoms
Symptoms may include seizures, weakness, speech problems, headaches, personality changes or visual loss.

Treatment
The prognosis in Glial tumors depends heavily on the degree of malignancy – benign in low-grade Astrocytomas.

It also depends on:

  • The age of the patient (young patients do better than old patients).
  • The location of the tumor. If the tumor is surgically accessible, a complete removal is attempted. A near-complete resection (less than 10 cc of residual tumor) of low-grade Astrocytomas prevents tumor recurrence for longer periods of time. But for large tumors, tumors deep in the brain, and/or tumors near speech and motor areas, a near-complete resection can be challenging.
  • The neurological condition of the patient. Patients who are neurologically normal do better than those with a deficit (e.g. paralysis of one side).
  • The response of that particular tumor to therapy, which can include at least one of the following: Surgery, Radiation and Chemotherapy.
BRAIN STEM GLIOMA

Description
Since parts of the brain stem control vital life functions that could be damaged by surgery, the role of surgery in certain Brain Stem tumors is questionable, frequently not recommended and should only be attempted by surgeons experienced in Brain Stem tumors. Some Brain Stem tumors, notably Pilocytic Astrocytomas, can be removed with proper technique if the tumor is in a place that allows surgical access. Surgery is rarely considered in patients with infiltrating lesions anywhere in the brainstem; attempting to remove the tumor will also remove important but infiltrated brain tissue that is responsible for important life functions. A devastating neurological deficit or death usually results. However, a stereotactic biopsy to obtain a tissue diagnosis in these cases is possible, and with the correct equipment in the hands of a surgeon experienced in these procedures, they are relatively safe.

Brain Stem Gliomas can range from very low-grade and potentially curable-grade to high-grade (malignant) tumors. The prognosis of Brain Stem Gliomas is related to tumor type and grade. And tumor type is frequently associated with tumor location within the brain stem. Invasive Fibrillary Astrocytomas and Mixed Gliomas have a preference for the pons as opposed to the other two parts of the brainstem. Because in these tumors, tumor cells co-exist with healthy brain tissue, they cannot be removed. Low-grade Pilocytic Astrocytomas tend to favor the midbrain and medulla. These slow growing tumors form a solid mass of tissue and can also form a cyst that displaces rather than invades brain tissue. These are potentially curable by surgery. Glioblastomas are rare in the brainstem but can occur anywhere.

Symptoms
Symptoms most commonly associated with Brain Stem Gliomas are: headaches, nausea, speech or balance abnormalities, difficulty swallowing, weakness or numbness of the arms and/or legs, facial weakness, and double vision.

With low-grade tumors, these symptoms may develop slowly and may go unnoticed for months, but a sudden onset of symptoms tends to occur with rapidly growing, high-grade tumors.

Treatment
Since parts of the brain stem control vital life functions that could be damaged by surgery, the role of surgery in certain Brain Stem tumors is questionable, frequently not recommended and should only be attempted by surgeons experienced in Brain Stem tumors. Some Brain Stem tumors, notably Pilocytic Astrocytomas, can be removed with proper technique if the tumor is in a place that allows surgical access.

Surgery is rarely considered in patients with infiltrating lesions anywhere in the brainstem; attempting to remove the tumor will also remove important but infiltrated brain tissue that is responsible for important life functions. A devastating neurological deficit or death usually results. However, a stereotactic biopsy to obtain a tissue diagnosis in these cases is possible, and with the correct equipment in the hands of a surgeon experienced in these procedures, they are relatively safe.

Most of these tumors will be treated by radiation and chemotherapy since they can reduce symptoms in infiltrating Brain Stem Gliomas and slow the tumor’s growth.  Slow growing, low-grade Brain Stem Gliomas can have long periods of remission following treatment. High-grade brain stem tumors usually have a grim prognosis in spite of radiation and chemotherapy.

CAROTID STENOSIS

Description
Carotid Stenosis is a narrowing of the carotid artery (usually the internal carotid artery) that can cause transient ischemic attacks (TIAs) with stroke-like symptoms due to blockage of a blood vessel by an atherosclerotic lesion or due to reduced blood flow.

Symptoms
Symptoms may include paralysis, weakness or numbness of one or both extremities on one side of the body; loss of vision in one eye or in part of one eye; loss of vision off to one side; sensory defect; or language disturbances ranging from mild to complete loss of speech.

Treatment
The least invasive treatment is medical management: aspirin or other anti-platelet medications, anticoagulation, blood pressure and cholesterol-fighting medications.

Following that is a surgical procedure, Carotid Endarterectomy. Surgically opening the artery reduces the rate of stroke over the next 18 months by 17 percent and the risk of death by 7 percent. Risks of this procedure increase considering age, cardiovascular and pulmonary disease, chronic TIAs and recent strokes.

CHORDOMA

Description
Chordomas are rare, low-grade tumors that occur at the sacrum, near the lower tip of the spine, or at the base of the skull. They are benign, slow-growing tumors that originate from cells left over from early fetal development. Chordomas at the end of the spine tend to be extradural (on the outside of the spinal cord), but those at the base of the skull can invade the bone, compressing parts of the brain in that region. A large Chordoma may even push into the brain stem or grow into the sinuses. The Skull Base Chordoma can block ventricles, causing hydrocephalus and can also metastasize (spread) or recur.

Symptoms
The most common symptoms of Chordomas are double vision and headaches. Chordomas of the lower spine (sacrum) may show a mass at the base of the spine and not produce symptoms until they compress the lower spinal nerves and cause urinary or rectal dysfunction. Less common symptoms include visual loss, hearing loss, difficulty swallowing, hoarse voice, facial numbness, incoordination, motor weakness and memory disturbance.

Treatment
Sacral Chordomas can be cured if found early and totally excised with a wide margin of healthy tissue. Care must be taken during a needle biopsy on the sacral mass, as this is all it takes to spread tumor cells around and render the lesion incurable.

With minimally invasive endoscopic procedures that approach the skull base through the nasal cavities, an apparent complete removal of skull-base tumors is possible. Radiation is standard treatment after surgery.

EPENDYMOMA

Description
Ependymomas are primary tumors of the central nervous system that are comprised of ependymal cells.  These are the cells that line the fluid-filled cavities of the brain: the two lateral ventricles, the third ventricle, the aqueduct, the fourth ventricle – as well as the central canal of the spinal cord.

Ependymomas that arise in the wall of the ventricles or aqueduct can eventually obstruct the flow of ventricular fluid.  This raises the pressure inside the patient’s head, causing hydrocephalus.  Both the trapped part of the ventricular system and untreated hydrocephalus can result in headaches, paralysis and eventually coma and death.

These tumors can also spill cells into the spinal fluid, a means of metastasis as the cells travel with the spinal fluid and deposit themselves anywhere the cerebrospinal fluid (CSF) goes.  They can then form another tumor mass – in another part of the ventricular system – at the base of the brain or within the spine.

Low-grade Ependymomas usually grow as a solid mass in part of the ventricle and usually do not seed the spinal fluid with tumor cells.  Furthermore, Low-grade Ependymomas are contained within the ventricular system, don’t invade the brain and can be cured by surgery.

High-grade Ependymomas tend to drop cells into the ventricular and spinal fluid, which then form distant metastatic tumors.  They can invade the surrounding brain tissue by mobile cells to form solid tumor masses within the substance of the brain.  These can be highly malignant and are not curable by surgery, radiation or chemotherapy, but treatment can reduce or eliminate symptoms and prolong life expectancy.

Symptoms
Depending on the location, the Ependymoma can cause a loss of part of the visual field, difficulty speaking, memory problems, weakness in the opposite side of the body and, of course, headaches due to elevated intracranial pressure. Hydrocephalus (an obstructed ventricular system), can cause headaches, double vision, drowsiness, difficulty walking – and eventually coma and death.

Treatment
Treatment will depend on its location and whether or not it produces blockage of ventricular fluid or hydrocephalus.

Surgery for malignant Ependymomas is usually followed by radiation and various types of chemotherapy.

MEDULLOBLASTOMA

Description
Medulloblastomas arise from undeveloped brain cells and are often located in the cerebellum or near the brain stem.  They can spread to the spinal cord through the cerebrospinal fluid (CSF).  Medulloblastomas may obstruct the fourth ventricle, causing hydrocephalus.

Symptoms
The most common symptoms are headaches, early morning vomiting, lethargy or sleepiness, lack of coordination, double vision and behavioral or personality changes. Signs of pressure can be seen behind the eye when examined with an ophthalmoscope.

Treatment
Surgery is the standard treatment when possible.  Chemotherapy is usually part of the treatment plan.  Radiation of the brain and spine is often recommended in adults and children over three years of age. A shunt may be needed to treat hydrocephalus.  This tumor may recur years later if not totally resected.

A newer, more promising treatment involves proton therapy.  With a medullobastoma, the cerebral spinal fluid pathway, the whole brain and the whole spine must be treated to a certain dose, followed by one further “shot” to the primary area.

The advantage to treating the whole spine with proton therapy is the elimination of low doses of radiation that in conventional treatment impacts on organs beyond the tissues in front of the spine – the heart, lungs, and in girls sometimes, the ovaries.  Proton therapy also decreases the risk of thyroid dysfunction or thyroid cancer.

MENINGIOMA

Description
Meningiomas are tumors that arise from the dura.  The dura mater is the membrane covering the entire interior of the skull.  It also creates compartments within the skull: the falx, which separates the right side of the brain from the left side and the tentorium, which forms the shelf separating the cerebrum from the cerebellum and brain stem.
Meningiomas are usually named for the part of the dura they come from.  Hence, a Meningioma starting in the falx is called a Falx Meningioma, a Meningioma in the tentorium is called a Tentorial Meningioma, a Meningioma in the cavernous sinus is called a Cavernous Sinus Meningioma and so on.  Convexity Meningiomas are tumors located over the outer surface of the brain.   Some Meningiomas are named for the bone that the tumorous dura covers.

Symptoms
Most Meningiomas are benign and slow growing and can grow for years without producing any symptoms. When they do produce symptoms, it’s due to irritation of compressed brain tissue (seizures), damage to brain tissue (neurological deficit such as paralysis) or elevation of intracranial pressure from the mass of the tumor itself.

Olfactory Groove Meningiomas grow from beneath both frontal lobes and can become very large before a diagnosis is made.  The first actual symptoms relate to a loss of the sense of smell, which usually goes unnoticed or blamed on allergies.

Malignant Meningiomas
Some Meningiomas are more aggressive than others and tend to recur faster. Atypical Meningiomas are classified Grade II and radiation therapy is recommended for these tumors following surgery.  Anaplastic Meningiomas have cells that exhibit a loss of structural differentiation, are abnormal in appearance and may show evidence of increased capacity for cell division, often a marker of malignancy.  Malignant Meningiomas (Grade III) and Papillary Meningiomas are malignant and will invade adjacent brain tissue.

Treatment
Radiation therapy is necessary following surgery.

OLIGODENDROGLIOMA

Description
Oligodendrogliomas comprise a class of Glial Tumors in which the oligodendroglial cell is the predominant cell type.  Normally, oligodendroglial cells form myelin, the fatty substance that surrounds the axons of nerve cells, providing insulation so that nerve cell electrical transmission is faster and more efficient.

Each of these cell types has a percentage of cells capable of mitosis (the process by which one cell divides to become two cells).  In Oligodendrogliomas, the mitotic rate of oligodendroglial cells exceeds the rate of the other cells.  Eventually, the oligodendroglial cells are the most numerous or “predominant” cell type, resulting in an Oligodendroglioma.

Oligodendrogliomas are often classified as: low-grade, anaplastic (cells exhibit a loss of structural differentiation, are abnormal in appearance and may show evidence of increased capacity for cell multiplication) or malignant.  Of course, low-grade tumors can become anaplastic and anaplastic tumors can become malignant.  Because the speed of this transformation varies widely from patient to patient, there is no consistent way of predicting the behavior of any Oligodendroglioma – particularly in adult patients.

Symptoms
Seizures occur in 50 to 80 percent of patients.  Other symptoms include: headache, nausea and vomiting, ataxia (difficulty with balance), dizziness and altered mental state.

There are three ways to judge the growth of Oligodendrogliomas:

  • Progressive enlargement on CT or MRI
  • Development of new symptoms
  • Development of new contrast enhancement.

Oligodendrogliomas, like most Glial tumors, have two growth patterns: solid tumor tissue and infiltrating tumor cells.

Treatment
It is difficult to surgically remove a solid tumor because it would require excising “healthy” tissue with “diseased” tissue.  Radiation therapy and chemotherapy can be effective.

OPTIC NERVE GLIOMA

Description
Named for its location on or near the nerve pathways between the eyes and the brain, an Optic Nerve Glioma can range from low-grade to high-grade.  This tumor is a type of Juvenile Pilocytic Astrocytoma (JPA) occurring most often in infants and children, though they can occur in adults as well.

Symptoms
Symptoms of an Optic Nerve Glioma include: headaches, progressive loss of vision and double vision.

Treatment
Surgery is standard treatment, usually followed by radiation therapy or chemotherapy.  Chemotherapy may be given to very young children instead of radiation therapy to avoid damage to the developing brain.

PINEAL TUMORS

Description
There are a number of different cells in the pineal gland that can grow into a tumor. The pinealocyte, the cell with the greatest number in the gland, can transform into a Pinealocytoma (Pinealoma) or to the malignant Pinealoblastoma. Primitive cells within the pineal gland can give rise to Germinomas or Teratomas. Teratomas are usually benign but can turn into Teratocarcinomas that can be highly malignant. Finally, the pineal gland has glial cells, and these can develop into Astrocytomas, Oligodendrogliomas, or Mixed Gliomas. Pilocytic Astrocytomas can develop in this area as well.

Symptoms
The most common symptoms are diplopia (loss of ability to look upward), double vision and nystagmus  (flickering of the eyes back and forth).  Other symptoms are related to hydrocephalus and increased intracranial pressure, such as: headaches, nausea, vomiting, lethargy, lack of coordination and altered mental function.

Treatment
Pineal tumors are in contact with the spinal fluid and therefore can seed the CSF (cerebrospinal fluid), giving the tumor cells access to the spine and other parts of the brain.  Because of the possibility of metastasis, these lesions must be treated with chemotherapy or cranio-spinal irradiation therapy following surgery.

PINEOBLASTOMA, PINEOCYTOMA AND GERMINOMA

Description
Pineoblastoma, Pineocytoma and Germinoma are all different types of tumors that can grow in the area of the pineal gland.

Germinomas are almost always found in male patients.

Symptoms
The most common symptoms are diplopia (loss of ability to look upward), double vision and nystagmus (flickering of the eyes back and forth).  Other symptoms are related to hydrocephalus and increased intracranial pressure, such as: headaches, nausea, vomiting, lethargy, lack of coordination and altered mental function.

Treatment
Pineal Tumors are in contact with the spinal fluid and therefore can seed the CSF (cerebrospinal fluid), giving the tumor cells access to the spine and other parts of the brain.  Because of the possibility of metastasis, these lesions must be treated with chemotherapy or cranio-spinal irradiation therapy following surgery.

PITUITARY TUMORS

Description
The pituitary gland produces hormones that affect growth and the functions of other glands in the body.  Tumors arising from the pituitary gland itself are called Adenomas, which are benign and slow growing.

Most Pituitary tumors grow in the front two-thirds of the gland, at the adenohypophysis, and are classified as secreting or non-secreting tumors.  The majority of Pituitary tumors are secreting tumors, producing excessive amounts of hormones and further classified by the type of hormone secreted.

Pituitary tumors can range from low-grade to high-grade. Pituitary Carcinoma is the rare malignant form of Pituitary Adenoma, diagnosed by metastases in or out of the nervous system.

Symptoms
The symptoms most commonly associated with Pituitary tumors include: headache, depression, vision loss, nausea or vomiting, behavioral and cognitive changes, cessation of menstrual periods (amenorrhea), leaking of fluid from the breasts (galactorrhea), hair growth in women, impotence in men, abnormal growth of hands and feet and abnormal weight gain.

There may be an over-secretion of hormones or impaired hormone production. Hormones most commonly affected include growth hormone, prolactin (controls lactation), sex hormones (menstrual cycle and other sex functions), thyroid gland hormones, adrenal gland hormones and vasopressin (water and electrolyte balance).

Treatment
If the tumor is large or compressing the optic nerve, the standard treatment is surgery.  This can be trans-sphenoidal surgery, when the surgeon gains access to the tumor by entering through the nasal passage.  Radiation therapy may also be used.  Some Pituitary tumors may be treated with medication, and observed with MRI scans.  Certain drugs can block the pituitary gland from overproducing hormones.  Follow-up with an endocrinologist may be necessary to manage hormonal changes.

For Pituitary Carcinoma, the range of symptoms is similar to the benign tumor, and treatment may include surgery, radiation, chemotherapy and hormone therapy.

ACUTE SUBDURAL HEMATOMA

Description
An Acute Subdural Hematoma (SDH) is a clot of blood that develops between the surface of the brain and the dura mater (the brain’s tough outer covering), usually due to stretching and tearing of veins on the brain’s surface.  These veins rupture when a head injury suddenly jolts or shakes the brain.

Traumatic acute SDHs are among the most lethal of all head injuries.  Associated with more severe generalized brain injury, they often occur with cerebral contusions.

Symptoms
Symptoms of an SDH include: confused speech, difficulty with balance or walking, headache, lethargy or confusion, loss of consciousness, nausea and vomiting, numbness, seizures, slurred speech, visual disturbances and weakness. The mortality rate for patients with an acute SDH ranges from 50 percent to 90 percent.  A significant percentage of these deaths result from the underlying brain injury and pressure on the brain that develops in the days after injury.

Approximately 20 percent to 30 percent of patients will recover full or partial brain function.  Post-operative seizures are relatively common in these patients.

Treatment
Immediate surgical treatment is crucial for Acute Subdural Hematomas larger than 1 cm at the thickest point.  For smaller hematomas, surgery may not be required.

A large craniotomy (surgery through an opening created in the skull) is often necessary to remove the thick blood clot and to reach bleeding sites. Cerebral contusions underlying an SDH are often removed during surgery.

ACOUSTIC NEUROMA (Vestibular Schwannoma)

Description
An Acoustic Neuroma is a tumor found in the area of the brain where the auditory nerve enters the bony opening of the skull between the brain and the inner ear.  This tumor grows in the vestibular nerve, which is involved in balance, hence the name Vestibular Schwannoma.

These tumors are typically benign.  Since treatment of Acoustic Neuromas is rarely urgent, patients are advised to obtain second opinions prior to treatment.

Symptoms
Primary symptoms are one-sided hearing loss, ringing in the ears and problems with balance.

Facial numbness, facial weakness, and taste changes occur as the tumor causes pressure on the fifth and seventh cranial nerves.  Difficulty swallowing and hoarseness may occur with larger tumors as they will place pressure on the lower cranial nerves.

Approximately 20 percent to 30 percent of patients will recover full or partial brain function.  Post-operative seizures are relatively common in these patients.

Treatment
If complete removal is possible, surgery is performed.  In large tumors, even if all of the tumor cannot be excised, surgery is typically required to help reduce swelling and pressure on the brain.  Radiation therapy is also an option.  For Acoustic Neuromas that are small and slow growing, no treatment may be necessary as long as the patient is monitored over time.

AVM (Arteriovenous Malformation)

Description
AVMs appear as a “tangle” of vessels caused by an abnormal collection of blood vessels where arterial blood in the brain flows directly into draining veins without any normal capillaries between. Typically, only one quarter of AVMs hemorrhage by age 15 while most become symptomatic by age 50.

AVMs are graded according to three important characteristics: size, location and pattern of venous drainage. Larger AVMs and those involved in more complex intricate areas of the brain, receive higher grades. Of course, higher grades mean greater difficulty in treatment and greater associated risks.

Symptoms
Symptoms of AVMs include hemorrhage, seizures, headaches, pressure on surrounding brain tissue, and bruits (murmurs) in a vessel caused by flowing blood that are audible to the patient.

Treatment
Surgery is the most common treatment, though radiosurgery may be advised for small AVMs, particularly those in critical locations. (With radiosurgery, the risk of hemorrhage does not decrease for two years so cutting into significant parts of the brain is avoided).

There are some AVMs that can be treated by minimally invasive endovascular embolization to block blood flow through the vessels.

BALLISM

Description
Ballism causes involuntary, violent movements of the body, involving the muscles where the limbs attach to the body. A stroke or tumor is the most common cause.

Symptoms
With Ballism, the patient suffers abnormal movements such as involuntary hurling and irregular violent movements of the shoulder and arm.

Treatment
The first choice of treatment is medication to help regulate brain chemicals and processes that control movement and emotions. If Ballism persists for more than two or three months, surgery is considered. Of course, an MRI to determine if a brain tumor is the cause is highly recommended.

EPILEPSY

Description
Epilepsy is a general term covering a variety of conditions in which seizures occur. Small brain tumors, other brain lesions or malformations of blood vessels can cause certain epilepsies.

Some causes of seizures include brain damage due to trauma, tumors or other brain lesions, abnormal development of the brain in early childhood, and a family history of epilepsy.

Symptoms
Symptoms can range from small “absence seizures” – the person is unresponsive for seconds or minutes – to more generalized seizures that could cause the person to fall, convulse and display repetitive behavior.

Treatment
For Epilepsy caused by small tumors, lesions or malformations of blood vessels, an MRI will pinpoint the problem area.  Surgical removal of the diseased tissue along with minimal amounts of surrounding tissue has an eighty percent efficacy rate of helping the patient live seizure-free.

CAVERNOUS ANGIOMA

Description
Cavernous Angioma involves abnormality of blood vessels along with large, adjacent capillaries. Blood flow through the vessels is slow.

Symptoms
Symptoms include seizures, headache, hemorrhage or mass effect (the compression of surrounding brain tissue).  Mass effect brings on additional symptoms: weakness, numbness, double vision, visual disturbance or language difficulties.

Treatment
Surgical removal of the vessel abnormality is the best option.

DERMOID TUMOR

Description
Normally benign, these slow-growing tumors can occur in various structures of the skull, spine and brain when normal developmental cells are trapped within the growing brain. Dermoid Tumors contain skin-like elements, including hair and sweat glands.

Symptoms
Like Epidermoid Tumors, symptoms of Dermoid Tumors include: seizures, fever, headaches, neck stiffness, facial pain, numbness, weakness and visual difficulties. Additional symptoms are back pain, hormonal changes and signs and symptoms of meningitis.

Treatment
Surgery is the optimal treatment. Steroid medication may be included for meningitis-type symptoms.

GANGLIOGLIOMA

Description
Gangliogliomas are rare brain tumors that cause seizures.

Symptoms
Seizures can be present for months or years before the diagnosis is made.

Treatment
Surgery for the complete removal of the tumor is optimal. But for cases where the tumor is located in critical areas of the brain, incomplete surgical removal is done. More than 95 percent of Gangliogliomas are low-grade and usually do not recur after removal.

NEUROFIBROMA

Description
Neurofibroma is a tumor of the nerve fiber and the most benign of nerve fiber tumors. Neurofibromas more frequently occur as part of Neurofibromatosis – a genetic disorder that may be associated with multiple Neurofibromas.  The disease is transmitted by an autosomal dominant mutant gene.

Symptoms
The spectrum of lesions varies widely, involving: small skin nerves appearing as small bumps, or involving large nerves, producing elephantiasis (enlargement of affected body parts).

Treatment
Surgery may be required to determine diagnosis, pain, progressive neurological complications, compression of adjacent tissues and suspicion of a malignant tumor.

SCHWANNOMAS

Description
Schwann cells are important for nerve regeneration.  Schwannomas are benign tumors of the nerve sheath.  These tumors grow slowly, occurring most often as single tumors but sometimes also as multiple lesions.  They can grow in any nerve containing Schwann cells, including cranial nerves.

Schwannomas seem to target the eighth cranial nerve.  Individuals with bilateral Schwannomas of the eighth cranial nerve most likely suffer from a genetic condition known as Type 2 Neurofibromatosis.

Symptoms
The most common symptoms are issues with nerve function and/or pain due to pressure on the surrounding nerve.

Spinal Canal Schwannomas can lead to compression of the spinal cord causing weakness, numbness, stiffness, paralysis, and trouble controlling urine or bowels.

Additionally, compression of the nerve root can cause pain shooting down the arms or legs, weakness or numbness.

Treatment
Surgery is the best treatment option even for Schwannomas that are asymptomatic, since the tumor will undoubtedly grow.  Recurrences following total removal are rare.

SPASTICITY

Description
Overactive reflexes, simultaneous activation of opposing muscle groups and resistance to passive movement of body and limbs are typical of Spasticity. Brain lesions in parts of the brain involved in motor functions are responsible for these abnormal muscle behaviors.

Symptoms
The painful symptoms of Spasticity include: scissoring of legs or flexing of thighs beyond normal range of motion. Individuals suffering from Spasticity have a very hard time sitting, driving or sleeping.

Treatment
Medications that are used for sedation, anti-seizure or muscle relaxants are the first treatment option. Surgery is considered when the patient fails to respond to medical treatment or when the side effects of these medications are unbearable.

SPINAL ARTERIOVENOUS MALFORMATION (AVM)

Description
Like Arteriovenous Malformations (AVMs) Spinal AVMs are abnormal collections of blood vessels – but in the spinal canal – connected between the arteries and veins but without intervening capillaries.

Spinal Dural AVMs are the most common type in adults.  Intradural AVMs are located outside the substance of the spinal cord.  Intramedullary AVMs are located within the substance of the spinal cord.

Symptoms
Most Spinal AVMs involve neurological symptoms that increase over months or years.  Back pain and progressive sensory loss and lower extremity weakness are typical.

Other symptoms include: sudden onset of weakness, numbness, difficulty urinating, urinary incontinence, fecal incontinence, or paralysis as a result of hemorrhage.

Treatment
Some Spinal AVMs can be treated with endovascular embolization of the AVM. But those that hemorrhage typically require surgery.

 

 

Malignant

LOW-GRADE ASTROCYTOMAS

Description
The Astrocytoma is derived from a normal supporting cell in the brain called the astrocyte. In a patient with one of these tumors, the cells in the Astrocytoma tumor are no longer normal in appearance.  The degree of this abnormality is used to determine the tumor’s grade, and the tumor’s grade determines the prognosis of the tumor.  Astrocytomas are graded from I to IV, with Grade I being the slowest growing and Grade IV being the most rapidly growing and malignant lesions.

Benign or low-grade Astrocytomas (Grade II Astrocytoma) are uncommon tumors, accounting for about 10 percent to 15 percent of Gliomas.  These tumors also have a more favorable prognosis compared to Anaplastic Astrocytomas and Glioblastomas.

Symptoms
Symptoms may include seizure, weakness or speech problems, headaches, personality changes or visual loss.

Treatment
The prognosis in Glial tumors depends heavily on the degree of malignancy (benign in low-grade Astrocytomas).

It also depends on:

  • The age of the patient (young patients do better than old patients).
  • The location of the tumor. If the tumor is surgically accessible,
  • Complete removal is attempted. A near-complete resection (less than 10 cc of residual tumor) of low-grade Astrocytomas prevents tumor recurrence for longer periods of time. But for large tumors, tumors deep in the brain, and/or tumors near speech and motor areas, a near-complete resection can be challenging.
  • The neurological condition of the patient. Patients who are neurologically normal do better than those with a deficit (e.g. paralysis of one side).
  • The response of that particular tumor to therapy, which can include at least one of the following: Surgery, Radiation and Chemotherapy.
BRAIN STEM GLIOMA

Description
The brain stem, located at the base of the brain, is a structure made up of cranial nerve nuclei and long nerve pathways that connect the spinal cord to the cerebrum and cerebellum.  The cranial nerve nuclei are involved in functions necessary for survival – breathing, digestion, heart rate and blood pressure, swallowing as well as hearing, facial sensation, facial movement and eye movements.  The central core of the upper brain stem (midbrain) is responsible for arousal and consciousness.

The brain stem has three distinct parts. From top to bottom, they are: midbrain (that connects to the cerebrum) the pons (that connects to the cerebellum) and medulla (that connects to the spinal cord).  These divisions are important when talking about Brain Stem Gliomas since tumors in each of the three parts will have very different symptoms and prognoses since certain Glioma tumor types seem to have a preference for one of the three parts of the brain stem.

Brain Stem Gliomas can range from very low-grade and potentially curable-grade to high-grade (malignant) tumors.  The prognosis of Brain Stem Gliomas is related to tumor type and grade and tumor type is frequently associated with tumor location within the brain stem.  Invasive Fibrillary Astrocytomas and Mixed Gliomas have a preference for the pons as opposed to the other two parts of the brain stem.  Because in these tumors, tumor cells co-exist with healthy brain tissue, they cannot be removed.

Low-grade Pilocytic Astrocytomas tend to favor the midbrain and medulla.  These slow growing tumors form a solid mass of tissue and can also form a cyst that displaces rather than invades brain tissue. These are potentially curable by surgery.  Glioblastomas are rare in the brain stem but can occur anywhere.

Symptoms
Symptoms most commonly associated with Brain Stem Gliomas are: headaches, nausea, speech or balance abnormalities, difficulty swallowing, weakness or numbness of the arms and/or legs, facial weakness, and double vision.

With low-grade tumors, these symptoms may develop slowly and may go unnoticed for months, but a sudden onset of symptoms tends to occur with rapidly growing, high-grade tumors.

Treatment
Since parts of the brain stem control vital life functions that could be damaged by surgery, the role of surgery in certain Brain Stem tumors is questionable, frequently not recommended and should only be attempted by surgeons experienced in Brain Stem tumors. Some Brain Stem tumors, notably Pilocytic Astrocytomas, can be removed with proper technique if the tumor is in a place that allows surgical access.

Surgery is rarely considered in patients with infiltrating lesions anywhere in the brainstem; attempting to remove the tumor will also remove important but infiltrated brain tissue that is responsible for important life functions. A devastating neurological deficit or death usually results. However, a stereotactic biopsy to obtain a tissue diagnosis in these cases is possible, and with the correct equipment in the hands of a surgeon experienced in these procedures, they are relatively safe. Most of these tumors will be treated by radiation and chemotherapy since they can reduce symptoms in infiltrating Brain Stem Gliomas and slow the tumor’s growth.

Slow growing, low-grade Brain Stem Gliomas can have long periods of remission following treatment. High-grade brain stem tumors usually have a grim prognosis in spite of radiation and chemotherapy.

CNS LYMPHOMA

Description
CNS Lymphoma is a cancer that develops from lymphocytes; cells in the lymphatic system, the tissues and organs that produce and store cells that fight infection (such as bone marrow, spleen, thymus and lymph nodes) and the network of vessels (similar to blood vessels) that carry a clear, watery fluid called lymph throughout the body.  Lymphomas can start in the brain (primary CNS Lymphoma) or begin in organ systems outside of the brain and spread (metastasize) to the brain (Secondary CNS Lymphoma).  Or they can start in the brain.  CNS Lymphoma can be primary (originating in the brain) or secondary (metastatic) and is more common in people whose immune systems have been compromised.

To diagnose Primary Central Nervous System (CNS) Lymphoma, a CT scan of many parts of the body must be done.  The absence of tumors elsewhere will help to confirm that the CNS is the primary source of the cancer.

Symptoms
Primary CNS Lymphoma is a very aggressive cancer, and usually causes multiple tumors throughout the central nervous system.

Mental status changes occur in one third of patients.  Primary symptoms include: weakness or numbness, partial seizures and cranial nerve palsies, visual changes, double vision, facial numbness, facial weakness, hearing loss and/or swallowing difficulties.  Increased intracranial pressure (headache, nausea, vomiting) and/or generalized seizures are common as well.

Treatment
Treatment for CNS Lymphoma involves radiation therapy, chemotherapy, and steroids.  Surgery is rarely an option because of the multitude of lesions. With this cancer, the main role of surgery is tumor biopsy.  Though steroids work on these tumors, the beneficial effects tend to fall off after six months of treatment.

In terms of other treatments: radiation therapy brings about complete response in 80% of patients but recurrence is almost definite.  With chemotherapy, systemic therapy is still underway.

EPENDYMOMAS

Description
Ependymomas are primary tumors of the central nervous system that are comprised of ependymal cells. These are the cells that line the fluid-filled cavities of the brain: the two lateral ventricles, the third ventricle, the aqueduct, the fourth ventricle – as well as the central canal of the spinal cord.

Ependymomas that arise in the wall of the ventricles or aqueduct can eventually obstruct the flow of ventricular fluid.  This raises the pressure inside the patient’s head, causing hydrocephalus.  Both the trapped part of the ventricular system and untreated hydrocephalus can result in headaches, paralysis and eventually coma and death.

These tumors can also spill cells into the spinal fluid, a means of metastasis as the cells travel with the spinal fluid and deposit themselves anywhere the CSF goes.  They can then form another tumor mass – in another part of the ventricular system – at the base of the brain or within the spine.

Low-grade Ependymomas usually grow as a solid mass in part of the ventricle and usually do not seed the spinal fluid with tumor cells.  Furthermore, Low-grade Ependymomas are contained within the ventricular system, don’t invade the brain and can be cured by surgery.

High-grade Ependymomas tend to drop cells into the ventricular and spinal fluid, which then form distant metastatic tumors.  They can invade the surrounding brain tissue by mobile cells to form solid tumor masses within the substance of the brain.  These can be highly malignant and are not curable by surgery, radiation or chemotherapy, but treatment can reduce or eliminate symptoms and prolong life expectancy.

Symptoms
Depending on the location, the Ependymoma can cause a loss of part of the visual field, difficulty speaking, memory problems, weakness in the opposite side of the body and, of course, headaches due to elevated intracranial pressure. Hydrocephalus (an obstructed ventricular system), can cause headaches, double vision, drowsiness, difficulty walking – and eventually coma and death.

Treatment
Treatment will depend on its location and whether or not it produces blockage of ventricular fluid or hydrocephalus. Surgery for malignant Ependymomas is usually followed by radiation and various types of chemotherapy.

GLIOBLASTOMA MULTIFORME

Description
Glioblastoma Multiforme (frequently referred to as Glioblastoma, GBM, or Grade 4 Astrocytoma), are the most malignant variety of these tumors. They are made up of cells that infiltrate brain tissue within a region – in some cases, multiple regions – of solid tumor tissue within the zone of infiltrated brain tissue. Mitoses, the process by which cells divide, is frequently noted by the pathologist as the surgical specimen is examined. In addition, regions of necrosis (dead tissue) are also noted where the tumor has grown so fast that parts of it has outpaced its blood supply. These tumors induce the formation of new but abnormal blood vessels. Identification of these blood vessels is also important in establishing the diagnosis.

Glioblastomas are still known to have the worst prognosis of all: With standard treatment, the average survival rate for adults with an anaplastic astrocytoma (low-grade glioma) is two to three years. For more aggressive glioblastomas (Stage IV), treated with temozolamide together with radiation therapy, the average survival rate is 14 to 15 months, with a 30% survival chance of two-years.

There is a 2009 study that reported that almost 10% of patients with Glioblastoma may live five years or longer. This may be related to a recent finding that there are Glioblastoma patients who have had their MGMT gene shut off by a process called methylation. The MGMT gene is thought to be a significant predictor of response.

Another consideration is that not all Glioblastomas have the same biologic abnormalities, which may explain the different responses patients had to the same treatment.

Symptoms
Glioblastoma symptoms may include: seizure, neurological deficits (weakness or speech problems), headaches, personality changes and visual loss.

Treatment
The prognosis for any patient with a GBM is also very dependent upon age (older people do not live as long as young patients) and performance status (patients who are neurologically normal and independent live longer than patients who have a neurological deficit).

When possible, surgery is performed to remove all cancerous tissue visible on the MRI, relieving intracranial pressure and possibly improving the effectiveness of other therapies.

Chemotherapy has been shown to add several weeks to survival.  Radiation implants (brachytherapy) have also been shown to increase survival but more than half of these patients require another operation to remove dead tissue resulting from the radiation.

Targeted therapy (also called precision medicine) focuses on matching patients to the right medicines, at the right time. This approach to biomedical research and medicine is a particularly pertinent in the area of brain tumors, especially with the most common and deadliest form of brain cancer, Glioblastoma Multiforme.

The thing is, GBMs can all respond differently to different treatments, according to four different genomic subtypes. This also explains why chemotherapy and radiation have been largely ineffective in treating GBM patients. If this proves true, treatments – or combinations of treatments – will need to be administered to distinct subgroups (or subpopulations) of patients whose tumors harbor the specific molecular alteration(s) and/or mutation(s) that the medicines can specifically effect.

These types of trials allow for molecular subtyping of patients, and, thus, the matching of patients to the right drugs at the right time.

In order to prove the success of targeted therapy, proper models for biomedical research and drug development need to be employed from basic research all the way through the clinical trial process.

BTF is working with these doctors and researchers. Glioblastomas need to be liquidated from the rhetoric of deadly brain tumors.

New Targeted Treatment: Optune for Glioblastomas
This treatment is for patients with newly diagnosed Glioblastomas, as well as for those with recurrent GBMs. A large clinical study has shown that Optune is as effective as chemotherapy, with patients self-reporting better quality of life.

For patients with newly diagnosed GBM, Optune would be used together with the chemotherapy temozolomide (TMZ) – only after surgery and radiation with TMZ. For those with recurrent GBMs, Optune would be used alone once treatment options like surgery and radiation have proven ineffective.

Optune uses 4 adhesive patches, called transducer arrays that are applied to the patient’s scalp and connected to the device and battery. The transducer array placement is determined based on each patient’s MRI results to help maximize the effect that Tumor Treating Fields (TTFields) have on the tumor. When turned on, Optune creates low-intensity electric fields which help slow or stop glioblastoma cancer cells from dividing and may also cause some of them to die.

For more information… view Targeted Therapy

MENINGIOMA

Description
Meningiomas are tumors that arise from the dura.  The dura mater is the membrane covering the entire interior of the skull.  It also creates compartments within the skull: the falx, which separates the right side of the brain from the left side and the tentorium, which forms the shelf separating the cerebrum from the cerebellum and brain stem.

Meningiomas are usually named for the part of the dura they come from.  Hence, a Meningioma starting in the falx is called a Falx Meningioma, a Meningioma in the tentorium is called a Tentorial Meningioma, a Meningioma in the cavernous sinus is called a Cavernous Sinus Meningioma and so on.  Convexity Meningiomas are tumors located over the outer surface of the brain.   Some Meningiomas are named for the bone that the tumorous dura covers.

Symptoms
Some Meningiomas are more aggressive than others and tend to recur faster. Atypical Meningiomas are classified Grade II and radiation therapy is recommended for these tumors following surgery.  Anaplastic Meningiomas have cells that exhibit a loss of structural differentiation, are abnormal in appearance and may show evidence of increased capacity for cell division, often a marker of malignancy.  Malignant Meningiomas (Grade III) and Papillary Meningiomas are malignant and will invade adjacent brain tissue.

Malignant Meningiomas
Some Meningiomas are more aggressive than others and tend to recur faster. Atypical Meningiomas are classified Grade 2 and radiation therapy is recommended for these tumors following surgery.  Anaplastic Meningiomas have cells that exhibit a loss of structural differentiation, are abnormal in appearance and may show evidence of increased capacity for cell division, often a marker of malignancy.  Malignant Meningiomas (Grade 3) and Papillary Meningiomas are malignant and will invade adjacent brain tissue.

Treatment
Radiation therapy is necessary following surgery.

METASTIC TUMORS

Description
Metastatic tumors do not start in the brain.  They travel to the brain from tumors that have started in some other part of the body such as the lung, breast, kidney or bowel.  These individual tumor cells usually get from their primary site to the brain via the blood stream.  Upon reaching the capillary blood vessels of the brain, the tumor cells take root and there continue to grow as another tumor. Sometimes multiple Metastatic tumors are formed and can be found in different areas of the brain.

The most common sites of the primary tumor that can spread to the brain are: lung, bowel, breast and kidney.  It is also not uncommon for Melanomas (a form of skin cancer) to travel to the brain.

Metastatic tumors are as malignant as the cancerous primary tumor from which it came and the edema of a Metastatic tumor can be more pronounced than that of a primary brain tumor. Edema adds to the mass effect and can cause dysfunction of the surrounding normal brain.

Symptoms
The mass of the tumor plus the volume of swollen brain around the metastatic tumor can irritate surrounding brain tissue and cause seizures.  If the metastatic tumor is located in an important part of the brain, the surrounding edema can also impair normal functioning of the brain.

Neurological deficits may include: weakness of face, arm or leg, trouble with speech or problems with walking, balance, memory or thinking.

Treatment
Depending on the location of the tumor, surgery and/or chemotherapy and radiation are recommended.  Anticonvulsants may be prescribed to prevent seizures.  Steroids may be prescribed to reduce some of the swelling.

MIXED GLIOMAS

Description
The brain is comprised of nerve cells – as well as other cells that support and protect the nerve cells, called glial cells.  Most brain tumors are named after the cells from which they develop.  A tumor of glial cells is known as a Glioma.

Astrocytomas, Ependymomas and Oligodendrogliomas are all types of Gliomas and they are named after the cells from which they grow: astrocytes, ependymal cells and oligodendrocytes.

A Mixed Glioma is a malignant tumor that contains more than one of these cell types.  The most common site for a Mixed Glioma is the cerebrum, the “main” part of the brain.  Like other malignant tumors it may spread to other parts of the brain.

Symptoms
The most common symptoms are headaches, lethargy or sleepiness, lack of coordination, and behavioral or personality changes.

Treatment
The possibility of surgery will be determined by the location of the tumor. Chemotherapy and/or radiation may also be recommended.

OLIGODENDROGLIOMA

Description
Oligodendrogliomas comprise a class of Glial Tumors in which the oligodendroglial cell is the predominant cell type.  Normally, oligodendroglial cells form myelin, the fatty substance that surrounds the axons of nerve cells, providing insulation so that nerve cell electrical transmission is faster and more efficient.

Each of these cell types has a percentage of cells capable of mitosis (the process by which one cell divides to become two cells).  In Oligodendrogliomas, the mitotic rate of oligodendroglial cells exceeds the rate of the other cells.  Eventually, the oligodendroglial cells are the most numerous or “predominant” cell type, resulting in an Oligodendroglioma.

Oligodendrogliomas are often classified as: low-grade, anaplastic (cells exhibit a loss of structural differentiation, are abnormal in appearance and may show evidence of increased capacity for cell multiplication) or malignant.  Of course, low-grade tumors can become anaplastic and Anaplastic tumors can become malignant.  Because the speed of this transformation varies widely from patient to patient, there is no consistent way of predicting the behavior of any Oligodendroglioma – particularly in adult patients.

Symptoms
Seizures occur in 50 to 80 percent of patients.  Other symptoms include: headache, nausea and vomiting, ataxia (difficulty with balance), dizziness and altered mental state.  There are three ways to judge the growth of Oligodendrogliomas: progressive enlargement on CT or MRI, development of new symptoms and development of new contrast enhancement.

Oligodendrogliomas, like most Glial tumors, have two growth patterns: solid tumor tissue and infiltrating tumor cells.

Treatment
It is difficult to surgically remove a solid tumor because it would require excising “healthy” tissue with “diseased” tissue.  Radiation therapy and chemotherapy can be effective.

OPTIC NERVE GLIOMA

Description
Named for its location on or near the nerve pathways between the eyes and the brain, an Optic Nerve Glioma can range from low-grade to high-grade.  This tumor is a type of Juvenile Pilocytic Astrocytoma (JPA) occurring most often in infants and children, though they can occur in adults as well.

Symptoms
Symptoms of an Optic Nerve Glioma include: headaches, progressive loss of vision and double vision.

Treatment
Surgery is standard treatment, usually followed by radiation therapy or chemotherapy.  Chemotherapy may be given to very young children instead of radiation therapy to avoid damage to the developing brain.

PINEAL TUMORS

Description
There are a number of different cells in the pineal gland that can grow into a tumor.  The pinealocyte, the cell with the greatest number in the gland, can transform into a Pinealocytoma (Pinealoma) or to the malignant Pinealoblastoma. Primitive cells within the pineal gland can give rise to Germinomas or Teratomas. Teratomas are usually benign but can turn into Teratocarcinomas that can be highly malignant.  Finally, the pineal gland has glial cells, and these can develop into Astrocytomas, Oligodendrogliomas, or Mixed Gliomas.  Pilocytic Astrocytomas can develop in this area also.

Symptoms
The most common symptoms are diplopia (loss of ability to look upward), double vision and nystagmus (flickering of the eyes back and forth).  Other symptoms are related to hydrocephalus and increased intracranial pressure, such as: headaches, nausea, vomiting, lethargy, lack of coordination and altered mental function.

Treatment
Pineal tumors are in contact with the spinal fluid and therefore can seed the CSF (cerebrospinal fluid), giving the tumor cells access to the spine and other parts of the brain.  Because of the possibility of metastasis, these lesions must be treated with chemotherapy or cranio-spinal irradiation therapy following surgery.

PINEOBLASTOMA, PINEOCYTOMA, AND GERMINOMA

Description
Pineoblastoma, Pineocytoma and Germinoma are all different types of tumors that can grow in the area of the pineal gland.Germinomas are almost always found in male patients.

Symptoms
The most common symptoms are diplopia (loss of ability to look upward), double vision and nystagmus, (flickering of the eyes back and forth).  Other symptoms are related to hydrocephalus and increased intracranial pressure, such as: headaches, nausea, vomiting, lethargy, lack of coordination and altered mental function.

Treatment
Pineal Tumors are in contact with the spinal fluid and therefore can seed the CSF (cerebrospinal fluid), giving the tumor cells access to the spine and other parts of the brain.  Because of the possibility of metastasis, these lesions must be treated with chemotherapy or cranio-spinal irradiation therapy following surgery.

PITUITARY TUMORS

Description
The pituitary gland produces hormones that affect growth and the functions of other glands in the body.  Tumors arising from the pituitary gland itself are called Adenomas, which are benign and slow growing.  Most Pituitary tumors grow in the front two-thirds of the gland, at the adenohypophysis, and are classified as secreting or non-secreting tumors.  The majority of Pituitary tumors are secreting tumors, producing excessive amounts of hormones and further classified by the type of hormone secreted.

Pituitary tumors can range from low-grade to high-grade. Pituitary Carcinoma is the rare malignant form of Pituitary Adenoma, diagnosed by metastases in or out of the nervous system.

Symptoms
The symptoms most commonly associated with Pituitary tumors include: headache, depression, vision loss, nausea or vomiting, behavioral and cognitive changes, cessation of menstrual periods (amenorrhea), leaking of fluid from the breasts (galactorrhea), hair growth in women, impotence in men, abnormal growth of hands and feet and abnormal weight gain.

There may be an over-secretion of hormones or impaired hormone production. Hormones most commonly affected include growth hormone, prolactin (controls lactation), sex hormones (menstrual cycle and other sex functions), thyroid gland hormones, adrenal gland hormones and vasopressin (water and electrolyte balance).

Treatment
If the tumor is large or compressing the optic nerve, the standard treatment is surgery.  This can be trans-sphenoidal surgery, when the surgeon gains access to the tumor by entering through the nasal passage.  Radiation therapy may also be used.  Some Pituitary tumors may be treated with medication, and observed with MRI scans.  Certain drugs can block the pituitary gland from overproducing hormones.  Follow-up with an endocrinologist may be necessary to manage hormonal changes.

For pituitary carcinoma, the range of symptoms is similar to the benign tumor, and treatment may include surgery, radiation, chemotherapy and hormone therapy.

PNET (Primitive Neuroectodermal Tumor)

Description
These tumors appear identical to Medulloblastomas but occur primarily in the cerebrum, the part of the brain associated with thought, movement and sensation. PNETs arise from undeveloped brain cells.  They are highly malignant and tend to spread throughout the central nervous system (CNS).  Additionally, PNETs tend to be large and commonly include areas of necrosis (dead cells) and cysts, so symptoms of increased intracranial pressure and seizures are common.

There are several tumor types in this category and the names of specific PNETs may be based on the tumor location.

Symptoms
Because a PNET can be located anywhere in the cerebrum, the symptoms of this tumor can vary, depending on location. Weakness or change in sensation on one side of the body can occur.  The patient may experience morning headaches or headaches that go away after vomiting.  Nausea, vomiting and seizures are common.  Unusual sleepiness or lethargy as well as behavioral or personality changes are also present with tumors in the cerebrum.

Unusual weight gain or weight loss may occur, particularly if the PNET is at the hypothalamus.

Treatment
Surgery is the standard treatment when possible, though not suggested for tumors in the pineal region or PNETs of the spine.  In adults and children over three years of age, surgery may be followed by radiation therapy to the whole brain and spinal cord, and chemotherapy.

In children under three years of age, surgery may be followed by chemotherapy or a clinical trial of chemotherapy to delay or reduce the need for radiation therapy.

RHABDOID

Description
Rhabdoid tumors are rare, highly aggressive tumors that tend to spread throughout the central nervous system (CNS), often appearing in multiple sites in the body – especially the kidneys.

These tumors may be difficult to classify and are sometimes confused with Medulloblastomas or PNETs (Primitive Neuroectodermal tumors).

Symptoms
Symptoms of a Rhabdoid tumor vary depending on the location of the tumor in the brain or body.

An Orbital tumor may cause the eye to protrude and balance problems to occur. External tumors cause noticeable lumps.

Treatment
Whenever possible, surgery is performed to remove as much of the tumor as possible. This is usually followed by chemotherapy and radiation therapy. In children under three years of age, surgery may be followed by chemotherapy alone.

Clinical trials are being studied using autologous bone marrow transplantation after high-dose chemotherapy for recurrent or multiple Rhabdoid tumors.

MALIGNANT NERVE SHEATH TUMORS (MNST)

Description
Malignant Nerve Sheath tumors (also called Neurofibrosarcoma) occur along the sheaths that surround peripheral nerves. These tumors usually begin as a localized tumor, but then metastasize, typically to the lungs.

Malignant Nerve Sheath Tumors can arise after previous radiation treatment of tumors. They also account for some soft-tissue sarcomas.

Symptoms
Nerve pain is a primary feature of an MNST. Other symptoms may include: a mass, or thickening in the muscle fibers, numbness, burning, or “pins and needles”, weakness in the affected muscles, dizziness or loss of balance.

Treatment
Surgery is the first and best treatment for MNSTs though some complications – loss of neurological function and profound excision of soft tissues – can occur. The tumor must be completely removed to avoid recurrence.

Radiation and chemotherapy are required after surgery.

 

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