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Brain Tumor Glossary
| A | B
| C | D | E | F
| G | H | I | J
| K | L | M |
| N | O | P | Q
| R | S | T | U
| V | W | X | Y
| Z |
A
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Acoustic Neuroma
The acoustic neuroma
is a benign tumor arising from the vestibular nerve (cranial nerve
number 8 out of 12 total). Common symptoms are loss of hearing,
ringing (tinnitus) and ataxia. The audiogram may show increased
"pure tone average" (PTA), increased "speech reception
threshold" (SRT) and decreased "speech discrimination"
(SD). The MRI commonly shows a densely "enhancing" (bright)
tumor in the internal auditory canal. Treatments include surgery,
radiosurgery and sometimes observation. Surgical approaches include
the suboccipital, translabyrinthine approach and middle fossa
approaches. For radiosurgery, single vs. fractionated (multiple
smaller treatments rather than one large treatment) are options.
The fractionated stereotactic radiosurgery
(FSR) appears to offer very high rates of control with preservation
of hearing and preservation of the facial strength.
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Agnosia
Loss
of ability to recognize objects, people, sounds, shapes, or smells.
Usually classified according to the sense or senses affected (hearing,
sight, smell, taste, touch), e.g. auditory agnosia, visual agnosia,
gustatory agnosia. Symptom common to tumors of the parietal lobe
of the cerebral hemispheres.
Deep parietal lobe tumors can result
in acalculia (inability to add, subtract), and agraphia (inability
to write). The parietal lobe is a sensory integration center,
and tumors in this location affect sensory integration.
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Anaplasia
Characteristics of
a cell (structure as seen under the microscope) that make it identifiable
as a cancer cell. These features can include bizarre shape of
the nucleus of the cell, evidence of cell division (mitosis) and
irregular shape of the cell and/or the nucleus of the cell. Examples
are anaplastic meningioma or anaplastic astrocytoma.
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Angiogenesis
Growth of new blood
vessels from surrounding normal tissue into growing tumor tissue.
Anti-angiogeneis drugs are being explored for the treatment of
many tumors.
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Anosmia
Absence of the sense
of smell. This can be a symptom of tumors of the middle of the
"skull base" such as the meningioma. The nerves for
smell (olfactory nerves) occupy the olfactory groove, a common
location for the meningiomas. Sometimes surgery can result in
anosmia if the surgery results in disruption or removal of the
olfactory nerves.
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Astrocytoma
Glial tumor that may
less invasive (pilocytic astrocytoma WHO Grade I) or moderately
invasive (fibrillary astrocytoma WHO Grade II). For fibrillary
astrocytomas common therapies are surgery or biopsy followed by
observation or conventional radiotherapy. For pilocytic astroytomas
the primary treatment is surgery.
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| Ataxia
Imbalance
of walking (gait). For patients with tumors, the location is commonly
within the "posterior fossa" (back of brain), the location
for the cerebellum. The cerebellum allows co-ordination of the
arms and legs. The tumors that may press on the cerebellum and
cause ataxia include acoustic neuromas, meningiomas, epidermoids
and astrocytomas (gliomas).
For
midline cerebellar tumors (medulloblastoma, for example) there
can be "axial" ataxia (inability to control the trunk
or midline structures of the body. For the peripheral cerebellar
tumors, the co-ordination of the distal extremities is affected.
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B
| Benign
Tumors
that have non-malignant appearance under the microscope and that
demonstrate a long natural history (grow slowly). These tumors
usually have slow rates of growth and have low infiltration into
surrounding normal brain. Under the microscope these tumors have
relatively normal appearing cellular structures and infrequent
signs of division (mitosis).
Examples of benign tumors include
acoustic neuromas, meningiomas and pituitary adenomas. The "benign"
tumors can still pose serious threats to health. If the location
is difficult to reach surgically and if growth occurs, critical
structures of the brain can be compressed and affected.
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Blood Brain Barrier
A protective barrier
formed by the blood vessels and glia of the brain. It prevents
some substances in the blood from entering brain tissue. The blood
brain barrier prevents many chemotherapy agents from entering
the brain. Because of the blood brain barrier and the restriction
of the entry of chemotherapy, techniques such as radiotherapy
and the stereotactic radiosurgery are appealing options for the
treatment of many brain tumors.
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Brain Metastasis
Tumors
that have spread to the brain from outside the brain. The brain
metastases can be single (solitary) or multiple. Commonly, brain
metastases arise from lung cancer, breast cancer and colon cancer.
Options for treatment include surgery, radiotherapy and radiosurgery.
For tumors not accessible by surgery, the radiosurgery offers
precise treatment while sparing the surrounding normal brain.
Radiosurgery can be given with or without whole brain radiation
(regular radiation).
The determinants of the best treatment
include the patient's age, the patient's "performance status"
(Karnofsky Performance Status) ("KPS"), the control
of the primary tumor (tumor outside the brain), and the presence
of any metastases other than to the brain.
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C
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Carcinoma
A carcinoma is a cancer
that starts outside of the brain, and is derived from "epithelial"
sites such as lung, breast, colon, prostate, and head and neck.
The carcinoma can be "adenocarcinoma" or "squamous
cell carcinoma." The carcinoma can spread "metastasize"
to the brain. The tumor that has spread to the brain (the "metastasis")
can be treated with surgery or radiosurgery. The choice depends
on the size, number, location and the rapidity of worsening of
the symptoms. The carcinoma can be "staged" A carcinoma
that starts outside the brain and spreads to the brain is "Stage
4" (on a 1 to 4 scale).
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Cerebellopontine Angle
The region in the
back of the brain that is the junction of the "cerebellum"
and the "pons." The pons is near the midline and is
the exit point for many important cranial nerves, including the
facial (facial strength), the auditory (hearing) and vestibular
(balance) nerves. Tumors in this region can cause symptoms including
hearing loss, ataxia (imbalance while walking), facial weakness,
difficulty with swallowing, speaking and facial numbness.
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Cerebellum
The portion of the
brain in the back of the head between the cerebrum and the brain
stem. The cerebellum controls balance for walking and standing,
and other complex motor functions.
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Cerebral Hemispheres
The two halves of
the brain. The hemispheres of the brain control muscle functions
of the body and also controls speech, emotions, reading, writing,
and learning. The right hemisphere controls muscle movement on
the left side of the body, and the left hemisphere controls muscle
movement on the right side of the body.
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Cerebrospinal Fluid
The clear fluid made
in the ventricular cavities of the brain that bathes the brain
and spinal cord. It circulates through the ventricles and the
subarachnoid space. If the flow is obstructed by tumor growth
or other process, hydrocephalus may result and a "shunt"
may be required. If the cerebrospinal fluid becomes infected,
the result is meningitis.
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Choroid Plexus Papilloma
Benign tumor arising
from the choroid plexus of the brain. The choroid plexus resides
in the "ventricles" (spinal fluid spaces) of the brain.
These tumors enhance brightly on MRI. Surgery is the primary treatment.
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Craniectomy
Surgical
operation on the brain in which a portion of the skull is drilled
away leaving an opening in the skull. This opening may be left
or may be covered by synthetic material (as in the "cranioplasty").
A common procedure is the "suboccipital craniectomy"
for tumors (e.g. acoustic neuromas or meningiomas) in the rear
or back of the brain (cerebellum; cerebellopontine angle).
The suboccipital craniectomy can
often result in persistent headaches after surgery.
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Craniotomy
Surgical operation
on the brain in which a portion of the skull is temporarily removed
in order to gain access to the brain. In this procedure holes
("burr holes") are drilled circumferentially around
the area to be removed. The "burr holes" are joined
using a high speed drill. The "bone flap" (circular
piece of skull) is then carefully lifted away, exposing the dura
underneath. The portion of the skull that is removed is replaced
(distinguish "craniectomy"). After the craniotomy, the
bone edges of the portion that was removed heals together with
the surrounding normal skull, resulting in a hard "fusion"
with resultant preservation of the protective skull.
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Cushings Disease
Cushings
Disease is overproduction of ACTH (adrenocorticoctropin hormone)
by a pituitary tumor or pituitary hyperplasia. The overproduction
of ACTH results in hypercortisolism or Cushings Syndrome characterized
by obesity, new hair growth, purple striae (discoloration of skin),
mental symptoms, poor wound healing, severe infections, muscle
wasting, acne and amenorrhea. The obesity results from the accumulation
of adipose tissue particularly in the facial, truncal and girdle
areas. Hypertension and osteoporosis are common. Mental symptoms
include emotional lability, irritability or psychosis.
Treatments
may include surgery, radiotherapy, radiosurgery or fractionated
stereotactic radiosurgery (FSR).
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D
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Diplopia
Double
vision. The diplopia is often caused by compression of the "cranial
nerves" that subserve eye motion. If one eye moves but the
other doesn't, diplopia will result.
•The cranial nerve III (oculomotor) causes medial, superior and
inferior motion.
•The cranial nerve IV (trochlear) causes inward and downward motion.
•The cranial nerve VI (abducens) causes lateral movement.
Diplopia may be caused, for example,
by the meningioma of the cavernous sinus that compresses one or
more of these cranial nerves.
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Dysphasia
Inability
to make speech that is understandable. The patient can form sounds,
but not speech. The "expressive" dysphasia can be caused
by frontal lobe tumors, usually on the "dominant" side
of the brain near the inferior motor strip.
In
the "receptive" dysphasia the patient cannot understand
his own speech and the speech of others, and can be unaware that
there is a speech problem. The receptive dysphasia can be caused
by tumors of the superior temporal lobe on the dominant side of
the brain (left side for most right handed persons).
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| Dura
The
dura is the tough, fibrous covering of the brain. During the craniotomy
or craniectomy (operation) the dura is opened with a knife and
folded back to expose the brain underneath. After the operation
the dura is usually closed with sutures in a "water tight"
fashion to prevent leakage of cerebrospinal fluid.
The meningiomas can arise from
the dura. During surgery for meningiomas with dural attachments,
resection of the dura with the meningioma is desirable to help
prevent recurrence of the meningioma.
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E
| Edema
Edema is swelling
of the brain secondary to the tumor, surgery or radiotherapy.
The edema is the result of increased water or fluid content within
the brain. The MRI can show edema as bright (white) signal on
the "T2 weighted" images. The "infiltrating"
tumors (such as glioblastomas) will commonly produce edema. Meningiomas
and metastases can result in edema as well. The edema is commonly
treated with "dexamethasone" (Decadron) that can reduce
the water content and hence reduce the edema. Other causes of
edema include stroke.
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Ependymoma
Ependymomas are tumors
arising from the cells that line the cavities holding the cerebrospinal
fluid (CSF) in the brain and the spinal cord. These tumors are
usually treated with surgery. Histologically (under the microscope)
these tumors are usually benign but can have atypical or even
malignant features. Radiosurgery is an option to boost regular
radiation or by itself to treat the ependymoma.
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F
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Facial Nerve
Although
it is considered a pure motor nerve, the facial nerve also innervates
a small strip of skin of the posteromedial aspect of the pinna
and around the external auditory canal. The nervus intermedius
of Wrisberg conducts taste sensation from the anterior two-thirds
of the tongue and supplies autonomic fibers to the submaxillary
and sphenopalatine ganglia, which innervate the salivary and lacrimal
glands.
A lower motor neuron lesion (LMNL)
of the nerve, also known as peripheral facial paralysis, results
in complete ipsilateral facial paralysis; the face draws to the
opposite side as the patient smiles. Eye closure is impaired,
and the ipsilateral palpebral fissure is wider.
In an upper motor neuron lesion
(UMNL), also known as central facial paralysis, only the lower
half of the face is paralyzed. Eye closure is usually preserved.
In peripheral facial paralysis,
a different type of clinical presentation is seen with four different
levels of nerve lesion.
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A) Lesions of the meatal or
canalicular segment. Facial paralysis with hearing loss, and
loss of taste in the anterior two-thirds of the tongue, imply
lesions in the internal auditory canal from fracture of the
temporal bone or at the cerebellopontine angle form compression
by tumors.
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B) Lesions of the labyrinthine
or fallopian segment. Lesions that spare hearing (with hyperacusis)
indicate lesions further down the course of the nerve. Loss
of taste in the anterior two-thirds of the tongue and loss of
tearing imply lesions that involve the chorda-tympani and the
secretomotor fibers to the sphenopalatine ganglion in the labyrinthine
segment, proximal to the greater superficial petrosal nerve
(GSPN). Distal to GSPN lacrimation is normal, but hyperacusis
is still present. Geniculate lesions in this segment cause pain
in the face.
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C) Lesions of the horizontal
or tympanic segment. The lesion is proximal to the departure
of the nerve to stapedius and results in hyperacusis, loss of
taste in the anterior two-thirds of the tongue, and facial motor
weakness.
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D)
Lesions of the mastoid or vertical segment. Hyperacusis is present
if the lesion is proximal the nerve to stapedius. It is absent
if the lesion is beyond the nerve to stapedius and only loss
of taste and facial paralysis occur. If the lesion is beyond
the chorda tympani in the vertical segment (as in lesions of
the stylomastoid foramen), taste is spared and only facial motor
paralysis is seen.
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FSR (Fractionated
Stereotactic Radiosurgery)
FSR (Fractionated
Stereotactic Radiosurgery) is radiosurgery that is given in smaller,
separate, daily treatments (fractions) rather than one large single
treatment. The total dose (Gray) to the tumor
is higher for the FSR treatment when compared to single "shot"
radiosurgery. But because the treatment is given over consecutive
days in smaller daily doses, there is much greater sparing (saving
the function) of normal neural (brain) tissues. This is because
normal tissues cannot tolerate large single doses of radiation.
These normal brain tissues can tolerate many smaller doses, however.
The tumor cannot tolerate either single large treatments or several
smaller treatments. FSR therefore exploits this important difference
to kill the tumor and spare normal brain. FSR results in less
inhomogeneity of the dose.
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G
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Germinoma
This tumor looks just
like ovarian or testicular cancer under the microscope. It is
the most common of the germ cell tumors of the brain. It may spread
or "seed" through the spinal fluid. About one third
of tumors in the pineal region are germinomas; however, this tumor
can occur in many locations within the brain. This tumor may cause
headaches, visual problems, hormonal disturbances and blockage
of spinal fluid (hydrocephalus). It is treated with surgery (often
a biopsy for making the diagnosis), radiotherapy and sometimes
chemotherapy. The germinoma is very responsive to radiation therapy,
but doctors may attempt to avoid the use of radiation in the very
young.
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| Gray
Gray
is the unit of radiation absorbed dose for the MKS (metric: meter
kilogram second) system. The Gray is equal to 100 rad (rad is
both singular and plural). The "centigray" (cGy) is
equal to 1/100 of a Gray and is equal to 1 rad.
Examples of common doses for single
"shot" radiosurgery are 12 to 14 Gray (1200 to 1400
centigray; 1200 to 1400 rad).
Examples of common doses for Fractionated
Stereotactic Radiosurgery are 25 Gray (2500 cGy (rad) in 5
fractions (separate daily doses) or 30 Gray (3000 cGy (rad) in
10 fractions.
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H
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Hemangioblastoma
These are usually
benign tumors of blood vessels, often found in the lower part
of the brain (posterior fossa). They may be associated with cysts.
Although usually solitary, multiple hemangioblastomas can occur
in Von Hippel-Lindau disease (see below), a hereditary disorder
that may also be associated with tumors of the retina, pancreas
and/or kidney. This tumor may cause dysfunction of the cerebellum
(causing difficulty with walking and/or co-ordination, for instance).
It also may block the drainage of spinal fluid, leading to hydrocephalus.
Treatment is usually by surgical removal, and can be curative.
There are some reports of successful treatment with radiosurgery.
Radiation therapy or radiosurgery may also be used if the tumor
cannot be removed completely.
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Hemianopsia
Loss of one half of
the visual field. May be "homonymous" (same half of
both eyes, i.e. left field of both eyes) or bitemporal (lateral
portion of visual field for each eye). The bitemporal hemianopsia
may be caused by pituitary tumor, craniopharyngioma or meningioma
that presses on the optic chiasm. The homonymous hemianopsia may
be caused by tumors that press on the optic pathway on one side
of the brain, posterior to the optic chiasm. These tumors can
include the "visual cortex" (part of the brain that
processes visual information) located in the "occipital lobe"
at the back of the brain.
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H
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Hydrocephalus
The abnormal accumulation
of cerebrospinal fluid in the ventricles of the brain. Tumors
may cause hydrocephalus by compressing or occluding the normal
pathways for flow of the cerebrospinal
fluid in the brain. To restore flow the operative placement
of the shunt may be required.
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Hyperfractionation
Hyperfrationation
is the administration of a larger number of smaller radiation
treatments (fractions). Hyperfractionation takes advantage of
the ability of normal brain to tolerate larger numbers of smaller
treatments, while the tumor cannot tolerate the smaller treatments,
resulting in killing of the tumor and preservation of the surrounding
normal brain.
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I
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Inhomogeneity
Inhomogeneity
of radiation dose refers to the "hot spot" in radiation
treatment plan. This can result from overlap of two or more "isocenters"
(center of rotation of the radiation machine) of treatment. The
inhomogeneity can result in toxicity (loss of function of normal
tissue) if the normal tissue is within the "hot spot."
Inhomogeneity is common with the "gamma knife."
The
size (magnitude) of the inhomogeneity can be minimized by the
larger collimators that are available using the fractionated
stereotactic radiosurgery (FSR) technique via the dedicated
linear accelerator. To reduce the effect of the inhomogeneity
"fractionation" (FSR) (multiple smaller treatments rather
than one large treatment) is used.
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Intrathecal Chemotherapy
Anticancer
drugs infused into the thin space between the lining of the spinal
cord and brain to treat or reduce the risk of cancers in the brain
and spinal cord. The drugs may be given through a "lumbar
puncture or lumbar "tap" or via the "Ommaya
reservoir."
The intrathecal chemotherapy bathes
the tumor via the spinal fluid. This therapy may be give on a
"cyclic" basis (regularly at specific intervals or cycles).
This therapy is consideration for tumors that have spread throughout
the spinal fluid and that coat the surface of the brain, the lining
of the brain, or the spinal cord.
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Isocenter
The isocenter is the
center of rotation for the linear accelerator that delivers the
radiosurgery. For cobalt source devices (gamma knife) the isocenter
is the point at which all of the sources point. It is the "focal
point" of the radiation treatment. The isocenter is within
the tumor target. The resultant dose conforms to the tumor target
and drops rapidly outside of the tumor target. A tumor may have
more than one isocenter. Multiple isocenters may be necessary
to cover the entire volume of the tumor target.
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L
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Lumbar Puncture (Tap)
The lumbar puncture
or "tap" is the insertion of a small needle into the
lower back, after local anesthetic, to obtain spinal fluid (cerebrospinal
fluid, or CSF). The CSF is then examined to determine the presence
of any cells (tumor cells from a brain or spinal tumor, or red
cells if a bleed is suspected in the brain). If tumor cells are
present, this may mean that the brain tumor is not well localized
and that therapy other than surgery or radiosurgery may be necessary.
The lumbar tap is only performed when the surgeon is convinced
that there is not increased pressure in the brain (increased intracranial
pressure).
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M
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Malignant
Cancerous: a growth
with a tendency to invade and destroy nearby tissue and spread
to other parts of the body. Under the microscope malignant tumors
show high rates of cellular division, bizarre-shaped nuclei, irregular
shape, and dense staining of the enlarged nucleus (high nuclear
to cytoplasmic ratio). Often specific stains are utilized by the
pathologist to further characterize the malignant tumor, including
estrogen or progesterone receptors (meningioma), Ki-67 (nuclear
proliferation antigen), and others. On examination of the tumor
during the operation, the malignant tumor shows irregular blood
vessels and infiltration (spread) into the surrounding normal
brain. This extension into the surrounding brain may make total
removal impossible. Examples include glioblastoma multiforme,
anaplastic astrocytomas, and malignant meningiomas.
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Meningioma
The meningioma is
a benign tumor of the cells that line the brain (meninges). The
meningioma arises between the skull and the brain. The meningiomas
have "pushing" borders and can compress but usually
not invade the brain (see "malignant meningioma"). These
tumors are typically benign and grow slowly, but may, on occasion
have a more aggressive course (atypical and malignant meningiomas).
The locations include convexity (curved part of skull), the cavernous
sinus, sphenoid wing, clivus and parasellar regions. Cerebellar
meningiomas also occur. Treatments include surgery, regular radiation
and radiosurgery. For the meningiomas that are difficult to safely
remove (cavernous sinus, parasellar, clivus) the radiosurgery
is a useful option for treatment. The fractionated stereotactic
radiosurgery results in greater sparing of the normal surrounding
tissues and the cranial nerves when compared to the single "shot"
radiosurgery techniques.
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| MRI
Magnetic
resonance imaging. A procedure in which a magnet linked to a computer
is used to create detailed pictures of areas inside the brain.
The MRI may visualize tumor "enhancement" that results
from the accumulation of the "gadolinium" that is injected
into the vein prior to the MRI. The gadolinium results in brightness
in the MRI picture wherever it accumuulates in the brain.
MRI may be used to quantitate "metabolites"
or biochemical compounds in brain and brain tumor to estimate
how actively growing the tumor may be. This is MRI spectroscopy.
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N
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Neurofibromatosis Type
II (NF II)
Neurofibromatosis
II is a common autosomal-dominant genetic disorder (parent has
50% risk that children will have NF II) caused by deletion of
chromosome 22 and resulting in bilateral acoustic neuromas, meningiomas
and optic nerve sheath meningiomas. Raised skin plaques and opacities
in the lenses of the eyes are common.
Multiple
Schwann cell tumors on the peripheral nerves, cranial nerves and
spinal roots are common in patients with bilateral acoustic neuromas
(NF II). For the acoustic neuromas arising from NFII the options
for treatment include surgery vs. radiosurgery.
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O
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Oculomotor Nerve
The
oculomotor nucleus of the nerve is located in the midbrain and
innervates the pupillary constrictors; the levator palpebrae superioris;
the superior, inferior and medial recti; and the inferior oblique
muscles. Lesions result in paralysis of the ipsilateral upper
eyelid and pupil, the inability to adduct and look up or down.
Frequently, the eye is turned out (exotropia). In subtle cases,
patients complain only of diplopia or blurred vision.
The exotropia seen in 3rd nerve
paralysis can be distinguished from that in internuclear ophthalmoplegia
because in the latter, convergence is preserved.
Paralysis of the third nerve is
the only ocular motor nerve lesion that results in diplopia in
more than one direction, thus distinguishing itself from 4th nerve
paralysis (which also can result in exotropia). Pupillary involvement
is an additional clue to involvement of the 3rd nerve. Pupil-sparing
3rd nerve paralysis occurs in diabetes mellitus, vasculitides
of other etiologies and certain brainstem lesions.
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Olfactory Groove
The
olfactory groove is the site of many meningiomas. The olfactory
nerve (cranial nerve #1 (of 12) may be affected, resulting in
"anosmia" (absence of sense of smell).
The olfactory groove arises in
the lamina cribrosa of the ethmoid bone, 1 to 2 centimeters anterior
to the tuberculum sellae. This means the front, middle part of
the skull, between the eyes. The operative approach may include
the "bifrontal" craniotomy.
For tumors of the olfactory groove, the bifrontal exposure includes
elevation of the frontal lobe(s) of the brain to gain the maximal
exposure. Alternative treatments for olfactory groove tumors such
as the meningiomas, esthesioneuroblastomas, brain metastases and
gliomas may include FSR (fractionated stereotactic
radiosurgery).
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| Oligodendroglioma
The oligodendroglioma
is a benign tumor that arises from the oligodendroglial cells.
The primary therapy is biopsy or surgery. These tumors are thought
to arise from the oligodendrocytes, which are the cells that wrap
around nerve cells and act as a form of electrical insulation
for conducting the nerve impulses. Recent evidence suggests that
they may actually arise from progenitor cells that are immature
oligodendrocytes. These tumors also tend to occur in young adults
and may contain calcium deposits that appear on brain scans. They
tend to be slower growing than low-grade astrocytomas, but have
the potential to turn into more aggressive tumors. Treatment is
usually surgical and radiation therapy may be recommended. Chemotherapy
is considered for progressive tumors, and usually recommended
for anaplastic (i.e. more malignant) oligodendrogliomas. (Also
see anaplastic oligodendroglioma).
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Optic Nerve Glioma
Optic nerve gliomas
occur in about 10% of patients with neurofibromatosis type I (NF
I -- see below), usually during childhood. They may involve the
optic nerve, optic chiasm and/or optic tract, which are various
parts of the visual system. The tumor usually does not spread
to other parts of the brain, unless it is a higher grade (more
malignant) glioma. In those without NF I, they may occur at any
age. These tumors may be treated with radiation and/or chemotherapy;
surgery is also sometimes used depending on the patient.
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P
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Pilocytic Astrocytomas
Pilocytic astrocytoma
is the most benign variant of astrocytoma in the new World Health
Organization classification. Nearly two thirds of cases occur
in the cerebellum, 25% to 30% in the region of the optic nerve/
chiasm and hypothalamus, and the remainder in the cerebral hemisphere.
In general the patient can be cured by complete resection of the
tumor, which is frequently possible depending on location. If
the tumor is deep in the brain, surgery may not result in complete
removal or may not be feasible at all. There is usually long-term
tumor stabilization without adjuvant radiation therapy after resection,
even if removal is incomplete.
Pilocytic astrocytomas in the chiasm and hypothalamus are the
most difficult to treat. They usually cause visual loss, endocrinological
dysfunction, and/or raised intracranial pressure from obstruction
of the foramen of Monro. At least 40% of optic/chiasmatic tumors
are associated with NF. Complete resection is rarely possible
and management of residual tumor is controversial given the risks
associated with radiation and chemotherapy, especially in children.
Options include conservative management, stereotactic biopsy with
or without radiation, resection with or without radiation, and/or
chemotherapy.
Pilocytic astrocytomas of the optic chiasm and hypothalamus typically
are histologically benign tumors that present in childhood. The
management of these tumors is controversial. Presumed hypothalamic/chiasmatic
astrocytomas have traditionally been treated with surgical biopsy
followed by conventional radiation therapy. More recently other
approaches have been advocated, including radical surgical debulking
as primary treatment for exophytic tumors; however, the benefit
of this strategy has been difficult to demonstrate. Radical surgery
carries the risk of damage to the hypothalamus, visual apparatus,
and vascular structures. Furthermore, these tumors often have
a long course, and the small groups of patients reported who have
been treated with aggressive surgery have had a relatively short
follow-up period.
The most important aspect of the pilocytic astrocytoma suitable
for radiosurgery is the size. Smaller tumors are treated more
effectively by the radiosurgery.
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Pineal Tumors
The
pineal gland is a small gland located in the cerebrum that produces
melatonin. Also called pineal body or pineal organ.
The pineoblastoma is a fast growing
type of brain tumor that occurs in or around the pineal gland,
a tiny organ near the center of the brain. Treatment is biopsy
or surgical removal followed by radiotherapy with or without chemotherapy.
The pineocytoma is a slow growing
type of brain tumor that occurs in or around the pineal gland,
a tiny organ near the center of the brain. Treatment is biopsy
or surgery followed by observation or radiotherapy.
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Pituitary Adenoma
Pituitary adenomas
are common benign tumors of the pituitary gland. It is said that
up to 10 percent of people will have a pituitary adenoma (which
might never have caused a problem) by the time of their death.
The pituitary gland is considered the "master gland"
of the body; it produces hormones that regulate the other glands.
Some tumors secrete one or more of these hormones in excess. Such
so-called secretory pituitary adenomas are usually found due to
hormonal imbalances that affect bodily functions. They may be
relatively small when detected.
Pitutary adenomas
have specific signs and symptoms that are primarily related to
the endocrinopathies produced by hypersecretion.
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The prolactin-secreting
pituitary adenomas are the most common, and account for approximately
30% of all pituitary tumors. The clinical findings are galactorrhea
and reproductive dysfunction.
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The endocrinopathy
of excess growth hormone results in enlargement of the extremities,
face and the soft tissues, producing a characteristic appearance
called acromegaly. Acromegaly can be associated with hypertension,
diabetes mellitus and cardiovascular disease. Further, acromegaly
is associated with decreased life expectancy.
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Pituitary Tumor
The pituitary tumor
starts in the pituitary gland. These tumors can produce prolactin
(prolactinomas), ACTH (Cushings disease), growth hormone, and
can, on occasion, produce no hormone (non-functional adenoma).
In addition to the endocrine effects, the pituitary tumors can
can cause visual abnormalities (decreased peripheral vision bilaterally)
(bitemporal hemianopsia) due to pressure upon the optic nerve(s)
or the optic chiasm (juncture of the optic nerves, above the pituitary
gland). The pituitary gland tumors may remain within the bony
confines of the "sella turcica" or may extend laterally
into the "cavernous sinuses." For the cavernous sinus
location, the surgical resection may be difficult because of the
closeness to the cranial nerves that subserve eye movements and
facial sensation. The treatments include surgery, radiation, radiosurgery,
medications, and sometimes, observation.
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Primitive Neuroectodermal
Tumor
This is a malignant
tumor arising from cells that are believed to remain from fetal
brain development. They usually occur in early childhood but may
become symptomatic in adult life. In children, they commonly occur
in or near a spinal fluid sac known as the fourth ventricle and
are called medulloblastomas. Under the microscope, a PNET is seen
to consist of densely-packed small cells that are usually blue
in color (when common tissue processing is performed). PNETs have
a tendency to spread over the brain and spinal cord by way of
the spinal fluid. Treatment usually begins with surgery. Radiation
therapy is used in adults, along with chemotherapy. Young children
are usually treated with chemotherapy alone, since radiation may
stunt intellectual development when given at an early age.
PNET staging is an important consideration, since the extent
of treatment needed depends on how widely the PNET has spread
at the time of diagnosis. This "staging process" requires
MRI scans of the brain and spinal column, as well as examination
of the spinal fluid under the microscope to look for tumor cells.
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Prolactinoma
The prolactinoma is
a type of pituitary tumor that produces prolactin. The prolactin
stimulates milk production from the breast. The prolactinoma may
present with visual symptoms due to compression of the optic chiasm
if the tumor is large. The prolactin levels in the blood help
to make the diagnosis, and these levels can be very high. The
levels of prolactin are useful to monitor the success of treatment
(lower after treatment).
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Q
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Quadrigeminal
Quadrigeminal refers
to the posterior midbrain (deep brain) that holds the superior
and inferior colliculi (for processing of vision and hearing).
Tumors in the region of the "quadrigeminal plate" include
the pineal tumors (pineocytomas, pineoblastomas). These tumors
can compress the "third ventricle (cavity holding CSF just
anteriorly) and result in hydrocephalus (obstruction of CSF flow
in the brain).
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R
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Radiation Therapy
Radiation therapy
(also called radiotherapy) uses high-energy radiation from x-rays,
neutrons, and other sources to kill cancer cells and shrink tumors.
Radiation may come from a machine outside the body (external-beam
radiation therapy) or from materials (radioisotopes) that produce
radiation that are placed in or near the tumor or in the area
where the cancer cells are found (internal radiation therapy,
implant radiation, or brachytherapy). Systemic radiation therapy
involves giving a radioactive substance, such as a radiolabeled
monoclonal antibody, that circulates throughout the body.
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R
| Radiosurgery
The precise, focal
delivery of radiation (Gray) to a target in
the brain with sparing of the surrounding normal brain. The localization
of the target is achieved during the "simulation" (special
scan) and the planning of the treatment is achieved using special
software specifically designed for radiosurgery treatments. The
precision is afforded by the "stereotactic" (precise
localization in space within the brain) determination of the target
volume. With sub-millimeter accuracy high doses of radiation can
be delivered to the isocenter. Radiosurgery
is a common treatment for acoustic neuromas, meningiomas, pituitary
adenomas, hemangioblastomas and gliomas. Fractionated Stereotactic
Radiosurgery (FSR) is non-invasive, precise,
outpatient treatment that may offer improved safety of treatment,
escalation of dose and outpatient treatment.
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S
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Schwannoma
A type of benign brain
tumor that begins in the Schwann cells that produce the myelin
that protects the acoustic nerve (the nerve of hearing). The schwannoma
is slow-growing. The options for treatment include surgery vs.
radiosurgery.
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Seizure
Seizures are uncontrolled
activity of the neurons of the brain. These can result in jerking
of the body (tonic clonic seizures; motor seizures), loss of consciousness
or awareness with "automatisms" such as lip smacking
or chewing (absence or petit mal seizures) or other types (sensory,
visual). Seizures can be caused by brain tumors. Treatment of
the tumor can reduce seizures. The primary (initial) treatment
is medication (example: Dilantin or Phenobarbital).
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| Shunt
A surgically created
diversion of fluid, for example blood or cerebrospinal fluid,
from one area of the body to another area of the body. The shunt
may be ventriculoperitoneal (brain to abdominal cavity) or ventriculoatrial
(brain to heart).
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Stereotaxis
Use of a computer
and scanning devices to create three-dimensional pictures. This
method can be used to direct a biopsy, external radiation, or
the insertion of radiation implants.
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Subependymal Giant
Cell Astrocytoma
Generally a benign
tumor, arising from tissue off the fluid sacs (ventricles) of
the brain. This tumor is often seen in children and some adults
with a condition called Tuberous Sclerosis. Tuberous Sclerosis
is characterized by seizures, certain skin abnormalities of the
face, and varying degrees of mental retardation. Treatment usually
consists of surgical removal, or observation if it is not causing
symptoms.
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Subependymoma
Like an ependymoma,
this tumor also arises from tissue that lines the ventricles.
However, it more often occurs in elderly patients. The behavior
is benign. Unfortunately, subependymomas often arise from the
brainstem and surgeons may have to leave some tumor behind if
they are to avoid neurologic damage.
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T
| Tentorium
The tentorium is the
fibrous separation of the top (superior, "supratentorial")
brain from the bottom (inferior, "infratentorial") brain.
It is shaped like a "tent." Supratentorial vs. infratentorial
is a common designation for describing the location of tumors.
The tentorium arises from the temporal, parietal and occipital
bones laterally and joins the midline fibrous separation called
the "falx." The tentorium is not very flexible, and
masses or tumors within the infratentorial space (e.g. cerebellum,
cerebellopontine angle) can exert considerable pressure upon the
brain with only small increases in size.
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Tinnitus
Tinnitus is the high-pitched,
"ringing" sound that is perceived by many patients having
the acoustic neuroma(s). The tinnitus may be constant or intermittent,
and may increase in intensity over time.
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Trigeminal Nerve
The
nucleus of the nerve stretches from the midbrain (mesencephalic
n.) through the pons (main sensory nucleus and motor nucleus)
to the cervical region (spinal tract of the trigeminal nerve).
It provides sensory innervation for the face and supplies the
muscles of mastication.
Paralysis of the first division
(V1) is usually seen in the superior orbital fissure syndrome
and results in sensory loss over the forehead along with paralysis
of the 3rd and 4th nerves.
Paralysis of the second division
(V2) results in loss of sensation over the cheek and is due to
lesions of the cavernous sinus; it also results in additional
paralysis of V1 and the 3rd and 4th nerves.
Isolated V2 lesions result from
fractures of the maxilla. Complete paralysis of the 5th nerve
results in sensory loss over the ipsilateral face and weakness
of the muscles of mastication. Attempted opening of the mouth
results in deviation of the jaw to the paralyzed side.
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X
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Xanthoastrocytoma
This is a rare tumor
usually seen in young adults, often found in a temporal lobe after
a seizure. It tends not to infiltrate (mix) with normal brain
tissue, but may spread along the meninges. The tumor and further
seizures are usually controlled by its surgical removal, but it
is intermediate in its' grade (i.e. degree of malignancy). Follow-up
scans over time are recommended. Radiation, radiosurgery and/or
re-operation may be suggested for a recurrent tumor.
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