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7.
What are the symptoms of brain tumors? Do all brain tumors cause headache?
Brain tumors may be
discovered even when they do not cause any symptoms because brain scans are
commonly performed in emergency rooms after head trauma. However, the nature
and duration of symptoms are important clues to the location of the tumor
and sometimes may even suggest the type of tumor present.
It is estimated that,
every day, ten million people in the United States suffer from headache.
Fortunately, more than 99% of adults who suffer from headache do not have a
brain tumor. However, about half of all brain tumor patients complain of
headache at the time of diagnosis. As children seem to suffer headaches less
frequently than adults, a child who complains of headache should always
raise the suspicion of a brain tumor.
The incidence of
headache in brain tumor patients is related to both the growth rate and
location of the tumor. Slow-growing tumors are more likely to cause seizures
than headaches, whereas faster growing tumors may cause headaches as an
initial symptom. Tumors that obstruct the flow of spinal fluid are more
commonly associated with headache.
Headaches associated
with brain tumors do not always follow a clear, progressive pattern of
increasing severity. The headache may be worse in the morning or interfere
with sleep, or it may occur with bending, lifting, or exercise. Headaches
caused by brain tumors may be relieved with medications used to treat
migraine or tension headaches, or even over-the-counter drugs such as
acetaminophen (Tylenol) or aspirin.
Other frequent symptoms noted by brain tumor
patients include nausea and vomiting, visual problems, seizures, weakness,
confusion, imbalance, depression, and fatigue. It is not unusual for family
members to note subtle changes in an individual's personality. These changes
may be difficult for a doctor to detect unless he is very familiar with the
patient. Neurological deficits are partial or complete loss of muscle
strength, sensation, or other brain functions that may become more
pronounced with fatigue. Almost all patients with brain tumors exhibit at
least some neurological deficit at the time of diagnosis, although it may be
very subtle.
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8.
Why does every doctor I see ask me so many questions about my symptoms, my
previous illnesses, and my family history?
A detailed medical
history is extremely important to a doctor. It helps determine the
length and severity of an illness. A patient who comes to the emergency room
with a severe headache, for example, may or may not need an emergency CT
scan of the brain. Perhaps the patient has a history of migraine headaches
for years and has had a normal CT scan in the past. Perhaps the patient fell
from a ladder and briefly lost consciousness. Perhaps the patient has had
escalating headaches for several weeks that are now unbearable. The doctor
must decide, based on an understanding of the patient's complaints, how to
direct the physical examination and what tests to order to confirm or rule
out a life-threatening condition.
Most brain tumor
patients have symptoms that may have initially been attributed to other
illnesses, depression, or stress. For example, it is common for patients to
complain of fatigue, a lack of concentration, or nausea. These
nonlocalizing symptoms may be easily dismissed by patients and their
doctors. Localizing symptoms, such as speech disturbance, weakness of
one side of the body, or seizures are more likely to raise the suspicion of
a neurological problem.
After taking a careful
history, sometimes the doctor can estimate the location of the tumor and its
growth rate. The doctor will need to determine whether there is a previous
history of cancer because of the possibility of brain metastases. The doctor
will also note if there is a family history of cancer or brain tumor, and if
the patient has other inherited conditions that may impact on the patient’s
care, such as bleeding disorders, diabetes, and heart disease.
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9. What is a
neurological examination?
The part of the
physical examination called the neurological examination actually
begins before the patient is aware of it. From the time the patient walks
into the examination room, the doctor observes his balance, rhythm, and
coordination. During the history, the doctor will observe the patient's
speech patterns, whether there is hesitation, difficulty finding words,
misuse of words, or slurring of words. Even the patient's eye movements can
suggest whether there is a neurological problem.
A neurological
examination may be as simple as testing strength, sensation, and
coordination in the emergency room setting, or testing a series of more
complex physical and memory tasks in the neurologist's office. A complete
neurological examination evaluates the following:
1. Appearance
and behavior: Is the patient appropriate for the situation?
2. Standing
and walking: Is there imbalance or jerking of movement?
3. Level of
consciousness: Is the patient fully alert?
4. Orientation
to time and place: Does the patient know the year, month, day, and where
he is at that
moment?
5. General
intellectual function: Does the patient misinterpret clues from the
environment or seem
confused?
6. Memory:
Can the patient remember three unrelated words for five minutes?
7. Speech:
Can the patient understand and respond to spoken language?
8. Cranial
nerve functions:
I. Can the patient smell?
II. Can the patient see with each eye separately?
III. Can the patient look up, down, and raise the eyelids?
IV. Can the patient look down and toward the nose?
V. Can the patient open the jaw? Does the patient have sensation on both
sides of the face?
VI. Can the patient move the eyes to the right and left?
VII. Can the patient close the eyes tightly and smile symmetrically?
VIII. Can the patient hear with each ear?
IX.
and
X. Can the patient swallow?
XI. Can the patient shrug his shoulders?
XII. Can the patient stick his tongue out?
9. Motor
function: Does the patient have normal muscle power in all extremities?
10. Reflexes:
Are the patient's reflexes symmetrical?
11.
Coordination: Can the patient touch his finger to his nose rapidly and
accurately?
12. Sensation: Can the patient perceive light touch, temperature, position, vibration,
and pain?
Some doctors use the Mini-Mental Status
Examination, which is a set of questions and tasks that can be easily
administered in the office or hospital setting. The Mini-Mental Status
Examination tests orientation, memory, calculation, language, and figure
drawing on a 30 point scale. The test results are kept with the patient's
chart to determine whether there has been a change in the patient's status
over time.
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10. After my surgery,
my surgeon told me that I have a brain tumor but says he can't tell me more
about it until the pathology report is completed. What is a pathology report
and why is it so important?
Because there are many
different types of brain tumors, your surgeon wants to give you as much
detailed information as possible about the kind of tumor you have and the
treatment you may need. The doctor cannot do that until a pathologist
examines the biopsy taken at surgery. Some tumors can be evaluated
within a few minutes, using thin slices of frozen tumor. However, most
tumors require processing over a period of several hours. During processing,
the water is removed from the specimen. Eventually, tiny pieces of the tumor
are embedded in paraffin wax. These small slabs of paraffin-embedded tissue
are thinly sliced, placed on microscope slides, and stained with special
chemicals that color cell proteins and DNA. These are called permanent
sections, and their detail provides the pathologist the most complete
information about the tumor.
The pathologist
provides information about the cells that make up the tumor, and identify if
the cells are native to the brain (a primary tumor) or if they have spread
to the brain from another location in the body (a metastatic tumor). The
pathologist determines whether the tumor is benign or malignant. The growth
rate, or proliferation index, can also be obtained from the biopsy
specimen, using special stains.
In some cases, the
pathologist may confer with other colleagues or send the slides to a
neuropathologist, a pathologist who specializes in the diseases of the
nervous system. Difficult cases may take several days of study to determine
the exact nature of the tumor. The final diagnosis is written in the
pathology report. The importance of this document cannot be overstated.
Determining eligibility for clinical trials, the need for further treatment
such as radiation therapy or chemotherapy, and the eligibility for insurance
or disability benefits are but a few of the reasons why the pathology report
is the single most important document to the brain tumor patient (See
Question 13).
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11. What are
the most common types of primary brain tumors?
Table 3 lists the most
common types of primary brain tumors in children and adults. In both
children and adults, gliomas are the most common type of tumor; however,
adults have a higher proportion of malignant, or high grade glioma, and
children have a higher proportion of slower growing, low grade glioma. In
addition, adults tend to have tumors of the cerebral hemispheres and
children more commonly have tumors of the cerebellum and brainstem. Some
tumor types, such as primitive neuroectodermal tumor (PNET), medulloblastoma,
and ependymoma, are much more common in children; meningiomas are much more
common in adults. Color plates 1-4 show four types of common tumors
as seen under the microscope.
Table 3:
Common
Types of Primary Brain Tumors in Adults and Children
|
CNS Tumors in Adults |
|
CNS Tumors in Children |
|
|
Glioblastoma multiforme and Anaplastic astrocytoma
Meningiomas
Low grade gliomas and brain stem gliomas
Pituitary adenoma
Acoustic neuroma
Other
|
35%
25%
12%
10%
7%
11%
|
Low grade gliomas and brain stem glioma
Primitive neuroectodermal tumors (PNET) and medulloblastoma
Ependymoma
Pineal region tumors, including germinoma
Glioblastoma and anaplastic astrocytoma
Others |
44%
24%
10%
6%
4%
12% |
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12. The surgeon
explained to me that I have a fast-growing type of tumor, but I think my
symptoms have been present for a long time. Is this possible? Should I get a
second opinion from another pathologist to make sure the diagnosis is
correct? How do I do this?
It is possible that
you have a slow-growing tumor that evolved into a faster-growing type. The
pathologist may have only seen the faster-growing component in the tumor
specimen studied. Not surprisingly, the treatment recommended for patients
with such mixed tumors depends on the more aggressive component.
A second review of
your pathology slides can be helpful, but pathologists often confer with
others in the same hospital or send the slides to a colleague for further
review. It is possible that your slides have been reviewed by a handful of
pathologists before the final report is issued. Nevertheless, an additional
review — even if the same pathologist does it — may be helpful if you
provide clinical information, such as what type of symptoms you have and how
long your symptoms have persisted. It is also beneficial if you forward the
pathologist a copy of your MRI.
Getting a second
opinion about your pathology slides is particularly important if a different
diagnosis will have an impact on your treatment and prognosis. You may ask
your surgeon if he recommends sending the slides to another laboratory or
research center that specializes in brain tumors. If you plan to participate
in a clinical trial, another review of your pathology slides by a
neuropathologist associated with the trial is often required.
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13. My doctor says
that my tumor has a low proliferation index, and that I may not need
treatment right away. What is the proliferation index, and how does it
determine my treatment?
The proliferation
index is determined by testing some of the brain tumor sample using a
special stain for MIB-1 or Ki-67. The proliferation index is the number of
cells involved in the process of cell division (the process that produces
new tumor cells) in relation to the total number of cells. Slow-growing
tumors have few dividing cells, meaning they have a low proliferation index
(sometimes less than 1%). Tumors that grow more rapidly may have
proliferation indices exceeding 20%.Tumors with a low proliferation index
grow relatively slowly and even without treatment, may not appear to have
observable growth on an MRI over many months. Tumors with a high
proliferation index may double in size over a period of a few weeks.
The decision to treat
a slow-growing tumor is often based on the type of symptoms that it causes,
its location, and the amount of residual tumor present after surgery. For
patients who have slow-growing tumors with very little residual disease, the
risk of radiation therapy, chemotherapy, and other forms of treatment may
outweigh the potential benefit, particularly if the patient does not have
symptoms. Your doctor may recommend careful follow-up with MRI and regular
neurological examinations.
Feature: How
to Read Your Pathology Report
1. The
hospital or laboratory that produces the report, usually where the surgery
was performed
2. Patient's
name, hospital or medical record number, and date of birth
3. The unique
number assigned to this case; the reference number to locate slides in the
future
4. The surgeon
submitting the specimen
5. The
impression of the surgeon before diagnosis, often the reason for the
procedure
6. The
surgeon's notes on the origin of the specimen
7. The final
diagnosis may appear before or after the more detailed descriptions of the
specimen
8. Preliminary
diagnosis by "frozen section" is sometimes helpful to guide the surgeon who
must decide if he should attempt to remove more tumor.
9. The
dimensions and appearance of the tissue received
10. This is the
description of the tumor cells themselves. Key phrases include:
a. Infiltrating neoplasm: Tumor cells that blend into the normal brain
without a clear separation.
b. Oligodendroglial differentiation: Based on the descriptions
preceding this phrase, the
pathologist comments about the probable origin of the tumor. In this case,
the evidence
suggests that the tumor derived from oligodendrocytes, rather than
astrocytes, ependymal
cells, etc.
c. Focal hemorrhage: Small collections of blood within the tumor.
d. Necrosis: Literally means dead cells. Often, tumors that have a
rapid rate of growth show
these areas that have not had sufficient blood supply to allow continued
growth.
e. Heightened cellularity:
The increased density of cells in a specific area, compared with what
would normally be expected.
f. Pleomorphism: Literally means many forms, depicting a
variety of cell sizes and shapes.
g. Vascular proliferation: An increase in the number of cells lining the
walls of blood vessels of the tumor.
h. Mitotic activity: The activity of cells that are dividing; often seen
as dark, irregularly-shaped nuclei.
i. Anaplastic: Growing without structure or form; not resembling orderly
normal tissue.
11. MIB-1
staining determines how many cells in a specimen are synthesizing DNA in
preparation of cell division; it provides an estimate for the rate of tumor
growth.
12. The
pathologist who reviews the slides and determines the final diagnosis
St. Mark's Medical Center1
Fordham Ridge,
Massachusetts
Sayers, Brian F.2
Specimen No. S02-8879993
MR No. 393898882
Procedure Date: 7/25/02
DOB: 6/26/52
Date of Report: 7/26/02
Physician: John Heiss, M.D.4
CLINICAL HISTORY: Right frontal brain tumor5
DESCRIPTION OF SPECIMEN: Brain, right frontal mass6
FINAL DIAGNOSIS: Anaplastic oligodendroglioma7
INTRAOPERATIVE CONSULT:8
An intraoperative consultation with frozen section is diagnosed as marked
edema, favor low-grade glioma; defer final diagnosis to permanent sections.
Conveyed to Dr. Heiss on 7/25/02 by Dr. Sandor.
GROSS DESCRIPTION:9
Specimen A is received fresh for frozen section labeled "right frontal brain
mass" and consists of several soft, tan-gray irregular fragments of tissue
which measure 0.8 ´
0.4 ´ 0.3 cm in aggregate. The entire specimen is
submitted.
Specimen B is received fresh labeled "right frontal brain mass" and consists
of a 3.5 ´
3.8 ´ 2.2 cm soft, gray to gray-white wedge shaped
portion of brain tissue. The entire specimen is submitted.
MICROSCOPIC DESCRIPTION:10
Sections reveal an infiltrating neoplasm dominated by cells with generally
rounded nuclei, variably developed perinuclear halos, microcystic change,
and a delicate, branching capillary network consistent, in aggregate, with
oligodendroglial differentiation. There is focal hemorrhage present, but no
frank necrosis is seen. Although much of the lesion appears low-grade, the
neoplasm contains areas of heightened cellularity, pleomorphism, early
vascular proliferation, and mitotic activity, with up to 2 mitotic figures
per high power field, commensurate with an anaplastic (Grade III) lesion.
Immunohistochemical stains reveal a moderate to high MIB-1 labeling index,
with labeling of approximately 10% of the neoplastic cells in the more
active areas, also commensurate with an anaplastic grade lesion.11
Dr. Sandor and Dr. Patronas have reviewed the slides and agree with the
final diagnosis.
Signed by: Veronica Besst, M.D.12
Entered: 7/26/02 1530
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14. Will my tumor
spread to another part of my body? Can it spread to another part of my brain
or spinal cord?
Metastatic tumors
originate in another cancer of the body, such as the breast, lung, or
kidney. They may spread to multiple sites, including the brain. In contrast,
primary brain tumors that originate in the brain, such as gliomas, rarely
spread to other organs of the body. Some rare primary brain tumors that do
metastasize include pituitary carcinoma, hemangiopericytoma, and papillary
meningioma.
Primary or metastatic
brain tumors may spread through the CNS through the spinal fluid, sometimes
causing symptoms such as back pain, weakness, or numbness. Leptomeningeal
metastases may be seen on MRI as a coating around the brain or spinal
cord. The MRI may also show tiny tumors in the lower spinal cord called
“drop” metastases. Some common systemic cancers associated with
leptomeningeal metastases include breast cancer, small cell lung cancer, and
melanoma. Primary brain tumors that can spread throughout the spinal fluid
include primary CNS lymphoma, germ cell tumors, and medulloblastoma.
Some primary brain
tumors have multiple sites in the brain at diagnosis and are termed
multifocal.. CNS lymphoma, germinomas, and, less commonly, malignant
gliomas can appear at multiple sites in the brain. Biopsy of at least one
area is necessary to distinguish between a multifocal primary tumor and a
metastatic tumor because treatment recommendations for these tumor types may
differ.
M.L's.comment:
I had no idea how
important the pathology report was. Although the actual report wasn't
reviewed with me, my neurosurgeon did discuss the specifics of it. He
indicated that the pathology report revealed that I had an
oligodendroglioma. However, after my neurosurgeon received the analysis he
sent it back to be re-tested because he thought that what he removed looked
like something other than an oligo. Nevertheless, the test came back again
with the same result. Clearly, an accurate diagnosis is extremely important
to my neurosurgeon because the recommended treatment hinges on the
interpretation of the pathology report. A copy of the pathology report was
one of the first documents that I had to send to my disability advisor after
my surgery so that I could apply for temporary disability.
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