Bone Cancer

What Is Cancer?

Cancer is a group of many related diseases. All forms of cancer involve out-of-control growth
and spread of abnormal cells.

Normal body cells grow, divide, and die in an orderly fashion. During the early years of a
person's life, normal cells divide more rapidly until the person becomes an adult. After that,
normal cells of most tissues divide only to replace worn-out or dying cells and to repair injuries.

Cancer cells, however, continue to grow and divide, and can spread to other parts of the body.
These cells accumulate and form tumors (lumps) that may compress, invade, and destroy normal
tissue. If cells break away from such a tumor, they can travel through the bloodstream, or the
lymph system to other areas of the body. There, they may settle and form "colony" tumors. In
their new location, the cancer cells continue growing. The spread of a tumor to a new site is
called metastasis. When cancer spreads, though, it is still named after the part of the body where
it started. For example, if prostate cancer spreads to the bones, it is still prostate cancer, and if
breast cancer spreads to the lungs it is still called breast cancer.

Leukemia, a form of cancer, does not usually form a tumor. Instead, these cancer cells involve
the blood and blood-forming organs (bone marrow, lymphatic system, and spleen), and circulate
through other tissues where they can accumulate.

It is important to realize that not all tumors are cancerous. Benign (noncancerous) tumors do not
metastasize and, with very rare exceptions, are not life-threatening.

Cancer is classified by the part of the body in which it began, and by its appearance under a
microscope. Different types of cancer vary in their rates of growth, patterns of spread, and
responses to different types of treatment. That's why people with cancer need treatment that is
aimed at their specific form of the disease.

In America, half of all men and one-third of all women will develop cancer during their
lifetimes. Today, millions of people are living with cancer or have been cured of the disease. The
risk of developing most types of cancer can be reduced by changes in a person's lifestyle, for
example, by quitting smoking or eating a better diet. The sooner a cancer is found, and the
sooner treatment begins, the better a patient's chances are of a cure.
What Is Bone Cancer?

Normal Bone Tissue

Bone is the supporting framework of the body. Most bones are hollow. The outer part of bones
consists of fibrous tissue called matrix onto which calcium salts are deposited. At each end of the
bone is a zone of cartilage, a softer form of bone-like tissue. Cartilage consists of a fibrous tissue
matrix mixed with a gel-like substance. Unlike bone, cartilage does not contain calcium.
Cartilage acts as a cushion between bones and, together with ligaments and some other tissues,
forms the joints between bones. The bone itself is very hard and strong. Some bone is able to
support as much as 12,000 pounds per square inch. It takes as much as 1200 to 1800 pounds of
pressure to break a femur (thigh bone).

The outside of the bone is covered with a layer of fibrous tissue called periosteum. The bone
itself contains two kinds of cells. The osteoblast is the cell responsible for forming bone and the
osteoclast is the cell responsible for dissolving bone. Although bone looks to be a very
unchanging organ, the truth is that it is very active. New bone is constantly forming, and old
bone dissolving.

Bone marrow is the soft tissue inside the hollow bones. The marrow of some bones consists only
of fatty tissue. The marrow of other bones is a mixture of fat cells and hematopoietic (blood-
forming) cells. These blood-forming cells produce red blood cells, white blood cells, and blood
platelets. There are some other cells in the marrow such as plasma cells, fibroblasts, and
reticuloendothelial cells.

Secondary and Primary Bone Cancers

There are really two main types of bone cancer. One type is cancer that has spread to the bone
from other organs. This is called metastati or secondary bone cancer. The cancer did not start in
the bone. A typical example is when lung cancer spreads to the bones. The cells of the cancer
look like lung cancer cells, not bone cancer cells, even after they have spread from the lungs to
the bones. If you are interested in reading about metastatic bone cancer, please refer to the ACS
document on Bone Metastases and the ACS documents on the specific place where the cancer
started, (Breast Cancer, Lung Cancer, Prostate Cancer, etc.).

This essay is on primary bone cancer. That means the cancer started in the bone.

Types of Primary Bone Cancers

There are several different types of bone tumors. Their names are based on the area of bone or
surrounding tissue that is affected, and the kind of cells forming the tumor. Some primary bone
tumors are benign (not cancerous) and others are malignant (cancerous). Most bone cancers are
called sarcomas. Sarcomas are cancers that mostly develop from bone, cartilage, muscle, fibrous
tissue, fatty tissue, or nerve tissue.
Benign bone tumors: Benign bone tumors do not spread to other tissues and organs, and are not
life-threatening. They are generally cured by surgery. Types of benign bone tumors include
osteoma, osteoid osteoma, osteoblastoma, osteochondroma, hemangioma, and chondromyxoid
fibroma. These benign tumors are not discussed further in the remainder of this document, which
is limited to bone cancers.

Osteosarcoma: Osteosarcoma (also called osteogenic sarcoma) is a cancerous tumor of the bone
itself, and is the most common primary bone cancer. Although osteosarcoma most often occurs
in young people between age 10 and 30, about 10% of cases develop in people in their 60’s and
70’s. This cancer is rare during middle age. More males than females get this cancer. These
tumors develop most often in bones of the arms, legs and pelvis. For more information look at
the ACS document on osteosarcoma.

Chondrosarcoma: This is a cancer of cartilage cells, and is the second most common primary
bone cancer. This cancer is uncommon in people younger than 20. After age 20, the risk of
developing chondrosarcoma continues to rise until reaching about 75 years. Men and women are
equally likely to get this cancer. Although this cancer usually occurs in bones of the arms, legs
and pelvis, the ribs and some other bones are occasionally affected. Although this cancer usually
develops from normal cartilage, it may also form within benign tumors of cartilage and bone
called osteochondromas.

Ewing's tumor: This cancer (also called Ewing's sarcoma) is named after Dr. James Ewing, the
doctor who first described it in 1921. Ewing's tumors usually but not always develop in bones,
and less than 10 percent develop in other tissues and organs. Ewing's tumors that start in bones
most often arise in the long bones of the legs and arms, but may also develop in the pelvis and
other bones. Ewing's tumor is the third most common primary bone cancer. Unlike
osteosarcoma, Ewing's tumors of bone form in the cavity of the bone. This cancer usually
appears in children and adolescents and is uncommon in adults over 30 years old. Ewing's
tumors occur most frequently in the white population and is rare among African-Americans and
Asian-Americans. For more information look at the ACS document on Ewing’s tumors.

Fibrosarcoma and malignant fibrous histiocytoma: These cancers usually develop from "soft
tissues" (types of connective tissues other than bone, such as ligaments, tendons, fat, and muscle)
and rarely start in bones. These cancers usually occur in elderly and middle aged adults. Bones
most often affected include those of the legs, arms, and jaw. For more information look at the
ACS document on Sarcoma -- Adult Soft Tissue Cancer.

Giant cell tumor of bone: This type of primary bone tumor has benign and malignant forms.
The benign form is most common. Only about 10 percent of giant cell bone tumors are cancerous
and spread to other parts of the body, but they often recur locally after surgery. These tumors
typically affect the arm or leg bones of young and middle-aged adults.

Chordoma: This primary tumor of bone usually occurs in the base of the skull and bones of
the spine. Surgery and radiation therapy are difficult because of the nearby spinal cord and
nerves that may be involved. Long term follow-up is important because these tumors can come
back, even ten or more years after treatment.
Other Cancers that Develop in Bones But Are Not Discussed Further in This Document

Lymphoma: Non-Hodgkin's lymphomas generally develop in lymph nodes, but occasionally

start in the bone. Primary non-Hodgkin's lymphoma of bone is considered a widespread disease,
because multiple sites in the body are usually involved. The outlook and treatment are similar to
other non-Hodgkin's lymphomas of the same subtype and stage. Treatment is similar to
lymphomas that start in lymph nodes and different from primary bone sarcomas. For more
information, read the ACS document on Non-Hodgkin's Lymphoma.

Multiple myeloma: Although multiple myeloma almost always starts in bones, doctors do not
consider this a bone cancer because it develops from plasma cells of the bone marrow. Although
it causes bone destruction, it is no more a bone cancer than is leukemia. It is treated as a
widespread disease. Occasionally, myeloma can be first found as a single tumor in a single bone,
but most of the time it will go on to spread to the marrow of other bones. For more information,
see the ACS document on Multiple myeloma.

] What Are The Key Statistics For Bone Cancer?

In 2001, about 2,900 new cases of cancer of the bones and joints will be diagnosed, and about
1,400 deaths from these cancers are expected. Primary cancers of bones account for less than 0.2
percent of all cancers.

Osteosarcoma is the most common primary bone cancer (35% of cases), followed by
chondrosarcoma (26%), Ewing's tumor (16%), chordoma (8%), and malignant fibrous
histiocytoma/fibrosarcoma (6%). Several rare types of cancers account for the remainder of
cases.

The prognosis for people with primary bone cancer varies greatly, depending on the specific type
of cancer and how far it has spread. If you have questions about your personal chances of cure of
bone cancer, or how long you might survive such a cancer, talk with the people who know your
unique circumstances best - your cancer care team.

What Are The Risk Factors For Bone Cancer?

A risk factor is anything that increases a person's chance of getting a disease such as cancer.
Different cancers have different risk factors. For example, smoking is a risk factor for cancers of
the lung, larynx, mouth, kidneys, and many other organs. Unprotected exposure to strong
sunlight is a risk factor for skin cancer. Scientists have found several risk factors that make a
person more likely to develop stomach cancer. Most people with one or more risk factors never
develop a cancer. Most people with bone cancers do not have any apparent risk factors.

Inherited genes: A very small number of bone cancers (especially osteosarcomas) appear to
have a hereditary basis. Children with certain rare inherited cancer syndromes have an increased
risk of developing osteosarcoma. The Li-Fraumeni syndrome makes people much more likely to
develop several types of cancer, including breast cancer, brain cancer, osteosarcoma, and other
types of sarcoma. Most cases are caused by a mutation of the p53 tumor suppressor gene.

Retinoblastoma is a rare eye cancer of children. Between 6% and 10% of cases are due to an
inherited tendency to develop this cancer. Children with this inherited form of retinoblastoma
also have an increased risk for developing osteosarcoma. This is due to the fact they have an
abnormal mutation of the retinoblastoma gene that predisposes them to developing cancer. Also,
radiation therapy for treating children with retinoblastoma increases their risk of osteosarcoma in
the bones of the skull.

Paget's disease: Paget's disease is a benign but precancerous condition that affects one or more
bones. This is mostly a disease of people older than 50, which results in formation of abnormal
bone tissue. Affected bones are heavy and thick yet weaker than normal bones and more likely to
fracture (break). Usually this condition is not life threatening. Bone sarcomas (usually
osteosarcoma) develop in about 5% to 10% of severe cases of Paget's disease, usually when
many bones are affected.

Multiple exostoses: Having multiple exostoses (overgrowths of bone tissue) increases a person's
risk of developing osteosarcoma.

Multiple osteochondromas: Osteochondromas are benign bone tumors formed by bone and
cartilage. Each osteochondroma has a very slight risk of developing into an osteosarcoma. Most
osteochondromas are cured by surgery. However, some people inherit a tendency to develop
many osteochondromas. Patients with multiple osteochondromas have an increased risk for
osteosarcoma because so many osteochondromas are present and because it may not be possible
to remove them all.

Multiple enchondromas: People with several of these benign cartilage tumors are at higher risk
for developing a chondrosarcoma than are people without such tumors. However, the risk that an
enchondroma will turn into a chondrosarcoma is quite low.

Radiation: Bone exposure to radiation may also increase the risk of developing bone cancer. A
typical x-ray of a bone is not dangerous, but exposure to large doses of radiation (for example,
radiation therapy to treat another cancer) does pose a risk. Being treated at a younger age and/or
being treated with higher doses of radiation (usually over 60 Gy) both increase the risk of
developing bone cancer. Exposure to radioactive materials such as radium and strontium may
cause bone cancer because these minerals accumulate in bones. Nonionizing radiation, such as
microwaves and electromagnetic fields from power lines, cellular phones, and household
appliances have not been convincingly linked to bone cancer risk.

Injuries: People have wondered whether injury to a bone can cause cancer, but this has never
been proven. Many people with bone cancer remember having hurt that part of their bone. Most
doctors feel this did not cause the cancer, but that the cancer caused them to remember the
incident, or that the injury drew their attention to that bone and caused them to notice a mass that
had already been present for some time.
Do We Know What Causes Bone Cancer?

The exact cause of most bone cancers is not known. Scientists, however, have found that bone
cancers are associated with a number of other conditions, which are described in the section on
risk factors. It is important to remember that most people with bone cancers do not have any
known risk factors and the causes of their cancers remain unknown at this time. Research is
under way to learn more about these causes.

During the past few years, scientists have made great progress in understanding how certain
changes in a person's DNA can cause bone cells to become cancerous. DNA carries the
instructions for nearly everything our cells do. We usually resemble our parents because they are
the source of our DNA. However, DNA affects more than our outward appearance. It influences
our risks for developing certain diseases, including some kinds of cancer.

Some genes (parts of our DNA) contain instructions for controlling when our cells grow and
divide. Genes that promote cell division are called oncogenes. Others that slow down cell
division or cause cells to die at the right time are called tumor suppressor genes. It is known that
cancers can be caused by DNA mutations (defects) that activate oncogenes or inactivate tumor
suppressor genes. Some people with cancer have DNA mutations they inherited from a parent.
These mutations increase their risk for the disease. Usually, however, DNA mutations are
acquired during life rather than inherited before birth.

The DNA mutations that cause some inherited forms of bone cancers are known. The Li-
Fraumeni syndrome is caused by inherited mutations that inactivate the p53 tumor suppressor
gene. This results in a very high risk of developing one or more types of cancer that include
breast cancer, brain cancer, osteosarcoma, and other sarcomas. Inherited defects of the Rb tumor
suppressor gene increase the risk of developing retinoblastoma, a type of eye cancer that affects
children. Children with this defect also have an increased risk for developing osteosarcoma.

When children inherit Rb or p53 mutations from a parent, these mutations are present in every
cell of their body and, therefore, can be detected by testing DNA of blood cells. Because every
person has two p53 and two Rb genes but passes only one of each to their children (the other
gene comes from their mate) the odds that a parent will pass their mutated gene on to a child are
1 out of 2.

The majority of bone cancers are not caused by inherited DNA mutations. They are the result of
mutations acquired during the person's lifetime. These mutations are present only in the cancer
cells and are not passed on to the patient's children. Although radiation is very useful in treating
some forms of cancer, it can also cause cancer by damaging DNA. This is why bones exposed to
radiation as a treatment for other cancers are more likely to develop bone cancer later in the
patient's lifetime.

Other DNA mutations have no apparent cause, but may result from random errors that occur
when cells reproduce. Before a cell divides, it must copy its DNA so that both new cells have the
same set of instructions. Sometimes this copying process is not completely accurate. Scientists
still do not know exactly why or how these mutations happen to some people but not to others.
Fortunately, cells have ways of "proofreading" DNA copies and repairing any errors. When cells
divide shortly after their DNA is damaged, new "daughter cells" may be formed before the
original cell has time to repair its DNA damage. Once the cells are formed, it is too late to repair
the damage. The result is that cell instructions for growth control can be permanently altered, and
a cancer (such as osteosarcoma) may develop. This is why normal situations (such as the teenage
growth spurt) and diseases (such as Paget's disease of bone, multiple exostoses, and multiple
osteochondromas) causing rapid bone growth increase the risk of developing osteosarcoma.

Although scientists are making progress in understanding this process, there are still some points
that are not completely understood. It is hoped that a more complete understanding will help in
developing ways to better prevent and treat bone cancers.

Can Bone Cancer Be Prevented?

Changes in lifestyle can help prevent many types of cancer. At present, however, there are no
known lifestyle changes that can prevent bone cancers.

Can Bone Cancer Be Found Early?

Tests are routinely used to detect early stages of some types of cancer (such as breast, cervical,
colorectal, and skin cancer) before they cause symptoms. At this time, there are no special tests
that are routinely recommended for early detection of bone cancers. The best strategy for early
diagnosis is prompt attention to the signs and symptoms of this disease.

Signs and Symptoms of Bone Cancer

Pain: Pain in the affected bone is the most common complaint of patients with bone cancer. At
first, the pain is not constant and may be worse at night or when the bone is used (for example
leg pain when walking). As the cancer grows, the pain will be there all the time. The pain
increases with activity and may result in a limp if a leg is involved.

Swelling: Swelling in the area of the pain may not occur until weeks later. Depending on the
location of the tumor, it may be possible to feel a lump or mass.

Fractures: Although bone cancer may weaken the bone it develops in, the bones do not usually
fracture (break). Patients with a fracture next to or through a bone cancer usually describe a limb
that was sore for a few months and suddenly became severely painful.

Generalized symptoms: If the cancer has spread, there may be generalized symptoms such as
weight loss and fatigue. Also, spread of the cancer to internal organs may cause symptoms. For
example, if the cancer spreads to the lung, the person may have trouble breathing.

Bone pain and/or swelling are more often due to benign conditions (such as injuries and arthritis)
than to cancers, so most people with these conditions do not have cancer. Nonetheless, if these
signs and symptoms persist for a long time, and do not appear to be due to a benign condition,
medical tests to look for a cancer should be done.
How Is Bone Cancer Diagnosed?

A patient's symptoms (described in the section on "Can Bone Cancer Be Found Early?"),
physical examination, imaging tests, and blood tests may suggest that bone cancer is present.
However, in most cases, this suspicion must be confirmed by examination of a tissue or cell
sample under a microscope. Other diseases, such as bone infections, can cause symptoms and
imaging results that could be confused with bone cancer. In most cases, a biopsy is necessary to
diagnose a patient's first bone metastasis, but additional bone metastases can usually be
diagnosed based on x-rays and other imaging tests.

Imaging Tests to Detect Bone Cancer

X-rays: Most bone cancers will show up on x-rays of the bone. The bone at the site of the cancer
may appear "ragged" instead of solid. The cancer may appear as a hole in the bone and there may
be a mass around the defect in the bone, which may extend into nearby tissues (such as muscle or
fat). The radiologist (doctor who specializes in reading x-rays) can often tell if a tumor is
malignant by its x-ray appearance.

Computed tomography: Commonly known as a CT or CAT scan, this test uses a rotating x-ray
beam to create a series of pictures of the body from many angles. A computer combines the
information from all the pictures to produce a detailed cross-sectional image. To highlight details
on the CT scan, a harmless dye may be injected into a vein before the x-rays are taken. CT scans
are often useful in diagnosing cancer in bones in the shoulder, pelvis, and spine.

Magnetic resonance imaging (MRI): This procedure uses large magnets and radio waves
instead of radiation to produce computer-generated pictures of internal organs. The pictures look
very similar to those of a CT scan, but are more detailed. MRI is especially useful in determining
whether cancer of bones of the spine is causing damage to the spinal cord. Because MRI images
clearly show soft tissues (muscle, fat, etc.) next to bones, they sometimes help surgeons in
planning exactly how they will approach the operation, and how much tissue next to the bone
must be removed.

Radionuclide bone scan: This procedure helps show if a cancer has metastasized to other bones.
It also can show the extent of damage the cancer has caused in the bone. The patient receives an
injection of radioactive material called technetium diphosphonate. The amount of radioactivity
used is very low and causes no long term effects. The radioactive substance is attracted to
diseased bone cells throughout the entire skeleton. Areas of diseased bone will be seen on the
bone scan image as dense, gray to black areas, called "hot spots." These areas may suggest
metastatic cancer is present, but arthritis, infection, or other bone diseases can also cause a
similar pattern. To distinguish among these conditions, the cancer care team may use other
imaging tests or take bone biopsies. Bone scans can find metastases earlier than regular x-rays.
They are useful in initially finding bone metastases as well as outlining the extent of the primary
cancer.
Biopsy

A biopsy (tissue sample) of the tumor or cancerous area must be taken for study under a
microscope to confirm that the diagnosis is cancer, and not some other bone disease. If cancer is
present, the biopsy will help determine whether it is a primary bone cancer or a secondary bone
cancer (bone metastasis). Several types of tissue and cell samples are used to diagnose bone
cancer. It is very important that the biopsy procedure be performed by a surgeon with experience
in diagnosis and treatment of bone tumors. Taking a biopsy sample from the wrong area may
make it necessary for the surgeon to remove more tissue than would be otherwise necessary and
may limit options of limb-sparing surgery for tumors affecting the arms or legs.

Needle biopsy: These are done after "freezing" the skin with local anesthetic. There are two
types of needle biopsies: fine needle biopsies and core needle biopsies. Fine needle aspiration
(FNA) uses a very thin needle and a syringe to withdraw a small amount of fluid and small tissue
fragments from the tumor mass. The doctor can aim the needle at a suspicious tumor or area that
can be felt near the surface of the body. If the suspicious area cannot be felt because it is deep
inside the body, the needle can be guided while it is viewed by a computed tomography (CT)
scan. The needles used for a core biopsy are slightly larger than those used in FNA. They remove
a small cylinder of tissue (about 1/16 inch in diameter and 1/2 inch long).

Surgical bone biopsy: First the surgeon will anesthetize the skin and tissue above the tumor. For
larger procedures, or in young children, the surgeon may use general anesthesia. Then the
surgeon will cut through the skin to remove a small part of a large tumor (incisional biopsy).

How Are Bone Cancers Staged?

The staging process collects and summarizes information about how big the cancer is and
whether it has spread. This helps doctors determine the prognosis (the outlook for chances of
survival) for a patient and select the most appropriate treatment options.

Collecting the information needed for staging involves biopsies, imaging of the main tumor
(usually with CT or MRI scans), and in some cases imaging other parts of the body to look for
metastases.

The most common system used to stage cancer is the TNM system of the American Joint
Committee on Cancer (AJCC). T stands for the size of the tumor, N stands for spread to lymph
nodes (bean-sized collections of immune system cells found throughout the body that help fight
infections and cancers), and M is for metastasis (spread to distant organs). In some bone and soft
tissue sarcomas, an additional factor, called histologic grade or G, is used to stage the tumor. The
histologic grade is based on how the cancer cells appear under the microscope. To assign a stage,
information about the tumor, its grade, lymph nodes, and metastasis is combined by a process
called stage grouping. The stages are usually described by Roman numerals from I to IV with
the letters A or B. However, staging of bone cancer involves several exceptions to that general
rule. The AJCC system for bone cancer staging defines stages I, II, and IV but intentionally
omits stage III. Also, the AJCC system is not generally used for some types of childhood bone
cancers (osteosarcoma and Ewing's tumor). The most commonly used system for these cancers
use only two stages -- localized and metastatic. (Refer to American Cancer Society documents
on Osteosarcoma or Ewing's Tumors for more information about staging of these cancers.)

Histologic Grade

G1: Well differentiated - low grade (the least aggressive appearing under the microscope)
G2: Moderately differentiated - intermediate grade (intermediate between G1 and G3)
G3: Poorly differentiated - high grade (more aggressive appearing under the microscope)
G4: Undifferentiated - high grade (most aggressive appearing under the microscope)

T Categories

T1: the tumor is confined within the cortex (outer shell of the bone)
T2: the tumor invades beyond the cortex

N Categories

N0: no lymph nodes have cancer cells in them

N1: lymph nodes are present that have cancer cells in them (very rare with bone cancer)

M Categories

M0: no distant metastases (spread) of bone cancer found

M1: distant metastases are found

Stage Grouping for Bone Cancer

Stage IA: G1,2 T1 N0 M0

Stage IB: G1,2 T2 N0 M0

Stage IIA: G3,4 T1 N0 M0

Stage IIB: G3,4 T2 N0 M0

Stage III: Not Defined

Stage IVA: Any G, Any T, N1, M0

Stage IVB: Any G, Any T, Any N, M1

Five-year Survival Rates by Stage

This staging system is useful in estimating prognosis and selecting treatment for people with
bone cancers. The average five-year survival rates are highly correlated with the stage of the
cancer The 5-year survival rate refers to the percent of patients who live at least 5 years after
their cancer is diagnosed. Many of these patients live much longer than 5 years after diagnosis,
and 5-year rates are used to produce a standard way of discussing prognosis. Of course, these 5-
year survival rates are based on patients with bone cancer diagnosed and initially treated more
than 5 years ago. Improvements in treatment often result in a more favorable outlook for recently
diagnosed patients.
The five-year survival for patients in the different stages is listed below. These rates reflect all
types of primary bone cancer combined. In actual practice, doctors also consider the type of bone
cancer. Refer to American Cancer Society documents on Osteosarcoma or Ewing’s Tumors for
more information on survival rates of these cancers by stage.

Stage of Bone Cancer (all types combined) 5-year Survival Rate

Stage IA 80%
Stage IB 64%
Stage IIA 66%
Stage IIB 54%
Stage IVA 44%
Stage IVB 17%

How Are Bone Cancers Treated?

After a bone cancer is found and staged, the cancer care team will recommend a treatment plan.
This is an important decision, so it is important to take time and think about all of the choices. In
choosing a treatment plan, factors to consider include the type, location and stage of the cancer,
as well as the patient's overall physical health.

Because many bone cancers are rare, it is often a good idea to seek an opinion from a major
cancer center that may have a wide experience in treating bone cancers. A second opinion can
provide more information and help the patient feel more confident about the treatment plan that
is chosen. Some insurance companies require a second opinion before they will agree to pay for
treatments. The usual treatments for bone cancers are described below. More specific treatments
are described in the ACS documents on specific types of bone cancers, such as Osteosarcoma or
Ewing’s Tumor.

Surgery

Depending on the type and stage of the bone cancer, surgery may be used to remove the cancer
and some of the surrounding tissue. The goal of surgery is to remove the tumor and at least 2 to 3
centimeters (about 1 inch) of the tissue surrounding the tumor. When the tumor is in the pelvic
bone, this amount of surgery is more difficult because the tumor may be next to vital organs that
can't be surgically removed.

Just a decade or two ago, bone cancer often meant loss of the entire affected limb. Today, the
trend is away from such radical surgery. Large segments of the bone can be removed and
replaced with bone grafts, and/or metal plates to maintain function in the affected area or limb.
Often patients can be treated with limb-sparing surgery after they received chemotherapy and/or
radiation therapy to first shrink the tumor. Patients treated this way have the same overall
survival rates as those who have amputations. Currently, amputations are recommended only for
very specific situations.

When amputation is considered necessary for treatment, a prosthesis (artificial limb) is usually
fitted shortly after surgery. If bone cancer spreads to the lung, the lung cancer sometimes can be
successfully removed by surgery.

Radiation Therapy

Radiation therapy uses high-energy radiation to kill cancer cells.

External beam radiation therapy uses radiation delivered from outside the body that is focused on
the cancer. This is the type of radiation therapy most often used to treat bone cancer.

Brachytherapy uses small pellets or seeds of radioactive material placed directly into the cancer.
Brachytherapy may be used as the only form of radiation therapy or in combination with external
beam radiation.

Radiation therapy is sometimes used as the primary (main) treatment of bone cancers in some
patients, especially in those whose general health is too poor to undergo surgery. After surgery,
radiation can be used as an adjuvant (additional) therapy to kill very small clusters of cancer
cells that cannot be seen and removed during surgery. Radiation therapy can also be used to
palliate, or ease, symptoms of bone cancer. It is most commonly used as the main treatment for
Ewing's tumors. For the rest, surgery is the main treatment although radiation may be added as
well.

Side effects of radiation therapy may include mild skin problems or tiredness. These often go
away after a short while. Radiation may also make the side effects of chemotherapy worse.
Abdominal radiation therapy may cause nausea and diarrhea, while radiation to the chest may
cause lung damage and lead to difficulty breathing and shortness of breath. Radiation of large
areas of an arm or leg can cause swelling, pain, and weakness.

Chemotherapy

Chemotherapy uses anticancer drugs that are given into a vein or taken by mouth. These drugs
enter the bloodstream and reach all areas of the body, making this treatment useful for cancer
that has spread or metastasized to other organs. Depending on the type and stage of bone tumor,
chemotherapy may be given as the primary (main) treatment or as an adjuvant (additional)
treatment to surgery. Chemotherapy for bone tumors generally uses a combination of several
anticancer drugs. The most commonly used drugs are methotrexate (given in high doses along
with calcium leucovorin), doxorubicin, and cisplatinum. Often these are given immediately after
surgery (adjuvant) to destroy any cancer cells that might have already spread to other parts of the
body. Sometimes they are given before surgery (neoadjuvant). Some doctors will even give
chemotherapy into an artery leading to the tumor to shrink it so that surgery will be easier. Other
drugs are sometimes used, particularly in patients where the bone cancer has spread. These drugs
include vincristine, etoposide, ifosfamide and actinomycin D.
Chemotherapy drugs kill cancer cells but also damage some normal cells. Therefore, careful
attention must be given to avoiding or minimizing side effects. These would depend on the type
of drugs, the amount taken, and the length of treatment. Temporary side effects might include
nausea and vomiting, loss of appetite, loss of hair, and mouth sores. Because chemotherapy can
damage the blood-producing cells of the bone marrow, patients may have low blood cell counts.
This can result in an increased chance of infection (due to a shortage of white blood cells),
bleeding or bruising after minor cuts or injuries (due to a shortage of blood platelets), and anemia
(due to low red blood cell counts).

Most side effects disappear once treatment is stopped. There are treatments for many of the side
effects of chemotherapy. For example, antiemetic drugs can be given to prevent or reduce nausea
and vomiting.

Clinical Trials

Studies of promising new or experimental treatments in patients are known as clinical trials. A
clinical trial is only done when there is some reason to believe that the treatment being studied
may be of value to the patient. Treatments used in clinical trials are often found to have real
benefits. There are three phases of clinical trials in which a treatment is studied before the
treatment is eligible for approval by the FDA (Food and Drug Administration).

The purpose of a Phase I study is to find the best way to give a new treatment and how much of
it can be given safely. Physicians watch patients carefully for any harmful side effects. The
research treatment has been well tested in laboratory and animal studies, but the side effects in
patients are not completely predictable.

Phase II trials determine the effectiveness of a research treatment after safety has been evaluated
in a Phase I trial. Patients are closely observed for an anticancer effect by careful measurement
of cancer sites present at the beginning of the trial. In addition to monitoring patients for
response, any side effects are carefully recorded and assessed.

Phase III trials require entry of large numbers of patients. Some trials enroll thousands of
patients. One of the groups may receive standard (the most accepted) treatment, so the new
treatments can be directly compared. The group that receives the standard treatment is called the
"control group." For example, one group of patients (the control group) may receive the standard
chemotherapy for a certain type of cancer, while another patient group may receive a different
type of chemotherapy, that may or may not contain an investigational drug, to see if this
improves survival. All patients in Phase III trials are monitored closely for side effects, and
treatment is discontinued if the side effects are too severe.

Researchers conduct studies of new treatments to answer the following questions:

• Is the treatment likely to be helpful?
• Does this new type of treatment work?
• Does it work better than other treatments already available?
• What side effects does the treatment cause?
 • Do the benefits outweigh the risks, including side effects?
• In which patients is the treatment most likely to be helpful?

However, there are some risks. No one involved in the study knows in advance whether the
treatment will work or exactly what side effects will occur. That is what the study is designed to
discover. While most side effects will disappear in time, some can be permanent or even life-
threatening. Keep in mind that even standard treatments have side effects. Depending on many
factors, a patient may decide that a clinical trial will be beneficial.

Enrollment in any clinical trial is completely up to you. Your doctors and nurses will explain the
study to you in detail and will give you a form to read and sign indicating your desire to take
part. This process is known as giving your informed consent. Even after signing the form and
after the clinical trial begins, you are free to leave the study at any time, for any reason. Taking
part in the study does not prevent you from getting other medical care you may need.

To find out more about clinical trials, ask your cancer care team. Among the questions you
should ask are:
• What is the purpose of the study?
• What kinds of tests and treatments does the study involve?
• What does this treatment do?
• What is likely to happen in my case with, or without, this new research treatment?
• What are my other choices and their advantages and disadvantages?
• How could the study affect my daily life?
• What side effects can I expect from the study? Can the side effects be controlled?
• Will I have to be hospitalized? If so, how often and for how long?
• Will the study cost me anything? Will any of the treatment be free?
• If I am harmed as a result of the research, what treatment would I be entitled to?
• What type of long-term follow-up care is part of the study?
• Has the treatment been used to treat other types of cancers?

You can get a list of current clinical trials by calling the National Cancer Institute's Cancer
Information Service toll free at 1-800-4-CANCER or visiting the NCI clinical trials website for
patients (cancertrials.nci.nih.gov) or healthcare professionals
(cancernet.nci.nih.gov/prot/protsrch.shtml).

Complementary and Alternative Methods

If you are considering any alternative or complementary treatments, it is best to discuss this
openly with your cancer care team and request information from the American Cancer Society or
the National Cancer Institute. Some alternative treatments can interfere with standard medical
treatments or may cause serious side effects.
Treatment Choices by Stage of Bone Cancer

This section provides an overview of treatment of bone cancer or different stages. However, the
details of treatment will also depend on the type of bone cancer. For more detail on treatment of
osteosarcoma or Ewing’s tumor, refer to ACS documents on these cancers.

Stage I: Most patients with stage I bone cancer have their cancers surgically removed. When it is
not possible to remove the tumor plus at least two centimeters (about 1 inch) of normal tissue
around the tumor, radiation therapy is generally added after surgery. Sometimes radiation
therapy and/or chemotherapy are given before surgery to shrink the tumor and improve the
chance of completely removing it.

Stage II: Surgical removal of the tumor is still the goal with stage II tumors. Ideally, surgical
treatment involves removal of the tumor and several centimeters (over an inch) of normal tissue
around the tumor in all directions. It is even more likely that chemotherapy alone or with
radiation therapy will be used in addition to the surgery.

Stage IV: The usual treatment for stage IVA bone cancer is surgical removal of the primary
(main) tumor with a wide (over 1 inch) margin of normal tissue whenever possible, and surgical
removal of nearby lymph nodes, followed by chemotherapy and perhaps, radiation therapy.

Because stage IVB bone cancers have spread to distant organs, cure is usually not possible.
However, cure is sometimes possible for patients whose main tumor and all of their metastases
can be removed by surgery. Those patients’ primary (main) tumors should be treated as in stage
II, and metastases should be completely removed, if possible.

For patients whose primary tumor and all metastases cannot be completely removed by surgery,
palliative radiation therapy and/or chemotherapy is recommended.

What Should You Ask Your Doctor About Bone Cancer?

As noted earlier, it is important to have honest, open discussions with your cancer care team.
They want to answer all of your questions, no matter how trivial they might seem. For instance,
consider these questions:
• What kind of bone cancer do I have?
• Has my cancer spread beyond the primary site?
• What is the stage of my cancer and what does that mean in my case?
• What treatment choices do I have?
• What do you recommend and why?
• What risks or side effects are there to the treatments you suggest?
• What are the chances of recurrence of my cancer with these treatment plans?
• What should I do to be ready for treatment?
• Based on what you've learned about my cancer, how long do you think I'll survive?
In addition to these sample questions, be sure to write down some of your own. For instance, you
might want more information about recovery times so you can plan your work schedule. Or, you
may want to ask about second opinions or about clinical trials for which you may qualify.

What Happens After Treatment For Bone Cancer?

During and after treatment for your bone cancer, you may be able to hasten your recovery and
improve your quality of life by taking an active role. Learn about the benefits and disadvantages
of each of your treatment options, and ask questions of your cancer care team if there is anything
you do not understand. Learn about and watch for side effects of treatment, and report these
promptly to your cancer care team so they can take steps to minimize them and shorten their
duration.

Remember that your body is as unique as your personality and your fingerprints. Although
understanding your cancer's stage and learning about the effectiveness of your treatment options
can help predict what health problems you may face, no one can say precisely how you will
respond to cancer or its treatment.

You may have special strengths such as a history of excellent nutrition and physical activity, a
strong family support system, or a deep faith, and these strengths may make a difference in how
you respond to cancer. In fact, behavioral scientists have recently found that some people who
took advantage of a social support system, such as a cancer support group, survived with a better
quality of life. There are also experienced professionals in mental health services, social work
services and pastoral services who may assist you in coping with your illness.

You can also help in your own recovery from cancer by making healthy lifestyle choices. If you
use tobacco, stop now. Quitting will improve your overall health and the full return of the sense
of smell may help you enjoy a healthy diet during recovery. If you use alcohol, limit how much
you drink. Have no more than one or two drinks per day. Good nutrition can help you get better
after treatment. Eat a nutritious and balanced diet, with plenty of fruits, vegetables, and whole
grain foods. Ask your cancer care team if you could benefit from a special diet -- they may have
specific recommendations for people who have had abdominal radiation therapy.

If you are in treatment for cancer, be aware of the battle that is going on in your body. Radiation
therapy and chemotherapy add to the fatigue caused by the disease itself. Give your body the rest
it needs so you will feel better as time goes on. Exercise once you feel rested enough. Ask your
cancer care team whether your cancer or its treatments might limit your exercise program or
other activities.

Surgery and radiation therapy may sometimes affect a person's feelings about their body, and
may lead to specific physical problems that affect sexuality. Your cancer care team can help with
these issues, so don't hesitate to share your concerns.

A cancer diagnosis and its treatment are major life challenges, with an impact on you and
everyone who cares for you. Before you get to the point where you feel overwhelmed, consider
attending a local support group. If you need individual assistance in other ways, contact your
hospital's social service department or the American Cancer Society for help in locating
appropriate services.

Follow-up tests: Frequent physical examinations are done to detect tumor recurrence or side
effects of treatment as early as possible. Chest x-rays and other imaging studies (such as
ultrasound, CT scans, and MRI scans) may be done to watch for recurrence or metastasis. Most
local recurrences and many distant recurrences (metastases) can be effectively treated,
particularly when they are found early. The lungs are the most common site of distant recurrence
of sarcoma. Many lung recurrences can be surgically removed often resulting in improved
survival and sometimes in a cure.

New symptoms : It is important for the patient to report any new symptoms, such as cough, to
the doctor right away, since they may indicate cancer recurrences or side effects of treatment.

What's New In Bone Cancer Research And Treatment?

Basic Research

There has been much progress in understanding how certain changes in the DNA of soft tissue
cells cause bone cancers to develop. This information is already being applied to new tests for
diagnosis and classification of bone cancer. This is important, since accurate classification helps
doctors in selecting the most appropriate treatment. It is hoped that this information will soon
lead to new strategies for treating these cancers, based on specific differences between normal
and malignant soft tissue cells.

Chemotherapy

Active research is ongoing in chemotherapy for all cancers. This includes studies of new drugs
and new ways to give drugs now available. For example, clinical trials are now testing the value
of giving chemotherapy before surgery, or of injecting chemotherapy directly into the artery that
supplies blood to the arm or leg containing a bone cancer (regional chemotherapy).

Radiation Therapy

Studies of new radiation therapy methods are also in progress. For example, the roles of external
beam radiation and brachytherapy (internal radiation) are being clarified.

Immunotherapy

Experimental treatments that boost the patient's immune reaction to fight soft tissue sarcomas
more effectively are being tested. Some treatments use drugs like interleukin-2 that boost the
immune system in general. In active immunotherapy, the patient is given vaccines that might
cause the immune system to recognize some of the abnormal chemicals in sarcomas and kill
these cells. Passive immunotherapy uses antibodies made in the laboratory to seek out sarcoma
cells that contain certain abnormal cancer cell proteins. Often, toxins or radioactive atoms are
attached to these antibodies, so that the cell-killing chemicals or radiation are targeted
specifically to the cancer cells and do not attack the healthy cells of the body.

Are There Other National Organizations Addressing Bone Cancers?

The following organizations can also provide additional information and resources . *

National Cancer Institute

Telephone: 1-800-4-CANCER
Internet Addresses: www.nci.nih.gov and cancernet.nci.nih.gov [Link]

*Inclusion on this list does not imply endorsement by the American Cancer Society

Additional Resources

Additional American Cancer Society Information

After Diagnosis: A Guide for Patients and Families (Booklet; Code #9440)

Caregiving: A Step-By-Step Resource for Caring for the Person with Cancer at Home (Book;
Code #9422)

Caring for the Patient with Cancer at Home (Booklet; Code #4656)

Questions and Answers About Pain Control (Booklet; Code #4518)

Other Publications*

*Inclusion on this list does not imply endorsement by the American Cancer Society

A Cancer Survivor's Almanac: Charting Your Journey. Edited by Barbara Hoffman, JD. National
Coalition for Cancer Survivorship. Minnetonka, MN: Chronimed Publishing, 1996.

Dollinger, Malin, Ernest H. Rosenbaum, and Greg Cable. Everyone's Guide to Cancer Therapy.
Kansas City: MO: Somerville House Books, 1997.

Morra, Marion and Eve Potts. Choices. New York: Avon Books, 1994.

References

American Cancer Society. Cancer Facts and Figures 2001. Atlanta, Ga: American Cancer
Society; 2001.

American Joint Committee on Cancer. AJCC Cancer Staging Manual. 5th ed. Philadelphia, Pa:
Lippincott-Raven: 1997; 143-148.
Bridge JA, Schwartz HS, Neff JR. Sarcomas of Bone. In: Abeloff MD, Armitage JO, Lichter AS,
Niederhuber JE, eds. Clinical Oncology. Philadelphia, Pa: Churchill Livingstone 2000. 2160-
2272.

Dorfman HD, Czerniak B. Bone Cancers. Cancer 1995; 75: 203-210.

Malawer MM, Link MP, Donaldson SS. Sarcomas of Bone. In: VT DeVita, S Hellman, SA
Rosenberg, eds. Cancer: Principles and Practice of Oncology. Philadelphia, Pa: Lippincott-
Raven; 1997: 1789-1816.

PDQ database. Bone Cancer. PDQ Physician Statement. Bethesda, Md: National Cancer
Institute; 2000. Available at cancernet.nci.nih.gov/Cancer_Types/

Revised 8/13/00