OSTEOSARCOMA

Osteogenic sarcoma
Definition
> Osteosarcoma is a cancer of the bone. It starts in immature bone cells that normally form new bone
tissue. Osteosarcoma usually arises in a bone and destroys local tissue and weakens the bone. It can
occur, very rarely, as a tumor of the soft tissues of the body, outside the bone itself. It usually occurs in
adolescents and young adults, but can occasionally occur in younger children.
Osteosarcoma most often starts in the bones around the knee joint, in the upper or lower leg next to the
knee, or in the thigh. The second most common place for osteosarcoma to develop is in the upper arm
bone, close to the shoulder. However, osteosarcoma can develop in any bone in the body.

> Osteosarcoma is a cancerous (malignant) bone tumorbone tumor that usually develops during the
period of rapid growth that occurs in adolescence, as a teenager matures into an adult.

> Osteosarcoma is a cancerous tumor that occurs in the bone.

Most osteosarcomas appear in the long bones of the body, such as the femur (the
thigh bone)
the tibia (the shinbone) or the humerus (the bone that runs from the shoulder to
the elbow),
although they can appear in any bones.

Causes/ Risk Factors

> Most osteosarcomas develop in people who have no other diseases and no
family history of bone cancer. Osteosarcoma may be triggered by an overactivity
of bone cells. In a very small number of families, the siblings develop
osteosarcoma. These families may be studied to see if a rare genetic defect may
be causing the tumor. If such a defect is found, it may help doctors identify other
family memebers that may be at risk and understand the process by which cancer
develops in other patients with osteosarcoma.
Osteosarcoma is probably caused by a combination of genetic changes that
together cause immature bone cells to become cancer cells instead of developing
into bone. The same gene that is commonly abnormal in patients that develop
eye tumors called retinoblastoma (the RB gene) may also be associated with
osteosarcoma. The RB gene is a "tumor suppressor" gene that normally controls
the growth of cells. When it becomes changed, or mutated, it can no longer
control cell growth, and tumors can form. Defects in another tumor suppressor
gene, P53, can also predispose individuals to osteosarcoma or other cancers.
These gene disturbances are very rare.

> Osteosarcoma is the most common cancerous (malignant) bone tumor in youth.
The average age at diagnosis is 15. Boys and girls have a similar
incidenceincidence of this tumortumor until late adolescence, at which time boys
are more commonly affected.
The cause is not known. In some cases, osteosarcoma runs in families, and at least one gene has been
linked to increased risk. This gene is also associated with familial retinoblastomaretinoblastoma, a
cancer of the eye that occurs in children.
Osteosarcoma tends to occur in the bones of the:
• Shin (near the knee)
• Thigh (near the knee)
• Upper arm (near the shoulder)
This cancer occurs most commonly in larger bones and in the area of bone with the fastest growth rate.
Osteosarcoma can occur in any bone, however.
Although it is rare, osteosarcoma can occur in adults.

> Osteosarcoma is most commonly found in adolescents and young adults. About
60% of cases
occur in people between 10 and 20 years of age.
There are no known risk factors for osteosarcoma, though young people may be
at higher risk if
they suffer from Retinoblastoma or Li-Fraumeni syndrome (see glossary). Adults
may be at
higher risk if they have a history of Paget's disease or radiation therapy used to
treat other
cancers.

Types
> The four main types of primary bone cancers are osteosarcoma,
Ewing’s sarcoma,
chondrosarcoma and malignant fibrous histiocytoma.
2
Ewing's sarcoma, the second most common form of bone tumor in children and
adolescents, is
a very rare tumor whose risk factors are not yet known.
Chondrosarcoma, a tumor based in the cartilage, is the second most common
type of tumor in
primary bone cancers. Chondrosarcoma is usually found in adults over 30, with
most patients
between 50 and 70 years of age.
Malignant fibrous histiocytoma (MFH), the rarest of the four most common
bone cancers, is
primarily found in adults over 30. Although most cases develop for unknown
reasons, a few
have been linked to Paget's disease or radiation therapy used to treat other
cancers.

Signs and Symptoms

> Symptoms
• Bone fracture (may occur after what seems like a routine movement)
• Bone painBone pain
• Limitation of motion
• Limping (if the tumor is in the leg)
• Pain when lifting (if the tumor is in the arm)
Tenderness, swelling, or redness at the site of the tumor

> Symptoms of osteosarcoma may include:

• Persistent pain, swelling or a firm lump on a bone, especially on an arm or leg.
• A limp (if the tumor affects the leg).
• Pain or difficulty breathing (if the tumor affects the ribs).
• A bone fracture that occurs spontaneously or after a minor bump.
Other symptoms include gradually worsening pain and swelling in an arm or leg,
near the knee
or shoulder.
The pain from the tumor may occur when the patient is resting or may awaken
the patient from
a sound sleep.

> The symptoms of osteosarcoma can vary, depending on the bone in which the
cancer develops. Symptoms may include:
• Pain in the bone or joint that gets worse over time
• A painless swelling or a noticeable mass in the arm or leg
• A broken bone that occurs without or with minimal injury or trauma
• Stiffness or swelling of joints (uncommon)
Back pain or loss of bowel or bladder control related to a tumor in the pelvis or at
the base of the spine. This is very rarely the first sign that a child has
osteosarcoma.

Diagnostic Tests/ Exams

> Exams and Tests
• Blood tests
• Bone scan to see if the cancer has spread to other bones
• CT scanCT scan of the chest to see if the cancer has spread to the lungs
• CT scan of the affected area
• Open biopsybiopsy (at time of surgery for diagnosis)
X-ray of the affected area

> After a full physical examination, the bone will be x-rayed. If the x-ray suggests
osteosarcoma,
further tests, such as a chest x-ray, blood test, CT scan, MRI, angiography, or
bone scan may be
performed. Finally, the diagnosis will be confirmed by a biopsy of the tumor.

Treatments
> Treatment
Treatment usually starts after a biopsy of the tumor.
Before major surgery to remove the tumor, chemotherapychemotherapy is usually given.
Chemotherapy is also used to kill or shrink any cancer cells that may have spread to other parts of the
body.
Common chemotherapy medicines include:
 • Cisplatin
• Carboplatin (Paraplatin)
• Cyclophosphamide (Cytoxan)
• Doxorubicin (Adriamycin)
• High-dose methotrexate with leucovorin
• Ifosfamide (Ifex)
Surgery is used after chemotherapy to remove any remaining tumor. In most
cases, surgery can remove the tumor while saving the affected limb (this is called
limb-salvage surgery). Rarely, more radical surgery (such as amputation) may be
necessary.

> The standard therapy for osteosarcoma is surgery with chemotherapy given
before and after
surgery. The chemotherapy is used to reduce tumor size or prevent any
recurrence of cancer
cells.
The percentage of destroyed tumor cells found in the tumor after surgery will
indicate the type
of chemotherapy treatment that should be used after the surgery.
Surgery for osteosarcoma uses one of three different procedures: limb-sparing;
rotational plasty
and amputation. The development of limb-sparing surgery has led to a large
decrease in
amputation rates among people with osteosarcoma. The decision to try limb-
sparing surgery
depends upon the tumor size, location in the body and response to chemotherapy
before the
surgery.
Limb-sparing surgery removes the cancerous tumor and bone, replacing it with
either a graft or
prosthesis to make the limb as functional as possible. Seventy percent to 90
percent of
osteosarcomas in the limbs can be treated by this method.
Rotation-plasty is a limb-sparing technique. The doctor removes a portion of the
leg, including
the knee. The lower part of the leg is rotated and reattached so that the ankle
becomes the
new knee, and a prosthetic device is attached to replace the ankle and foot.
Amputation is the removal of the limb. In most cases, prosthesis can be used to
replace the
limb.

Possible Complications
> Possible Complications
• Limb removal
• Spread of cancer to the lungs
Side effects of chemotherapy
Anatomy and Physiology

Skeletal System
The skeletal system provides four basic functions:

• Support for tissues and muscle

• Protection for vital organs
• Movement through bones and attached muscles
• Storage for minerals and immature blood cells

Growth
Ossification is the process by which bone is formed. Some bones (e.g. the flat bones of the skull)
are formed in one stage from the connective tissue. This process is known as intramembranous or
direct ossification.

Other bones (e.g. short bones) are formed from the cartilaginous model of the future bone
developed in the embryo, being dissolved and replaced by bone cells. This process is known as
endochondral or indirect ossification - most bones are formed this way.

Support
Bones and cartilage that make up the skeleton are the only rigid materials in the body. The 206
bones of the skeleton provide a framework and points of attachment for many of the soft tissues of
the body. The five main classifications of bones are : Long (e.g. femur), Short (e.g. tarsal bones of
the foot), Flat (e.g. frontal bone of the skull), Irregular (e.g. vertebrae) and Sesamoid (e.g. knee
cap)

Protection
These structures protect some of the vital tissues and functional organs of the body. Typical examples
are:

• Skull - protects the brain

• Vertebrae - protects the spinal cord
• Thoracic cage - protects the heart and lungs

Movement
Bones act as levers during movement and provide solid structures to which muscles are attached. The
joints allow movement between bones and these movements are directly related to the type of joint and
range of motion. Joints fall into one of three categories: Fixed fibrous or Synarthroses (e.g. bones of
the skull), Slightly moveable or Amphiarthroses (e.g. symphysis pubis) and Freely movable or
Diarthroses.

Freely Movable joints comprises of four main groups: Ball and Socket (e.g. hip), Hinge (e.g. elbow),
Pivot (e.g. radius and ulna) and Gliding (e.g. carpal joint of the wrist)

GLOSSARY
Angiography, Chest x-ray, CT scan, MRI – Special tests done by a radiologist
that give a
more detailed view of bone, nearby muscles, blood vessels, organs and other
tissues.
Biopsy – Removal of a small piece of tissue, which is then examined for cancer
cells.
Bone scan – A test performed by a radiologist that allows the doctor to check the
skeleton for
the presence of cancer.
Cartilage – A dense, elastic tissue, which cushions bones at the joints, connects
muscles with
bones, and provides structure to the nose and other body parts.
Chondrosarcoma – second most common type of cancer to arise from bones,
accounting for
20 to 25 percent of all primary bone cancers, most often found in adults.
Ewing's sarcoma – the second most common form of malignant bone tumor in
children and
adolescents.
Graft – replacement of diseased bone tissue with the patient’s own tissue
(autograft) or tissue
from a donor (allograft).
Li-Fraumeni syndrome – (LFS) – a rare cancer syndrome that runs in families.
LFS is caused
by a gene mutation. Family members who have LFS are at risk for many different
cancers,
including breast cancer and osteosarcoma.
Limb-sparing surgery – The surgical removal of an arm or leg tumor so that the
limb is
preserved.
Malignant fibrous histiocytoma (MFH) – a type of cancerous tumor that can
originate in
either bone or, most often, in the soft tissues that connect, support or surround
organs and other
body structures.
Osteosarcoma – a primary cancerous tumor that develops in a bone.
Paget's disease of bone – a chronic skeletal disorder which may result in
enlarged or deformed
bones in one or more parts of the skeleton.
Primary cancer – Cancer can begin in any organ or tissue of the body. The
original tumor is
called the primary cancer or primary tumor. It is usually named for the part of the
body or the
type of cell in which it begins.
Prosthesis – a synthetic device used to replace a missing body part.
4
Retinoblastoma – a cancer in the retina of one or both eyes. It usually occurs in
young
children.
Rotation-plasty – a limb salvage technique. The doctor removes a portion of the
leg, including
the knee. The lower part of the leg is rotated and reattached so that the ankle
becomes the new
knee, and a prosthetic device is attached to replace the ankle and foot.
Secondary cancer – Cancer which has spread (metastasized) from another part
of the body.
Syndrome -- a group of symptoms that indicate or characterize a disease,
psychological
disorder, or other abnormal condition.
Tumor – An abnormal mass of tissue. Tumors are either benign (non-cancerous)
or malignant
(cancerous).