Bone Tumor

Definition

A bone tumor is an abnormal growth of cells within the bone that may be noncancerous
(benign) or cancerous (malignant).

Alternative Names

Tumor - bone

Causes, incidence, and risk factors

The cause of bone tumors is unknown. They often arise in areas of rapid growth. Possible
causes include:

 Inherited genetic mutations

 Radiation
 Trauma

But in most cases no specific cause is found.

Osteochondromas are the most common noncancerous (benign) bone tumors, and occur most
often in people between the ages of 10 and 20. Some benign bone tumors go away on their
own and do not require treatment. These benign tumors are monitored periodically by x-ray.

Cancers that start in the bones are referred to as primary bone tumors. Cancers that start in
another part of the body (such as the breast, lungs, or colon) are not considered bone tumors.
Multiple myeloma often affects or involves the bone, but is not considered a bone tumor.

Cancerous (malignant) bone tumors include:

 Chondrosarcoma
 Ewing's sarcoma
 Fibrosarcoma
 Osteosarcomas

The most common cancers that spread to the bone are cancer of the:

 Breast
 Kidney
 Lung
 Prostate
  Thyroid

These forms of cancer usually affect older people.

Bone cancer was once very common among people who painted radium on watch faces (to
produce glow-in-the-dark dials). The painters would "tip" the brush with their tongue (in
order to produce fine work) and absorb minute amounts of radium, which deposited in the
bone and caused cancer. The practice of using radium paint was abandoned in the middle of
the 20th century.

The incidence of bone cancer is higher in families with familial cancer syndromes. The
incidence of bone cancer in children is approximately 5 cases per million children each year.

Symptoms

 Bone fracture, especially fracture from slight injury (trauma)

 Bone pain, may be worse at night
 Occasionally a mass and swelling can be felt at the tumor site

Note: Some benign tumors have no symptoms.

Signs and tests

 Alkaline phosphatase
 Bone biopsy
 Bone scan
 MRI
 X-ray of bone

This disease may also affect the results of the following tests:

 Alkaline phosphatase isoenzyme

 Calcium (ionized)
 Extremity x-ray
 Hands x-ray
 Joints x-ray
 PTH
 Serum calcium
 Serum phosphorus

Treatment
Benign bone tumors may not require treatment, but may be looked at regularly to check if
they grow or shrink. Surgical removal of the tumor may be necessary.

Treatment for malignant tumors that have spread to the bone depends on the primary tissue or
organ involved. Radiation therapy with chemotherapy or hormone therapy is often used.

Tumors that start in the bone (primary malignant tumors of the bone) are rare and require
treatment at centers with experience treating these cancers. After biopsy, a combination of
chemotherapy and surgery is usually necessary. Radiation therapy may be needed before or
after surgery.

Support Groups

You can often help the stress of illness by joining a support group where members share
common experiences and problems. For this condition, see cancer - support group.

Expectations (prognosis)

The outlook depends on the type of tumor. The outcome is expected to be good for people
with noncancerous (benign) tumors, although some types of benign tumors may eventually
become cancerous (malignant).

With malignant bone tumors that have not spread, most patients achieve a cure. Because the
cure rate depends on the type of cancer, location, size, and other factors, discuss your
situation with your doctor.

Complications

 Pain
 Reduced function, depending on the extent of the tumor
 Side effects of chemotherapy (depending on the type of chemotherapy)
 Spread of the cancer to other nearby tissues (metastasis)

Calling your health care provider

Call your health care provider if you have symptoms of a bone tumor.

References

Skubitz KM, D'Adamo D. Sarcoma. Mayo Clin Proc. 2007;82:1409-1432.

Osteosarcoma Health Article

Definition

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

Osteogenic sarcoma

Causes, incidence, and risk factors

Osteosarcoma is the most common cancerous (malignant) bone tumor in youth. The average
age at diagnosis is 15. Boys and girls have a similar incidence of this tumor 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 retinoblastoma, 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.

Symptoms

 Bone fracture (may occur after what seems like a routine movement)
 Bone 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

Signs and tests

 Blood tests
 Bone scan to see if the cancer has spread to other bones
 CT scan of the chest to see if the cancer has spread to the lungs
 CT scan of the affected area
 Open biopsy (at time of surgery for diagnosis)
  X-ray of the affected area

Treatment

Treatment usually starts after a biopsy of the tumor.

Before major surgery to remove the tumor, chemotherapy 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.

Support Groups

Association of Cancer Online Resources -- www.acor.org

Cure Search (formerly the National Childhood Cancer Foundation) --www.curesearch.org

Expectations (prognosis)

If the tumor has not spread to the lungs (pulmonary metastasis), long-term survival rates are
very high. If the cancer has spread to other parts of the body, there is still a good chance of
cure with effective treatment.

Complications

 Limb removal
 Spread of cancer to the lungs
 Side effects of chemotherapy

Calling your health care provider

Call your health care provider if you have persistent bone pain, tenderness, or swelling.
References

Skubitz KM, D'Adamo D. Sarcoma. Mayo Clin Proc. 2007;82:1409-1432.

Baker MH. Bone tumors: primary and metastatic bone lesions. In: Goldman L, Ausiello D,
eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 212.

Review Info

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine,

Department of Medicine, University of Washington School of Medicine; Yi-Bin Chen, MD,
Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed
by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. , 11/05/2009

Fibrosarcoma Health Article

Definition

Fibrosarcoma is a malignant tumor that arises from fibroblasts (cells that produce connective
tissue). This is a type of sarcoma that is predominantly found in the area around bones or in
soft tissue.

Description

Fibrosarcomas are the result of fibroblasts, which produce connective tissue such as collagen.
Fibrosarcoma tumors are consequently rich in collagen fibers. The immature, proliferating
fibroblasts take on an interlacing, or herringbone, pattern.

Fibrosarcomas can form from fibroblasts in soft tissue such as muscles, connective tissues,
blood vessels, joints, and fat. Soft tissue fibrosarcoma normally occurs in fibrous tissue of the
body's trunk and the extremities such as the arms and legs. Soft tissue fibrosarcomas are
extremely rare, with approximately 500 new cases reported each year.

Sarcomas of the bone

Fibrosarcoma can also occur in bones. While a bone is made up of inorganic molecules such
as calcium phosphate, it also has an organic element made up of 95% collagen, similar to the
collagen found in the skin. Fibrosarcomas of the bone usually occur in long bones in the bone
marrow cavity where collagen is formed. The bones that predominantly yield fibrosarcomas
are those in the legs, arms, pelvis, and hip.
Sarcomas of the bone are rare and represent about 0.2 percent of all new cancer cases each
year. The two most common forms of bone cancer are osteosarcoma and Ewing's sarcoma.
Among the less common are chondrosarcoma, fibrosarcoma, and malignant fibrous
histiocytoma, all of which arise from spindle cell neoplasms.

Demographics

Fibrosarcomas typically develop in people between the ages of 25-79. The peak age of
occurrence is 55-69 years. Generally, fibrosarcomas develop equally in men and women,
though they are rare in children.

Infantile fibrosarcoma, also known as congenital fibrosarcoma or juvenile fibrosarcoma, is

unique. Under microscopic examination, it is similar to fibrosarcomas seen in adults.
However, infantile fibrosarcomas have a more positive prognosis with a post-treatment, five-
year survival rate of 83% to 94%.

Causes and symptoms

Fibrosarcomas of the bone are sometimes connected with underlying benign bone tumors.
Both fibrosarcomas of soft tissue and of the bone can develop as a result of exposure to
radiation. This can result as a side effect from previous radiation therapy for unrelated
primary cancer treatment. Individuals with other bone diseases, such as Paget's disease and
osteomyelitis, are at a higher risk for developing fibrosarcomas.

There are many symptoms associated with the onset of fibrosarcomas. The following is a list
of the main symptoms that may be present:

 pain
 swelling

 firm lump just under the skin or on a bone

 broken bone

 impeded normal range of motion

 neurologic symptoms

 gastrointestinal bleeding (seen in soft tissue abdominal fibrosarcomas)

 urinary frequency (seen in pelvic fibrosarcomas)

 urinary obstruction (seen in pelvic fibrosarcomas)

Diagnosis

In order to diagnose fibrosarcoma, a doctor will take the patient's medical history and will
conduct a thorough physical exam. Blood tests will be performed to rule out other conditions
and to identify cancer markers.
The most revealing initial exam is an x ray. It can show the location, size, and shape of the
tumor. If a malignant tumor is present, the x ray will expose a soft tissue mass with ill-
defined edges. This procedure takes less than an hour and can be performed in the doctor's
office.

Once there is evidence of a tumor, one or more of several other procedures may be
performed, including computed tomography (CT) scans, magnetic resonance imaging
(MRI), angiograms, and biopsies.

Treatment team

The patient's primary care physician may perform the initial diagnostic tests. However, in
order to comprehensively diagnose and treat fibrosarcomas, the primary care physician will
refer the patient to an oncologist (cancer specialist). Radiologists, pathologists, and surgeons
will also be involved to read x rays, examine tissue samples, and, if needed, remove the
tumor.

Other individuals might be involved with the treatment of fibrosarcoma, including nurses,
dieticians, and physical or vocational therapists.

Clinical staging, treatments, and prognosis

After the physician makes the diagnosis, it is important to determine the stage of the cancer.
This will help reveal how far the cancer has progressed and how much tissue has been
affected.

The American Joint Committee on Cancer developed the most widely used staging system for
fibrosarcomas. The foremost categories of this system include grade (G), size of the tumor
(T), lymph node involvement (N), and presence of metastases (M). Low grade and high grade
are designated G1 and G3, respectively. The size of the tumor can be less than 5 centimeters
(2 inches), designated as T1, or greater than 5 centimeters, designated as T2. If the lymph
nodes are involved, N1 is designated, while no lymph involvement is designated N0. Finally,
there may be a presence of distant metastases (M1), or no metastases (M0). The following is
a list of stages and their indications:

 Stage IA: (G1, T1, N0, M0)

 Stage IB: (G1, T2, N0, M0)

 Stage IIA: (G2, T1, N0, M0)

 Stage IIB: (G2, T2, N0, M0)

 Stage IIIA: (G3, T1, N0, M0)

 Stage IIIB: (G3, T2, N0, M0)

 Stage IVA: (Any G, any T, N1, M0)

 Stage IVB: (Any G, any T, N1, M1)

Tumors with lower stage numbers, such as IA and IB, contain cells that look very similar to
normal cells, while tumors with higher stage designations are composed of cells that appear
very different from normal cells. In higher staged tumors, the cells appear undifferentiated.

Physicians can employ several courses of treatment to remove fibrosarcomas. The most
effective treatment is surgical removal; this is used as a primary treatment for all stages of
fibrosarcoma. When performing the surgery, the surgeon will remove the tumor and some
healthy soft tissue or bone around it to ensure that the tumor does not recur near the original
site.

Prior to surgery, large tumors (greater than 5 centimeters, or 2 inches) may be treated with
chemotherapy or radiation in order to shrink them, thus rendering the surgical procedure
more effective.

Even individuals with low-grade fibrosarcoma who have undergone surgery experience a
moderate risk of local recurrence. To combat recurrence, adjuvant chemotherapy (the use of
one or more cancer-killing drugs) and radiation therapy (the use of high-energy rays), such as
irradiation and brachytherapy, are also used to complement surgery. Employing
chemotherapy or radiation therapy individually without surgery is much less effective.

After therapy, low-stage fibrosarcomas (stages IA and IB) have greater five-year survival
rates than high stages (Stages IVA and IVB). Because high-grade tumors are more aggressive
and more highly metastatic than lower grade tumors, patients with high-grade tumors have a
lower survival rate. Not only is the grade of the tumor (the estimate of its aggressiveness)
important in determining prognosis, the age of the patient is also crucial. Generally,
fibrosarcomas that occur in childhood and infancy have a lower mortality rate than those that
occur in adults. Additionally, patients with fibrosarcomas that occur in the extremities have a
better survival rate than those with fibrosarcomas in the visceral region.

Metastases appear later in the development of fibrosarcomas. The lungs are the primary sites
of metastasis for fibrosarcomas that develop in the extremities. Once metastasis to the lungs
has occurred, the chances of survival are significantly decreased.

Alternative and complementary therapies

Many individuals choose to supplement traditional therapy with complementary methods.

Often, these methods improve the tolerance of side effects and symptoms, as well as enrich
the quality of life. The American Cancer Society recommends that patients talk to their doctor
to ensure that the methods they choose are safely supplementing their traditional therapy.
Some complementary cancer therapies include the following:

 yoga
 meditation

 religious practices and prayer

 music therapy

 art therapy

 massage therapy
  aromatherapy

Coping with cancer treatment

Chemotherapy often results in several side effects, depending on the drug used and the
patient's individual tolerance. Patients may have to deal with nausea, vomiting, loss of
appetite (anorexia), and hair loss (alopecia). Many times, chemotherapy as well as radiation
therapy are better handled if patients are eating well. Nurses and dieticians can aid patients in
choosing healthful foods to incorporate into their diet.

If the fibrosarcoma necessitated a limb amputation, then patients will need to learn how to
cope with a prosthetic device. Both physical and vocational therapists can effectively help
patients adjust and learn how to use the prosthetic device to perform their daily activities.

Clinical trials

Fibrosarcomas are rare, but advances are being made in both diagnostic and curative
procedures. Although surgery is the most effective treatment, both pre-and post-operative
adjuvant therapies are being researched to complement surgery.

Exploring the results of chemotherapy trials uncovers a trend of improved results with more
intense regimens—meaning higher and more prolonged doses of drug therapy. Drugs that are
being studied in 2001 include cyclophosphamide, doxorubicin, methotrexate, vincristine,
dacarbazine, dactinomycin, or a combination of two or three of these.

Patients should consult with their physicians or contact the American Cancer Society to learn
what procedures are currently being investigated in clinical trials. In some cases, insurance
companies will not cover procedures that are part of clinical trials. Patients should talk with
their doctor and insurance company to determine which procedures are covered.

Prevention

The prevention of cancer can be assisted by avoiding known chemical carcinogens such as
alpha-naphthylamine, carbon tetrachloride, and benzene. Another way to avoid developing
cancer is to minimize exposure to penetrating radiation such as x rays and radioactive
elements. Medical x rays revolutionized the field of medicine and are used to detect and treat
many diseases. In most cases, the benefits of medical x rays outweigh the risks.

Special concerns

Treatment, especially surgical amputation, can take a physical and psychological toll on
cancer patients and their families. To deal with the psychological impact, there are many
different support groups and psychotherapists that can help. Some therapists will consider
amputation a posttraumatic stress disorder, and treat it accordingly. To deal with their
condition, relying on faith practices can also be beneficial for cancer patients. Patients should
discuss all options with their physician to determine what is available.
Once the cancer has been treated, patients should make sure to schedule follow-up
appointments with their physicians. Physicians will want to monitor the patient for side
effects or possible recurrence that may develop years after treatment.

Resources

BOOKS

Cordon-Cardo, Carlos. "Sarcomas of the Soft Tissues and Bone." In Cancer Principles and
Practice of Oncology., edited by Vincent T. DeVita, Jr. et al. New York: Lippincott-Raven
Publishers, 1997, pp.1731-82.

Rosen, Gerald. "Sarcomas of Nonosseous Tissues." In Cancer Medicine, edited by Robert C.

Bast Jr. et al. London: BC Decker, Inc., 2000, pp.1901-1921.

PERIODICALS

Palumbo, Joseph S., et al. "Soft Tissue Sarcomas of Infancy." Seminars in Perinatology
(August 1999): 299-309.

ORGANIZATION

American Cancer Society (National Headquarters). 1599Clifton Road, N.E. Atlanta, GA

30329. (800) 227-2345. <http://www.cancer.org>.

Cancer Research Institute (National Headquarters). 681 Fifth Avenue, New York, NY 10022.
(800) 992-2623. <http://www.cancerresearch.org>.

Sally C. McFarlane-Parrott