Osteosarcoma Symptoms, Facts, Risks

What is Osteosarcoma?

Osteosarcoma or osteogenic sarcoma is a rare type of bone cancer. It develops in the osteoblast cells that form bone. It is a tumor in the distal femur and proximal tibia of adolescent patients. It happens mostly in children, teens, and young adults. When cells grow new bone form a cancerous tumor, this ailment happens.

Doctors use chemotherapy and surgery to treat this cancerous disease. These techniques are useful only before the spreading of cancer. Earlier, physicians used surgical resection techniques such as amputation or reconstruction with auto- or allograft.

The bones that are most often affected include:

  1. The femur (thigh bone) near the knee
  2. Tibia (shin bone) near the knee
  3. Humerus

Symptoms of Osteosarcoma

In osteosarcomas, the muscles in the cancerous leg or arm appear smaller than those in the opposite limb. Symptoms of osteosarcoma include pain in the legs. Symptoms may vary according to the location of the tumor. The most common symptoms include:

  1. Bone fractures
  2. Limping of leg
  3. Limitation of motion of joints
  4. Swelling at the site of the tumor
  5. Bone pain or tenderness of the bone
  6. A mass or tumor that can be felt through the skin
  7. Increased pain while lifting (if it affects an arm)
  8. Broken bone (sometimes after even a simple movement)

Fast Facts About Osteosarcoma

  1. An osteosarcoma tumor causes dull pain in the bone or joint around the tumor.
  2. Blood tests are not needed to diagnose osteosarcoma, but they may be helpful.
  3. Osteosarcoma accounts for about 3% of all childhood cancers.
  4. It is most often diagnosed between the ages of 10 and 30, with
  5. Most diagnoses occur in teens.
  6. The average for tumor development is 14-16 years.
  7. Osteosarcoma can come back after treatment.
  8. The lungs are the most common site for recurrence.
  9. About 1,000 new cases of osteosarcoma are reported each year in the U.S.
  10. It is the most common primary bone tumor in children.
  11. It is the third most common cancer in adolescence.
  12. The 5-year relative survival rate for SEER stage “regional” is 65 %.
  13. The 5-year relative survival rate for SEER stage “localized” is 77 %.
  14. Stage 4 osteosarcoma can be found in the tissue outside the bone, although it is rare.
  15. The most common sites are
  • the humerus (10%, with 90% of tumors in the proximal humerus)
  • the tibia (19%, with 80% of tumors in the proximal tibia)
  • the femur (42%, with 75% of tumors in the distal femur)

Osteosarcoma Risk Factors

A risk factor is anything that affects the chance of getting a disease such as cancer. Different cancer types have different risk factors. The exact cause is not known. However, doctors believe that inherited or acquired DNA mutations inside bone cells cause this bone cancer. The most common risk factors for osteosarcoma include:

Age

What is the most common age for females to be diagnosed with osteosarcoma?

Osteosarcoma is the most common bone cancer in children. The average age at diagnosis is 15 years. Boys and girls equally develop this tumor until their late teens. However, it occurs more often in boys. It is also common in people over aged people such as above 60.

In children, the average age of diagnosis is 15 due to growth spurts at this age. The risk is highest for those between the ages of 10 and 30. There is a link between rapid bone growth and the risk of tumor formation.

Adults are at higher risk if they have a history of Paget’s disease. The risk goes down in middle age. It can also be seen in adults over the age of 60. In older adults, the risk rises due to a long-standing bone disease.

Gender

Osteosarcoma is more common in males. Before adolescence, it occurs equally in boys and girls while after adolescence, it occurs slightly more often in boys. Females have their growth spurts earlier so they tend to develop it slightly earlier.

Race/ethnicity

Osteosarcoma is slightly more common in African-Americans and Hispanic/Latinos. It is comparatively not common in whites.

Height Factor

This bone cancer is more common in tall children. This thing suggests that osteosarcoma may be related to rapid bone growth.

Rapid bone growth

The risk of osteosarcoma increases during a child’s growth spurts. Children who are tall for their age may be more likely to develop this disease as their bones are growing quickly.

Genetic factors

Most osteosarcomas are not caused by inherited gene changes. Rather they are the result of gene mutations acquired during the person’s lifetime. Sometimes radiation therapy changes these genes. This may be due to the retinoblastoma gene, which can be associated with eye cancers in young children or with other tumors in children and young adults in which a mutation of the p53 gene is involved.’

Individuals with a family history of cancers and who have retinoblastoma, have a higher incidence of sarcoma.

Radiation to bones

Radiation therapy can damage the DNA inside cells. So such people who are under radiation treatment, have a higher risk. Being treated at a young age and with higher doses of radiation both increase this risk. Anyhow, the effect of x-rays, CT scans, bone scans, or other imaging tests is not known.

Certain bone diseases

People with certain non-cancerous bone diseases have an increased risk of developing osteosarcoma.

  • 1) Paget disease of the bone: Bone sarcomas develop in about 1% of people with Paget disease.
  • 2) Hereditary multiple osteochondromas: Osteochondroma has a very small risk of developing into a bone sarcoma such as chondrosarcoma, but less often it can be osteosarcoma.

Inherited cancer syndromes

People with certain inherited cancer syndromes have an increased risk. However, this is rare. Inherited cancer syndromes are as follows:

  • (1) Children with Rothmund-Thomson syndrome also are more likely to develop osteosarcoma. Such children are short and tend to have skin and skeletal problems.
  • (2) There is an increased chance of developing osteosarcoma, brain tumors, breast cancer, and other types of sarcoma if a patient has Li-Fraumeni syndrome.
  • (3) Rare eye cancer of children which is called retinoblastoma.
  • (4) Diamond-Blackfan anemia, Werner syndrome, and Bloom syndrome may also cause osteosarcoma.

Diagnosis of osteosarcoma

Doctors use a variety of tools to diagnose osteosarcoma. They conduct a physical examination to look for swelling and redness in the bone. Tests to diagnose osteosarcoma include:

  1. Sometimes, a simple blood test is used to check for tumor markers. These markers are chemical readings in the blood that indicate the presence of cancer.
  2. CT scan: a 3-D X-ray used to examine bones and soft organs in the body
  3. X-ray: produces images of dense tissue inside the bone. If an X-RAY shows a tumor in the bone, then doctors use a biopsy to remove a sample of tissue to test.
  4. MRI: uses sound waves and powerful magnets to create images of internal organs
  5. PET scan: It is a full-body scan.
  6. Biopsy: In this case, it is the removal of a sample of tissues from the bone for testing purposes.
  7. Bone scan: a sensitive imaging test that shows bone abnormalities.

Treatment of osteosarcoma

Treatment of osteosarcoma in children includes chemotherapy followed by surgery and then more chemo. Most osteosarcomas are treated with neoadjuvant chemotherapy before surgery for about 10 weeks. Patients are treated then again after surgery (known as adjuvant chemotherapy) for up to a year.

Patients with high-grade tumors receive about three months of chemotherapy before surgery. This is known as neo-adjuvant therapy. Osteosarcoma is mostly treated with a combination of therapies that can include surgery, chemotherapy, and radiation therapy.

There are no known ways to prevent osteosarcoma, presently. However, the risks of many adult cancers can be reduced with certain lifestyle changes.

Chemo drugs used to treat osteosarcoma

  1. Methotrexate (given in high doses along with leucovorin to avoid adverse effects)
  2. Epirubicin
  3. Etoposide
  4. Gemcitabine
  5. Topotecan
  6. Carboplatin or Cisplatin
  7. Cyclophosphamide
  8. Doxorubicin (Adriamycin)
  9. Ifosfamide
  10. Usually, two or more drugs are given together. Some common combinations include:
  11. High-dose methotrexate, doxorubicin, and cisplatin (sometimes with ifosfamide)
  12. Ifosfamide and etoposide
  13. Doxorubicin and cisplatin
  14. Ifosfamide, cisplatin, and epirubicin

Side effects of chemotherapy

Chemo drugs can cause adverse effects. Children tend to have less severe side effects from chemo than adults. They can recover from side effects more quickly. Therefore, doctors give them higher doses of chemo to try to kill the tumor.

The side effects of chemo depend on the type and dose of drugs given and the length of time they are taken.

General side effects of chemo

  1. Diarrhea
  2. Hair loss
  3. Loss of appetite
  4. Mouth sores
  5. Nausea
  6. Vomiting

Is osteosarcoma aggressive/deadly or curable?

Osteosarcoma is a malignant bone tumor that most commonly causes patients to die of pulmonary metastatic disease. In about 20% of cases of patients, osteosarcomas have spread at the time of diagnosis. It is a deadly form of musculoskeletal cancer. This cancer is tough to treat, but some patients can be cured if Surgery removes the metastasis of this cancer. Chemotherapy increases the cure rates.

Osteosarcoma is a disease of adolescents and young adults. However, it can occur in older people. In younger individuals, it is a highly aggressive tumor. In older individuals, radiation exposure, fibrous dysplasia, or Paget’s disease may increase the probability of this disease.

Potential Long-Term Complications of Osteosarcoma

Chemotherapy and surgery can not completely cure osteosarcoma. Doctors may suggest amputation to stop the spread of cancerous cells. This is the surgical removal of the cancerous limb.

Osteosarcoma can also spread to the lungs. Signs that bone cancer has metastasized (spread) to the lungs include:

  1. Chest pain
  2. Chronic cough
  3. Coughing up blood
  4. Shortness of breath
  5. Hoarseness
  6. Wheezing

Chemotherapy given to your child before and after surgery may produce adverse side effects which may include:

  1. Anemia (low RBC count)
  2. Fatigue
  3. Constipation & diarrhea
  4. Infections
  5. Fluid retention
  6. Vomiting & nausea
  7. Pain
  8. Hair loss

Survival Rates for Osteosarcoma

Keep in mind that survival rates are estimates. Survival rates tell about the percentage of people with the same type and stage of cancer who are still alive a certain amount of time after diagnosis. The prognosis is good if the tumor is confined to the original bone. 3 out of 4 people can be cured if their tumors have not metastasized elsewhere. The survival rate is about 30% if cancer spreads to distant parts of the body.

If the disease is localized, the long-term survival rate is 70 to 75 percent. If osteosarcoma has already spread to the lungs or other bones at diagnosis, the long-term survival rate reduces to 30%.

These numbers are based on people diagnosed with osteosarcoma between 2009 and 2015.

SEER stageThe 5-year relative survival rate
Localized77%
Regional64%
Distant27%
All SEER stages combined60%

Classification and Staging for Osteosarcoma

Doctors use the Musculoskeletal Tumor Society Staging System or the American Joint Commission on Cancer guidelines. They use the following terms to describe the stage of osteosarcoma and to decide on the treatment plan:

Localized

The tumor is only in the bone where it began and there is no sign that cancer has spread outside of the bone where it started.

Metastatic/Distant

The tumor has spread from the bone where it began to another part of the body. Cancer has spread outside the bone and into nearby structures, Most often, it has spread to the lungs or other bones.

Recurrence

Recurrent osteosarcoma is a tumor that has recurred during or after treatment. It can come back in the same place where it started or in another part. In most cases of recurrence, the cancer returns within two to three years after the patient finish preliminary treatment. Later recurrences are also possible.

Osteosarcoma recurs most often in the lungs and rarely in other bones. If there is a recurrence, the cancer is staged again and the phenomenon is called re-staging.

[Note: The featured image is a derivative of “Figure 2: Showing a firm, sessile tumor like growth in right mandibular alveolar ridge extending up to buccal vestibule, two weeks after incisional biopsy.” originally published in an article by & with the title “Halder GC, Patsa S, Jadav RB, Ray JG. Osteosarcoma of mandible: A case report. Int J Case Rep Images 2015;6(5):280–285.”, used under CC BY. “The featured image” is licensed under CC BY by King.]

Other factors that can affect prognosis

Factors other than the cancer stage also affect survival rates. For example, factors that have been linked with a better prognosis include:

  1. Being female
  2. Being young aged (child or teen, as opposed to adult)
  3. The tumor is on an arm or leg
  4. The tumor being completely removable
  5. Normal blood alkaline phosphatase and LDH levels
  6. The tumor has a good response to chemotherapy

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