Osteogenic Sarcoma

What is osteogenic sarcoma?

Summary

Osteogenic sarcoma is a cancer of the bone that usually develops when bones are rapidly growing during adolescence. It is the most common primary tumor of the bone. Although any bone in the body can be affected, the thigh (femur), upper arm (humerus), shin (tibia), and pelvis are most commonly affected.

The primary symptom is achy pain specific to the bones affected by the tumor that may become progressively worse. People affected will likely experience difficulty moving the cancerous limb as well as fractures and swelling in the vicinity.

Treatment usually includes a combination of chemotherapy and surgery to remove the tumor. Rarely, radiation therapy will be used as a follow-up in order to destroy any remaining cancer.

Recommended care

You should visit your primary care physician who will be able to coordinate your care with a cancer specialist (oncologist). This condition is treated with chemotherapy and surgery. Unfortunately, surgery may involve amputation of the entire limb.

Osteogenic sarcoma symptoms

Osteogenic sarcoma can be diagnosed by X-ray, but usually require bone scans, MRI or CT scans, blood tests, and biopsy. Symptoms of osteogenic sarcoma can be defined by those that are more common and bone-specific, as well as further neurologic and systemic symptoms.

Bone-specific symptoms

These symptoms occur directly due to the presence of tumors and may include:

• Bone pain: Achy pain is the most common symptom. It may initially be tolerable and intermittent but often becomes worse and more constant as the tumor grows. It may be worse at night and with activity.
• Bone fractures: The tumor can disrupt the bone structure and create a weak area. This can lead to fracturing with routine movements (e.g., walking on a leg that has a tumor or throwing with an arm that has a tumor). A fracture is typically experienced as new, sudden, sharp pain over an area where prolonged, dull, achy pain was previously felt.
• Limp: Tumors in the legs typically lead to limping. Roughly 60 percent of cases occur in the legs.
• Difficulty lifting: Tumors in the arm result in pain on lifting. Roughly 10 percent of cases occur in the arm.
• Swelling: Over time, a lump in the soft tissue surrounding the bone tumor may appear. It may be warm and reddish. Severe swelling can limit the range of motion of the nearest joint.

Neurologic symptoms

If the tumor compresses a nerve, you may experience peripheral nervous system symptoms. These include:

• Focal weakness: Weakness in a single location, such as one hand or foot.
• Numbness over a patch of skin
• Tingling or "pins and needles" sensations in the skin

Other systemic symptoms of cancer

The following are other more widespread symptoms that can be experienced.

• Malaise: General feelings of illness and fatigue.
• Weight loss

Osteogenic sarcoma causes

In most cases, the causes of osteogenic sarcoma are not well understood. A minority of cases can be explained by genetic causes, prior radiation therapy, and Paget disease.

Genetics

Most cases of osteogenic sarcoma occur in the absence of known genetic mutations. However, it has been found to occur more frequently in families affected by several genetic syndromes, including:

• Familial retinoblastoma: A genetic mutation of the RB1 gene is associated with both retinoblastoma (a cancer of the eye) and osteogenic sarcoma. Children who have had retinoblastoma are at greater risk of osteogenic sarcoma.
• Li-Fraumeni Syndrome: This involves a mutation in the p53 gene and is associated with a variety of cancers.
• Werner Syndrome: This involves a mutation in the WRN gene and is associated with premature aging.

Radiation therapy

Osteogenic sarcoma has been found to be associated with prior radiation therapy for other cancers. Tumors usually develop 10 to 20 years after radiation exposure.

Paget disease

This disease involves accelerated bone turnover that transforms into osteogenic sarcoma in approximately one percent of cases.

Who is most likely to be affected

The following people are most likely to experience this condition.

• The average age of diagnosis is 15 years old
• Diagnoses most commonly occur in the 2nd and 6th decades of life (bimodal distribution)
• People affected are on average taller than the average population
• Youth cases occur more commonly in males than females
• Adult cases occur more commonly in females
• Occurs more commonly in Asian/Pacific-Islanders and African-Americans than in other groups

Treatment options and prevention for osteogenic sarcoma

Treatment depends on the size and location of the tumor and how far the cancer has spread. Research has shown that outcomes are typically most favorable when treatment includes both chemotherapy and surgery. In some cases, treatment may also involve radiation therapy.

Chemotherapy

Medication with drugs that disrupt the growth of cancer cells can help shrink the tumor.

• Types: Medications such as cisplatin, doxorubicin, and methotrexate in combination.
• Prognosis: Due to potential side effects, people who receive these medications are encouraged to receive follow-up monitoring of their heart and hearing for several years following the medications.

Surgical excision

Surgery aims to completely remove the cancer cells from the bone.

• Types: Depending on the age of the child, the size and location of the tumor, and other factors, your orthopedic surgeon may offer limb-sparing surgery or removal of the whole limb (amputation). Advances in chemotherapy and imaging have enabled a greater proportion of limb-sparing surgeries in recent years.
• Prognosis: A complete or partial artificial limb (prosthesis) can replace the removed portion. As the child grows, the limb may be continually adjusted as appropriate for the child's developing body.

Radiation therapy

X-rays in very high dose can be used to kill leftover cancer cells if the entire tumor cannot be removed by surgery. It can also be used to reduce pain in advanced cases. Radiation therapy is usually not required for osteosarcoma.

Duration of treatment

Length of treatment is variable but typically lasts five to eight months, with several years of close follow-up.

Prognosis

With treatment, the prognosis of osteogenic sarcoma is generally good. The five-year survival rate for localized osteosarcoma is 60 to 80 percent. Children have better outcomes than adults. Children who complete treatment are often able to walk and run without crutches but may be encouraged to refrain from certain strenuous activities to avoid further fractures. Without proper treatment, the cancer can progress to death within several months. If metastases are present at initial diagnosis, outcomes are less favorable.

When to seek further consultation for osteogenic sarcoma

If your child has a limp or constant bone pain, accompanied by fever, night sweats, weight loss, or loss of appetite, these symptoms may suggest serious conditions such as joint infections, cancer, or inflammatory conditions that require prompt evaluation and treatment. In addition to examining the child, your physician may order tests such as X-rays, CT scans or MRIs, drainage of the joint fluid, and/or blood work. If imaging is concerning for bone cancer, you may be referred to an orthopedic surgeon for a biopsy of the bone to determine further treatment.

If you or your child develops a limp that does not improve

Children can often develop limps after mild sports injuries, such as bruises or sprains, but these typically resolve quickly. However, limping in a child can be a sign of cancer or other serious conditions that require further evaluation. If the pain is so severe that it prevents the child from bearing weight on the joint or if the pain does not quickly improve, it is important to seek further medical attention.

If your child develops acute, sharp pain over an area or chronic, dull pain

Children with bone tumors can develop fractures with everyday movements, such as lifting, walking, or throwing. If your child has had dull pain for a few weeks and suddenly develops severe pain in that area, it may be a sign of a fracture, even if no obvious injury has occurred.