Osteosarcoma

We know how difficult a diagnosis of osteosarcoma can be, both for your child and for your whole family. That’s why Boston Children's Hospital physicians focus on family-centered care: From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs. We’ll work with you to create a care plan that’s best for your child. 

Our pediatric oncologists and orthopedic surgeons have a wealth of experience treating osteosarcoma. Dana-Farber/Children’s Hospital Cancer Center was one of the first centers in the United States to use adjuvant chemotherapy and perform limb-salvage surgery for patients with osteosarcoma.

What are the treatments for osteosarcoma?

Treatment options will vary greatly, depending on your child's situation. Your child's doctor and other members of your care team will discuss the options with you in-depth. Prompt medical attention and aggressive therapy are important for the best prognosis.

Traditional treatments for osteosarcoma

Treatments for osteosarcoma may involve a combination of therapies including surgery, chemotherapy and, rarely, radiation. In most cases, children receive chemotherapy before surgery (neoadjuvant), a surgical procedure to remove the tumor and additional chemotherapy after surgery (adjuvant).

Surgery

Depending on the size and location of the tumor and whether the tumor has spread, your child may receive one of the following surgical treatments:

• Limb-salvage surgery helps preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor. This type of surgery is done whenever possible, as long as the tumor can be completely removed using this method. The goal of limb-salvage surgery is to preserve as much use of the limb as possible and preserve limb function.
   
• Amputation may be necessary if the tumor cannot be completely removed (for example, if it involves the nerves and blood vessels) or if limb function cannot be preserved through limb-salvage surgery. If amputation is necessary, your child may be fitted for a prosthesis following surgery. Our physical therapists will work with your child to help him adjust to the prosthesis.
   
• Rotationplasty is an operation that preserves the lower leg and uses the ankle as a knee joint—especially useful in very young children where limb length would be an issue. This operation may be performed when the tumor is very large and complete removal would be difficult.

Surgery is personalized to help your child achieve the best functional and cosmetic outcome and to allow him to remain active and healthy. Talk to your child’s oncologist or orthopedic surgeon for more information on these procedures.

If the osteosarcoma has spread to other parts of the body, such as the lungs, additional surgery may be required, and our oncologic surgeons will remove any metastases that may have formed.

Chemotherapy

Chemotherapy is a group of drugs that interfere with the cancer cells' ability to grow or reproduce.

• Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
• Often, a combination of chemotherapy drugs is used.
• Certain chemotherapy drugs may be given in a specific order, depending on the type of cancer it is being used to treat.

While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.

Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:

• orally, as a pill to swallow
• intramuscularly, as an injection into the muscle or fat tissue
• intravenously, directly to the bloodstream
• intrathecally, directly into the spinal column with a needle

The chemotherapy used for osteosarcoma is given intravenously.

Radiation therapy

To treat some cancers, our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child’s surgery. However, this is rarely used to treat osteosarcoma.

Rehabilitation

Rehabilitation is an extremely important part of your child’s osteosarcoma care. This includes both physical and occupational therapy and help adapting to social situations. Your child’s physician will discuss what types of lifestyle changes may be necessary and our experienced physical and occupational therapists will work to optimize his mobility after surgery.

Unfortunately, some cases of osteosarcoma require partial or complete amputation of an arm or leg. At Children’s, our NOPCO Brace Shop provides each patient with an expertly-made, customized prosthesis and our physical therapists help children adjust to using them.

Supportive care

This is any type of treatment to prevent and treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment. Supportive treatment is an important part of preventing short- and long-term complications of the disease and treatment.

What is the recommended long-term care for children treated for osteosarcoma?

Children treated for osteosarcoma should visit a survivorship clinic every year to:

• manage disease complications
• screen for early recurrence of cancer
• manage late effects of treatment

A typical follow-up visit may include some or all of the following:

• a physical exam
• laboratory testing
• imaging scans

Through the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber Cancer Institute, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team.

• Our childhood cancer survivorship clinic is held weekly.
   
• In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists or alternative/complementary therapy specialists.
   
• We also offer the following services:
  • patient and family education
  • psychosocial assessment
  • genetic counseling
  • reproductive and fertility evaluation and counseling
  • opportunities to speak with other childhood cancer survivors

What treatments are available for relapsed or recurrent osteosarcoma?

Unfortunately, about 30 percent of children treated for osteosarcoma will experience relapse, a return of the disease.

Our pediatric oncologists and surgeons are experienced in treating relapsed osteosarcoma. For children with relapsed disease, we offer the highest standard of care, including additional surgery and chemotherapy. We also provide access to newly developed treatments through phase I and phase II clinical trials at Children’s and Dana Farber, and we are a Children’s Oncology Group site.

What is the long-term outlook for children with osteosarcoma?

Your child’s chance of recovery and treatment options depend on a number of different factors:

• the extent of the disease
• the size and location of the tumor
• the presence or absence of metastasis
• the tumor's response to therapy
• the age and overall health of your child
• your child's tolerance for specific medications, procedures or therapies
• new developments in treatment

In general, osteosarcomas in the limbs tend to be more treatable. Osteosarcomas in other locations are more difficult to treat. Prompt medical attention and appropriate therapy are important for the best prognosis.

Continual follow-up care is essential if your child has osteosarcoma. Side effects of surgery and chemotherapy, as well as recurrence of the disease, can occur in survivors of osteosarcoma.

Resources and support

We understand that you may have a lot of questions if your child is diagnosed with osteosarcoma. Will it affect my child long-term? What do we do next? We’ve tried to provide some answers to those questions in these pages, but there are also a number of resources and support services to help you and your family through this difficult time.

Long-term follow-up

Childhood cancer was once considered to always be fatal, but today, the majority of pediatric cancer patients can expect to be long-term survivors.

Survivorship comes with numerous complex issues, including the long-term effects of treatment, the risk of second cancers, as well as social and psychological concerns. Children treated for osteosarcoma may require long-term physical and occupational therapy and in some cases may need a brace or prosthesis to help them remain mobile and active.

Since 1993, physicians, nurses, researchers, and psychologists in the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber Cancer Institute have helped thousands of survivors of pediatric cancers, treated at the Institute and at other hospitals in New England and elsewhere, to manage these long-term consequences.

Founded in 1972, Children’s Hospital Boston’s NOPCO Brace Shop provides orthotics and prosthetics for children treated for osteosarcoma.

To make an appointment at the Perini Clinic, please contact the Clinic's facilitator at (617) 632-5124 or e-mail Perini_Clinic@dfci.harvard.edu.