Treatments for Neurofibrosarcoma in Children

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Surgery

Surgery includes biopsy and removal of the entire tumor and nearby tissue. Depending on the location and size of the tumor, it may be necessary to remove all or part of the limb. In most cases limb-sparing surgery is used to avoid amputation.

The following is a description of both procedures.

  • Limb-salvage surgery:
    • Limb-sparing surgery is indicated only if your child's orthopedic surgeon thinks that it is possible that the tumor (and wide margins of healthy tissue surrounding the tumor) can be removed.
    • In this type of surgery, all tissue involved with the tumor, including some of the nerve and other tissue surrounding it, are removed, and unaffected tendons, nerves and vessels are saved.
    • If bone is also removed, it is usually replaced with a bone graft or with a metal rod. Your child might need later surgery to repair or replace rods, which can become loose or break.
    • This type of surgery requires intensive rehabilitation. It may take as long as a year for your child to regain full use of a leg following limb-salvage surgery.
    • Radiation therapy and/or chemotherapy may be given either before surgery to shrink the tumor, or after surgery to kill remaining cancer cells.
    • Some patients who have limb-sparing procedures may eventually have to undergo amputation.
  • Amputation:
    • In certain cases, your child's orthopedic surgeon may determine that the tumor cannot be removed; if, for example, it involves the nerves and blood vessels.   In these cases, amputation is the only option.
    • During the operation, doctors ensure that muscles and skin form a cuff around the amputated bone.
    • A cast is applied in the operating room which permits a temporary artificial leg (prosthesis) to be applied during the first few post-operative days for walking.
    • Crutches are used for several weeks. As the swelling decreases (10 to 14 days) the patient is fitted for a plastic, temporary socket and prosthesis, which is used for 3 to 4 months until the stump is healed sufficiently to accept a permanent artificial leg.
    • The advantages of an amputation are that it is a simple operation with minimal chances of surgical complication and it definitively removes the local tumor. The functional outcome is also good with the modern prostheses available today, including "immediate-fit" prostheses applied in the operating room.
    • Although your child will probably have a limp with above-the-knee amputations, the procedure is functional and stable. He will be able to walk, climb stairs, swim (with the prosthesis on or off) and participate in many sports.

Radiation therapy

Radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors. This is often used together with surgery, either before or after removal of the tumor. On rare occasions, radiation alone is used for treatment of the primary tumor.

Chemotherapy

Chemotherapy is a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce.

How is chemotherapy given?

Different chemotherapies may be given:

  • as a pill to swallow
  • as an injection into the muscle or fat tissue
  • intravenously (directly to the bloodstream, also called IV)
  • intrathecally(directly into the spinal column with a needle)

How is chemotherapy used?

This depends on many factors. Some things to keep in mind:

  • A number of combinations of chemotherapy drugs are currently being used to stabilize and, in some cases, to shrink the size of the tumor.
  • Depending on the type of cancer, chemotherapy drugs may be given in a specific order.
  • Chemotherapy may be used alone for some types of cancer, or in conjunction with other therapy such as radiation or surgery.

Does chemotherapy come with bad side effects?

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 your child, family and your child's health care team prepare for and sometimes prevent these symptoms from occurring.

Continuous follow-up care

A schedule of follow-up care will be determined by your child's physician and other members of the care team to monitor ongoing response to treatment and possible late effects of treatment.

Treatment options will vary greatly, depending your child's situation. Your child's physician and other members of his care team will discuss these with you in-depth.

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- Sandra L. Fenwick, President and CEO

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