Surgery for Ewing Tumors

Surgery is an important part of treatment for most Ewing tumors (Ewing sarcomas). Surgery is usually done for two reasons:

• To diagnose the cancer (with a biopsy)
• To remove the tumor(s)

Whenever possible, it’s very important that the biopsy and the surgery to remove the tumor be planned together, and that an experienced orthopedic surgeon at a cancer center does both operations. The biopsy needs to be done in a certain way so that there is a better chance less extensive surgery will be needed later on. 

The main goal of surgery is to remove all of the cancer. If even a small amount of cancer is left behind, it might continue to grow to make a new tumor, and might even spread to other parts of the body. To lower the risk of this happening, surgeons remove the tumor plus some of the normal tissue that surrounds it. This is known as a wide excision.

A doctor called a pathologist will look at the removed tissue with a microscope to see if the margins (outer edges) have cancer cells.

• If cancer cells are seen at the edges of the tissue, the margins are called positive. Positive margins can mean that some cancer was left behind.
• When no cancer cells are seen at the edges of the tissue, the margins are said to be negative, clean, or clear. A wide excision with clean margins helps limit the risk that the cancer will come back in the place where it started.

Types of surgery for Ewing tumors

Many types of surgery can be used for Ewing tumors. The choice depends on several factors, including:

• The tumor’s size and location
• The patient's age and overall health
• How likely it is that surgery can remove the tumor with clean margins
• How surgery would change the function of the affected part of the body

Tumors in some soft tissues and certain bones can be removed without causing major disability or deformity. Other tumors might not be able to be removed completely without affecting the function or appearance of that part of the body.

Although all operations to remove Ewing sarcomas are complex, tumors in the arms or legs are generally not as hard to remove as those in other parts of the body, such as the base of the skull, the chest wall, the spine, or the pelvis (hip bones).

Tumors in the arms or legs

For most tumors in an arm or leg, surgery can remove part or all of the affected bone while leaving the arm or leg basically intact. This is known as limb-sparing surgery (or limb-salvage surgery). The bone that is removed is replaced either with a bone graft (a piece of bone from another part of the body or from another person) or with an internal prosthesis (a rod-shaped device made of metal and other materials that replaces part or all of a bone). Some newer devices combine a graft and a prosthesis.

If the tumor is in the upper part of the leg, part of the femur (upper leg bone), including the knee, can be removed and replaced with a prosthesis for the bone and knee, which is connected to the lower leg. Tumors in the lower part of the leg are harder to treat this way, because it is harder to remove and reconstruct parts of the lower leg. The humerus (upper arm bone) is also suitable for limb-sparing surgery.

Limb-sparing surgery is a very complex operation. The surgeons who do this type of operation must have special skills and experience. The challenge for the surgeon is to be sure to remove the entire tumor while still saving the nearby tendons, nerves, and blood vessels to keep as much of the limb’s function and appearance as possible. If the tumor has grown into these structures, they will need to be removed as well.

Using an internal prosthesis in a growing child is especially challenging. In the past, it often required several operations over time to replace the prosthesis with a longer one as the child grew. Newer prostheses have become very sophisticated and can often be made longer without any extra surgery. They have tiny devices in them that can lengthen the prosthesis when needed to make room for a child’s growth. But even these prostheses may need to be replaced with a stronger adult prosthesis once the child’s body stops growing.

Some patients may not be able to have limb-sparing surgery because their tumors are in parts of bones that are hard to replace or because the tumors also extend into vital nerves or blood vessels that can’t be removed without severely damaging the limb. These patients usually get radiation therapy instead of surgery.

In rare cases, amputation (removal of part or all of the limb) may be the best option, especially if the cancer comes back in the place where it started, and radiation therapy has already been used.

For an amputation, surgery is usually planned so that muscles and the skin will form a cuff around the remaining bone. This cuff will fit into the end of a prosthetic (artificial) limb. Another option might be to implant a prosthesis into the remaining bone, with the end of the prosthesis remaining outside the skin. This can then be attached to an external prosthesis.

Tumors in the chest wall

For a Ewing tumor in the chest wall, the surgeon often must remove the diseased area and also remove nearby ribs, which might then be replaced with a man-made material. If the tumor has spread to the lungs, the chest can be opened and the lung tumors removed during an operation called a thoracotomy. Radiation therapy is often given to the chest as well.

Tumors in the pelvis (hip bones)

Pelvic tumors can be hard to treat with surgery, and in many cases radiation therapy may be the preferred treatment. But if the tumor responds well to initial chemotherapy, surgery (sometimes followed by radiation therapy) may be an option. Pelvic bones can sometimes be reconstructed after surgery, but in some cases pelvic bones and the leg they are attached to might need to be removed.

Tumors in the spine

Tumors in or right next to the spine can often be hard to remove completely, so radiation therapy is sometimes a better option. If surgery is done, radiation is often given afterward to try to kill any remaining tumor cells.

Possible side effects of surgery for Ewing tumors

Short-term risks and side effects: Surgery to remove a Ewing tumor is often a long and complex operation. Serious short-term side effects are not common, but they can include reactions to anesthesia, excess bleeding, blood clots, and infections. Pain is common after the operation, and strong pain medicines might be needed for a while after surgery as the site heals.

Long-term side effects: The long-term side effects of surgery depend mainly on where the tumor is and what type of operation is done.

Complications of limb-sparing surgery can include possible breaking or loosening of bone grafts or prostheses. This is more likely than with bone surgery done for other reasons because the chemo used before and after surgery can increase the risk of infection and affect wound healing. Infections in the area can be very serious because they can be hard to treat, and might require further surgery. Infections are also a concern in people who have had amputations, especially of part of a leg, because the pressure placed on the skin at the site of the amputation can cause the skin to break down over time.

Rehabilitation after surgery

This might be the hardest part of treatment, and it can’t be described here completely because it will be different for each patient. Whenever possible, patients and parents should meet with a rehabilitation specialist before surgery to learn about their options and what might be required after surgery.

Physical therapy and rehabilitation are very important for patients who have had surgery for Ewing tumors. Following the recommended rehab program offers the best chance for good long-term limb function. Even with proper rehab, people might still have to adjust to long-term issues such as changes in how they walk or do other tasks, and changes in appearance. Physical, occupational, and other therapies can often help people adjust and cope with these challenges.

Rehab after limb-sparing surgery

Even when only the tumor and part of the bone is removed in a limb-sparing operation, the situation can still be complicated, especially in growing children. Children who have had limb-sparing surgery may need more surgery in the coming years to replace the internal prosthesis with one more suited to their growing body size, and some may eventually need an amputation.

It takes about a year, on average, for patients to learn to walk after limb-sparing surgery on a leg. Physical rehabilitation after limb-sparing surgery is extremely important. If the patient doesn’t actively take part in the rehabilitation program, the salvaged arm or leg might become useless.

Rehab after amputation

If a limb is amputated, the patient must learn to adjust to new ways of doing some things, often with the use of a prosthetic limb. This can be particularly hard for growing children if the prosthetic limb needs changing to keep up with their growth. With proper physical therapy, patients are often able to walk on their own about 3 to 6 months after a leg amputation.

Considering your options

Both limb-sparing surgery and amputation can have pros and cons. For example, limb-sparing surgery, although often preferred by patients over amputation, tends to lead to more complications because of its complexity. Growing children who have limb-sparing surgery are also more likely to need further surgery later on.

When researchers have looked at the results of the different surgeries in terms of quality of life, there has been little difference between them. Perhaps the biggest problem has been for teens, who may worry about the social effects of their operation. Emotional issues can be very important, and all patients will need support and encouragement. (See Living as a Ewing Tumor Survivor.)