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Surgery is an important part of treatment for Ewing sarcoma. Surgery is done for 2 reasons:

• To collect a part of the tumor (biopsy) for diagnosing the cancer
• To remove the tumor(s)

Whenever possible, it’s very important that the biopsy and the surgery to remove the tumor be planned together. The biopsy needs to be done in a certain way so that there is a better chance less extensive surgery will be needed later.

The main goal of surgery after a few cycles of chemo, when possible, 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 surgery? is known as wide excision.

After surgery, a doctor called a pathologist will look at the removed tumor with a microscope to see if the margins (outer edges) have cancer cells.

• If cancer cells are seen at the edges, the margins are positive. Positive margins can mean that some cancer was left behind after surgery.
• 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 where it started.

Whether the margins are positive or negative, more treatment will likely be needed after surgery. The margins help doctors decide what treatments will be needed for the best chance of success.

Types of surgery for Ewing sarcoma

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

• The tumor’s size and location
• The person's age and overall health
• How likely it is surgery can remove the tumor with clean margins
• How surgery would change the function or appearance 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 function or appearance.

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).

If the tumor is in an arm or leg

Tumors in the arms or legs might be treated with either:

• Limb-sparing (limb-salvage) surgery: removing the cancer and some surrounding tissue but leaving the limb basically intact
• Amputation: removing the cancer and all or part of an arm or leg

Limb-sparing surgery 

Most people with tumors in an arm or leg can have limb-sparing surgery, but this depends on where the tumor is, how big it is, and if it has grown into nearby structures.

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

The section of bone removed with the tumor can be 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 device made of metal and other materials that replace part or all of a bone). Some newer devices combine a graft and a prosthesis.

Complications of limb-sparing (salvage) surgery

All surgery has a risk of complications. Surgery to remove Ewing sarcoma 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.

A graft or rod can become infected, loose, or broken as well. Someone who has limb-sparing surgery might need more surgery in the future, and some might still eventually need an amputation.

Prostheses for children

Using an internal prosthesis in a growing child is especially challenging. In the past, it 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 more surgery. They have tiny devices in them that can lengthen the prosthesis when needed to make room for a child to grow. But even these prostheses might need to be replaced with a stronger adult prosthesis once growing stops.

Walking after limb-sparing surgery

It takes about a year, on average, for people to relearn to walk after limb-sparing (salvage) surgery on a leg. Physical rehabilitation after limb-salvage surgery is more intense than after an amputation, and it is extremely important. Without rehab, the salvaged arm or leg won’t work well. In many cases, some limitations may be placed on physical activity to protect the salvaged limb from injury.

Amputation

If the tumor is very large, or if it extends into the nerves and/or blood vessels, it might not be possible to save the limb and amputation may be the best option. Some people might choose amputation even when saving the limb might be an option, because of physical activity limitations.

The surgeon can determine how much of the arm or leg needs to be amputated based on the results of imaging tests and an examination of the tissue removed during surgery.

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.

Reconstructive surgery

Reconstructive surgery can help some people who have lost a limb to function as well as possible. For example, if the leg must be amputated mid-thigh (including the knee joint), the lower leg and foot can be rotated and attached to the thighbone, so that the ankle functions as a new knee joint. This surgery is called rotationplasty. The person would still need a prosthetic limb to replace the lower part of the leg.

If Ewing sarcoma is in the shoulder or upper arm and amputation is needed, in some cases, the area with the tumor can be removed and the lower arm reattached so that the arm is functional but much shorter.

Complications of surgery

All surgeries have some risk of complications. Surgery to remove Ewing sarcoma 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.

If an amputation is done, rehabilitation will be needed to 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, a person will often be able to walk on their own in about 3 to 6 months.

If the tumor is in the chest wall

For Ewing sarcoma 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.

If the tumor is in the pelvis (hip bones)

Tumors in the pelvic bones can often be hard to remove completely with surgery. But if the tumor responds well to chemotherapy first, surgery (sometimes followed by radiation therapy) may get rid of the cancer. Pelvic bones can sometimes be reconstructed after surgery, but sometimes pelvic bones and the leg they are attached to might need to be removed.

If the tumor is 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.