Overview
Ewing’s sarcoma is a relatively rare cancer and is best treated in specialized medical centers. These centers will have a multidisciplinary team of specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team incorporates the skills of the primary care physician, an orthopedic surgeon experienced in bone tumors, a pathologist, radiation oncologists, pediatric oncologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others in order to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life.
Surgery is an integral part of the treatment of localized Ewing’s sarcoma and in selected cases of metastatic or recurrent Ewing’s sarcoma. Reconstructive surgery is also an important component of the overall management of Ewing’s sarcoma requiring the skills of an orthopedic surgeon or other subspeciality surgeons specializing in the area of the primary tumor site. Surgery is increasingly being performed following initial treatment with chemotherapy and/or radiotherapy to reduce the tumor mass. This strategy is adopted in order to decrease the size of the tumor before surgery, often in an attempt to avoid amputation.
Types of Surgery Performed for Ewing’s Sarcoma
Amputation: In the past, complete removal of the affected limb was the main treatment for patients with Ewing’s sarcoma and resulted in the cure of approximately 20 percent of patients. With the use of neoadjuvant (before surgery) chemotherapy, limb preservation is now possible in over 70 to 80 percent of patients with localized Ewing’s sarcoma.12 When primary treatment involves limb preservation, amputation is often used to treat recurrences.
Limb Salvage Surgery for Localized Ewing’s Sarcoma: Wide local excision after neoadjuvant chemotherapy is the most common approach to the treatment of patients with localized Ewing’s sarcoma. Wide local excision involves surgical removal of the cancer along with some surrounding normal tissue.
Surgery for Metastatic or Recurrent Ewing’s Sarcoma: It is important that the initial surgery remove as much cancer (both the primary cancer and operable areas of metastatic cancer) as possible. Surgery for metastatic lung nodules often involves the removal of only a small part of the lung, but may occasionally involve more extensive lung surgery or even removal of an entire lung. When cancer is present in both lungs, a separate incision for each lung is usually performed.
Recurrence of Ewing’s sarcoma following initial treatment is most common in the lung. Patients with recurrent Ewing’s sarcoma confined to the lungs can often be treated successfully with surgery. Complete surgical removal of the recurrent cancer is linked with better prognosis.
Reconstructive Surgery: There are a number of procedures for limb reconstruction after surgical removal of the primary Ewing’s sarcoma. These include bone grafts (using the patient’s own bone or bone from a donor) and prosthetic implants.
Rotationplasty is a technique used commonly in patients with Ewing’s sarcoma that involves the lower femur or upper tibia. This technique is used when the tumor is large and amputation is the only option. It is called a rotationplasty because the distal (far) portion of the leg is rotated 180 degrees and reattached to the thigh. The concept of the rotation is for the ankle to become a functional knee joint when the length of the leg is adjusted to match the opposite knee. This is difficult to visualize and it is recommended that families view a video of the actual procedure in order to understand what is taking place. These video tapes can also be downloaded from several web sites on Ewing’s sarcoma.
Strategies to Improve Treatment:
The progress that has been made in the treatment of Ewing’s sarcoma has resulted from improvements in surgery, chemotherapy and radiation therapy, as well as from doctor and patient participation in clinical studies. Future progress in the treatment of Ewing’s sarcoma will result from continued participation in clinical research. Currently, there are several areas of active exploration aimed at improving the treatment of localized Ewing’s sarcoma.
Reconstructive Surgery: Most of the surgical research in patients with Ewing’s sarcoma is in the field of reconstructive surgery. Patients and families will need to carefully review the large array of options and the latest prostheses available following limb salvage surgery. Some of these options will be complex and difficult to understand, but they can dramatically affect quality of life following surgery for Ewing’s sarcoma. It is important to understand these options before making a decision with the operating surgeon.
References:
1 Aksnes LH, Hall KS, Folleraas G et al. Management of high-grade bone sarcomas over two decades: The Norwegian Radium Hospital Experience. Acta Oncol 2006;45:38-46.
2 Wafa H, Grimer RJ. Surgical options and outcomes in bone sarcoma. Expert Rev Anticancer Ther. 2006;6:239-248.