Childhood Cancer

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Childhood Cancer Survivors

Ewing Sarcoma

Ewing sarcoma gets its name from the physician who first described it in 1921, Dr. James Ewing. For many years it was believed that Ewing sarcoma occurred only in the bone; however, other tumors within soft tissues have since been found to be similar. These include extraosseous Ewing sarcoma (EES) and peripheral primitive neuroectodermal tumor (PNET). Together, these malignancies are called the Ewing sarcoma family of tumors (ESFT).

Each year in the United States, about 650-700 children and adolescents younger than age 20 are diagnosed with a bone tumor, of which only 200 are Ewings Sarcoma. Ewings sarcoma tumors may occur from ages 5 to 10. The peak incidence is between 10-15 years of age. Boys tend to be diagnosed with this disease more often than girls, and there is a much higher incidence in white children than children of other races. The treatment and late effects are similar for both types of tumors and are addressed together in this section.

Treatment

ESFT tumors usually require, multiagent induction chemotherapy followed by local treatment with surgery, definitive radiation, or a combination of surgery and radiation followed by additional chemotherapy.

Surgery

Before the development of limb-salvage surgery and newer radiation techniques, most children with extremity tumors had the affected limb amputated. Many children now are treated with state-of-the-art radiation therapy and/or have limb-salvage procedures that use autologous grafts, allografts, or endoprostheses. In some cases, orthopedic reconstruction is required after removal of the tumor.

Radiation

Radiation is needed to treat children diagnosed with ESFT tumors that cannot be completely removed. Some chest wall tumors are treated with whole-lung radiation.

The current guideline for radiation treatment for ESFT Tumors includes Involved Field Radiation with limited margin. Whole-lung radiation also was used in some studies to reduce the number of pulmonary relapses; however, this resulted in significant toxicity when combined with systemic chemotherapy. Currently, lung radiation is used only for some chest wall tumors.

Chemotherapy

In the 1960s before chemotherapy became a standard treatment against ESFT tumors, very few children survived. Chemotherapy improved the long-term survival rate and made it easier to remove the tumor by reducing the size before surgery. Treatment of Ewing sarcoma now includes chemotherapy for all children. This is necessary even for children with localized disease.

Late effects of Ewing sarcoma

This section briefly outlines some common and uncommon late effects from treatment. Remember that you may develop none, one, or several of these problems in the months or years after treatment ends.

Damage to soft tissues and bones. One of the most common and troublesome late effects from radiation treatment for ESFT tumors is damage to soft tissues and the underlying bones. If the leg of a young child gets high doses of radiation, it stops growing and will be shorter than the nonirradiated leg. Radiation around the arm or leg can result in fibrosis (meaning scarring), swelling, and poor function. Most of these changes happened with older radiation techniques.

Loss of function can also be minimized or prevented by a comprehensive physical therapy program during and after treatment. For more information, see Chapter 18, Muscles and Bones.

Heart problems. Heart problems can occur months or years after treatment with anthracyclines (i.e., doxorubicin, idarubicin, or daunorubicin) and/or chest radiation. Most ESFT tumor survivors receive a large amount of chemotherapy which may affect the heart. Symptoms include shortness of breath, fatigue, wheezing, anxiety, poor exercise tolerance, rapid heartbeat, and irregular heartbeat. Regular follow-up with oncology provider and cardiologist is crucial throughout life. Survivors may have no symptoms, but problems may be found on cardiac tests such as echocardiograms, electrocardiograms (EKGs), and Holter monitors. For more information, see Chapter 13, Heart and Blood Vessels.

Fertility. Abdominal radiation and high doses of cyclophosphamide and/or ifosfamide can affect fertility. For more information on fertility, see Chapter 10, Hormone-Producing Glands, and Chapter 3, Relationships.

Digestion. Abdominal radiation can also cause problems with digestion and absorption of food. For more information, see Chapter 16, Liver, Stomach, and Intestines.

Secondary cancers (subsequent malignancy). There is a small chance of developing a secondary cancer in the radiated area. For more information, see Chapter 20, Subsequent Malignancies.

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