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Ewing's Sarcoma

Pediatric and adult sarcoma patients at Johns Hopkins are treated by a team of sarcoma experts that include medical and radiation oncologists, orthopedics and plastic surgeons.  Through the Johns Hopkins Sarcoma Center, patients also have access to the latest clinical trials and treatments integrated into a comprehensive care plan.

Sarcoma Experts

Johns Hopkins sarcoma experts use the latest technology, surgical techniques and clinical trials to treat sarcomas in patients of all ages.  Our multidisciplinary team meets weekly to review and coordinate care for all sarcoma patients. 

About Ewing's Sarcoma

Sarcomas are a rare type of malignant tumor that can be divided into two groups: bone sarcomas and soft tissue sarcomas. Each year, approximately 8,900 new cases of bone and soft tissue sarcomas are diagnosed. Of the approximate 8,900 new cases, 650-700 children and adolescents younger than 20 years of age are diagnosed with bone sarcomas and 850-900 with soft tissue sarcomas.

Ewing's sarcoma is the second-most common cancerous bone tumor affecting children and young adults. It gets its name from Dr. James Ewing, the doctor who first described the tumor in the 1920s.

Ewing’s sarcoma may arise anywhere in the body, but it usually originates in the long bones of arms and legs, the pelvis or the chest. Though more rare, it may also develop in the skull or flat bones of the trunk. While it is most often a bone tumor, Ewing’s sarcoma can develop in the soft tissue near bones as well.

Under the microscope, Ewing’s sarcoma is characterized by small, round, blue cells. Primitive neuroectodermal tumors (PNET) are very rare molecularly-related tumors that often arise outside of the bone and are treated the same as Ewing's sarcoma.

While Ewing’s sarcoma can occur anytime during childhood, it most commonly develops during puberty when bones are growing rapidly. This type of cancer is uncommon in African-American, African, and Chinese children.

Ewing's Sarcoma Treatment

Treatment options for Ewing’s sarcoma include surgery, chemotherapy and radiation therapy. Several cycles of chemotherapy (treatment with anticancer drugs) is usually suggested as the first stage of therapy to shrink the primary or main tumor. Chemotherapy drug regimens may include adriamycin, vincristine, cyclophosphamide, etoposide and ifosfamide.

Unlike osteosarcomas, Ewing’s sarcomas are sensitive to radiation, which can be used treat the primary site of the disease. Radiation occasionally is used as an alternative treatment to surgery in areas where it is unlikely that all tumor cells will be removed, such as the spine, or if it is too difficult to restore function after surgery, such as the pelvis.

Generally, if the cancer can be removed, surgery is recommended since radiation therapy may have profound side effects, especially in young children. Patients treated with surgery alone or surgery and radiation have a lower chance of cancer recurrence than if they were treated with radiation alone. Additional chemotherapy is given following surgery or radiation therapy to destroy any tumor cells that may have spread to other parts of the body.

Follow-Up and Survivorship Care

Ewing’s sarcoma patients will be monitored with x-rays of the original tumor every three to six months for three to five years. Similarly, patients will have regular CT scans of the lungs and periodic bone scans to detect recurrence as early as possible. For the rest of their lives, patients will have yearly x-rays of the area of the original tumor to monitor any reconstructive devices and healing of the limb. Exercises may be suggested to increase the function of the affected limb.

 

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