Localized pain is the most frequent symptom. Systemic symptoms like fever, weight loss, or anemia may occur. Fever is related to cytokines produced by the tumor cells. Other symptoms depend on the organs closest to the tumor.

Disorders caused by nerve compression or mechanical disorders are associated with pelvic tumors. Respiratory disorders or pleural effusion are associated with costal tumors. Radicular compression is associated with vertebral tumors.

The tumor initially develops most frequently in bone, particularly in the pelvis (30%), thorax (20%), femur (16%), tibia (9%), vertebrae (8%) and humerus (5%). The disease has a high potential for metastases.

No specific blood tests are used in the diagnosis of Ewings sarcoma. However, the following tests and procedures may be used to diagnose or stage the malignancy: physical exam, magnetic resonance imaging, X-ray, fluorodeoxyglucose positron emission tomography (FDG-PET), laboratory tests (blood cultures, C-reactive protein levels, erythrocyte sedimentation rate and complete blood count). A biopsy may be done to definitively diagnose Ewings sarcoma. [3]

There is no standard staging system. Ewing sarcoma is described based on whether the cancer is localized or metastatic.

Treatment starts systematically with chemotherapy with alternating courses of 2 regimens: vincristine, doxorubicin, and cyclophosphamide; and ifosfamide and etoposide. Chemotherapy is administered to reduce the size of the primary tumor and to eradicate or prevent metastases [4].

For more severe forms of the disease (limited response to initial chemotherapy or metastases already have been diagnosed), high dose chemotherapy with stem cell transplantation is suggested as a last resort treatment.

Only patients who have a good initial response to standard chemotherapy are considered for stem cell transplantation. Surgery and radiotherapy are recommended whenever the location of the tumor allows. Local radiotherapy sometimes is necessary, with surgery or alone for inoperable tumors [5] [6].

The main prognostic factors of Ewing’s sarcoma are: age, general health of the patient, location and size of the tumor, the stage of the disease, surgical outcome and response to treatment.
The survival of patients with Ewings sarcoma depends highly on the initial manifestation of the disease.

Approximately 80% of patients present with localized disease, whereas 20% present with clinically detectable metastatic disease, most often to the lungs, bone, or bone marrow. Less common sites include the central nervous system and lymph nodes.

Five-year survival for localized Ewings sarcoma is 70% to 80% when treated with chemotherapy [7].

Most cases of Ewings sarcoma occur due to a translocation between chromosomes 11 and 22. This translocation joins the Ewing’s sarcoma gene (EWS) on chromosome 22 to a gene of the ETS family, friend leukemia insertion (FLI1), on chromosome 11.

Ewings sarcoma is the second most common bone cancer in children, but it's also relatively rare uncommon. It accounts for only 1% of all childhood cancers. The incidence is 0.6 per million with a rate of 0.3 cases per million in children under 3 years of age, and as high as 4.6 cases per million in adolescents aged 15–19 years. The incidence of these tumors in males is higher than in females. It very rarely occurs in adults over the age of 30.

Classic Ewings sarcoma is a poorly differentiated, homogeneous population of small round-cells with high nuclear-to-cytoplasmic ratio, characterized by strong expression of glycoprotein MIC2 and CD99.

There are no guidelines for prevention of Ewings sarcoma.

Ewings sarcoma is a malignant small round cell bone tumor with strong metastatic potential. It is a primary bone cancer that affects mainly children and adolescents. The most common areas in which it occurs are the pelvis, the femur, the humerus, the ribs and clavicle. Ewings sarcoma also may be found in the soft tissues of the trunk, arms, legs, abdominal cavity, head and neck.

Other names for Ewings sarcoma include primitive neuroectodermal tumor, Askin tumor, extraosseous Ewing sarcoma and peripheral neuroepithelioma. All these types are called Ewing sarcoma family of tumors [1] [2].

Ewings sarcoma is a type of tumor that forms from a certain kind of cell in bone or soft tissue. It may be found in the bones of the skull, arms, hands , legs, feet, chest, pelvis, and spine. Ewings sarcoma also may be found in the soft tissue of various areas. They most commonly occur in teenagers and young adults.

Signs and symptoms

• Swelling
• Painful lump
• Fever for no known reason
• Bone fracture for no known reason

Examinations

The following tests may be used to diagnose the disorder: physical exam, magnetic resonance imaging (MRI), computed tomography (CT) scan, positron emission tomography (PET) scan, bone marrow aspiration and biopsy, X-ray, laboratory studies (complete blood count, blood chemistry studies).

Staging is the process that used to find out if cancer has spread in the body. The disease is grouped to localized or metastatic. Localized Ewings sarcoma refers to a tumor which is found where it began. Metastatic Ewings sarcoma means that the cancer has spread from the bone or soft tissue in which it began to other parts of the body.

Treatment

Three types of standard treatment are used: Chemotherapy is part of the treatment for all patients with Ewings tumors. Chemotherapy may also be given to shrink the tumor prior to surgery or radiation therapy. Radiation therapy is used when the tumor cannot be removed surgically. It is also used to make the tumor smaller, and decrease the amount of tissue that required to be removed during surgery. Surgery is usually done to remove cancer that is left after chemotherapy or radiation therapy.

1. Iwamoto Y., Diagnosis and treatment of Ewing's sarcoma, Jpn. J. Clin. Oncol. 37 (2) 2007: 79–89.
2. Bernstein M, Kovar H, Paulussen M, Randall RL, Schuck A, Teot LA, Juergens H. Ewing's sarcoma family of tumors: current management. Oncologist. 2006; 11(5): 503-19.
3. Paulussen M, Craft AW. Ewing Tumours: Management and Prognosis in, Education Book, International Society of Paediatric Oncology, 37th Congress of the International Society of Paediatric Oncology Vancouver, British Columbia, Canada - September 20-24, 2005.
4. Dai X, Ma W, He X, Jha RK., Review of therapeutic strategies for osteosarcoma, chondrosarcoma, and Ewing’s sarcoma. MED Sci Monit. 2011; 17 (8): RA177–RA190.
5. Spira A. and Ettinger D., The Use of Chemotherapy in Soft-Tissue Sarcomas. The Oncologist. 2002 vol. 7 no. 4 348-359.
6. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. Feb 20 2003;348(8):694-701.
7. Lahl M, Fisher VL, Laschinger K. Ewing sarcoma family of tumours: an overview from diagnosis to survivorship, Clin J Oncol Nurs 12 2008 (1): 89–97.