Ewing's sarcoma overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Assistant Editor(s)-In-Chief: Michael Maddaleni, B.S.

Overview

Ewing's sarcoma is the common name for primitive neuroectodermal tumor. It is a rare disease, and until recently, it had a very low long term survival rate. It is a small round-cell tumor in which cancer cells are found in the bone or in soft tissue (rarer). The most common areas in which it occurs are the pelvis, the femur, the humerus, and the ribs.

Classification

There is no classification system established for Ewing sarcoma.

Pathophysiology

Ewing sarcoma may occur anywhere in the body, but most commonly in the pelvis and proximal long tubular bones. The pathogenesis of Ewing sarcoma include t(11;22) chromosomal translocation. On microscopic histopathological analysis, presence of small round cells that have a high nuclear to cytoplasmic ratio, vacuolated cytoplasm, and faded boundaries are characteristic findings of Ewing sarcoma.

Causes

There are no established causes for Ewing sarcoma.

Differential Diagnosis

Ewing sarcoma must be differentiated from osteosarcoma, hematological malignancy, eosinophilic granuloma, and malignant fibrous histiocytoma.

Epidemiology and Demographics

Ewing sarcoma is the second most common malignant bone tumor in patients younger than 20 years. Ewing sarcoma typically occurs in children and adolescents between 10 and 20 years of age. The overall incidence of Ewing sarcoma is 0.1 cases for 100,000 individuals. The incidence of Ewing sarcoma in children aged 10 to 19 years is 1 case for 100,000 individuals. Males are more commonly affected with Ewing sarcoma than females.

Risk Factors

Common risk factors in the development of Ewing sarcoma are age, gender, and race.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Ewing sarcoma.

Natural History, Complications and Prognosis

Common complications of Ewing sarcoma include pathologic fracture and metastasis. Pretreatment factors that influence outcome of the Ewing sarcoma are site of the tumor, size of the tumor, serum LDH levels, and site of metastasis. After administration of preoperative chemotherapy, patients with minimal or no residual viable tumor have a significantly better prognosis than do patients with larger amounts of viable tumor.

Diagnosis

History and Symptoms

The most common symptoms of Ewing sarcoma include localized bone pain, swelling, fever, loss of appetite, and weight loss. The affected bone is not as strong as normal bones and may fracture with minor trauma (a pathological fracture).

Physical Examination

Common physical examination findings of Ewing sarcoma are fever, localized swelling, and tenderness at the site of the tumor.

Laboratory Findings

Laboratory tests for Ewing sarcoma include complete blood count, erythrocyte sedimentation rate, serum alkaline phosphatase, and lactate dehydrogenase

Biopsy

Biopsy of Ewing sarcoma is important for confirming the diagnosis and determining histologic subtype. Biopsy may be performed percutaneously with either a fine-needle, or wide-bore needle, or through a formal incision.

References

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