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[Ewing sarcoma--current opinion].

Zoran Vukasinović, Vladan Stevanović, Dusko Spasovski

Srpski Arhiv Za Celokupno Lekarstvo

|October 3, 2006

Summary

Ewing tumor family comprises bone and soft tissue cancers sharing a specific genetic translocation. Treatment involves multimodal chemotherapy, radiation, and surgery, with prognosis depending on tumor location and spread.

Area of Science:

Oncology
Genetics
Pathology

Context:

The Ewing tumor family includes bone, extraosseous, primitive neurectodermal, and Askin tumors.
These tumors share a characteristic reciprocal translocation (11; 22) (q24; q12) and originate from a common stem cell.
Ewing tumor is the most prevalent form, representing 60% of cases and is the second most common primary malignant bone tumor.

Purpose:

To outline the characteristics, presentation, and treatment of the Ewing tumor family.
To identify prognostic factors influencing patient outcomes.

Summary:

Ewing tumors commonly present with localized lesions (80%) or metastatic disease (20%), often manifesting as pain, swelling, or pathological fractures, particularly in long bones.
While extremities are common sites (50%), head/neck localizations are rare; paraspinal or pelvic tumors may cause back pain.

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Systemic symptoms like fever or weight loss can indicate metastatic disease, frequently involving the lungs, bone, and bone marrow.

Impact:

Multimodal treatment combining chemotherapy, radiation, and/or surgical resection offers the best therapeutic approach.
Favorable prognostic indicators include distal extremity or axial skeleton involvement.
Poor prognostic factors encompass proximal tumor sites, pelvic girdle involvement, metastatic disease, and low post-chemotherapy necrosis.