James Ewing first described the tumor that was to be named after him in the 1920s. Ewing sarcoma (ES) is a rare, extremely aggressive bone and soft-tissue cancer most common in the long bones and pelvis, but also occur rarely in the hand (<.2%of Ewing sacroma in hand).3 The most common location in the hand is the metacarpal of the long finger. Approximately 25% of patients have metastases at the time of diagnosis. Hand metastases typically occur in the lung. The diagnosis is largely based on genetic testing for characteristic fusion oncogenes expressed by the tumor. The best prognoses are associated with: location in the distal extremities, soft-tissue tumors, smaller tumors, patient age <15 years, female gender. Overall, outcomes have not improved in 20 years because the mechanism of metastasis is almost completely unknown. Clinical trial results for molecular targeted agents have been disappointing. Immunotherapy approaches show greater promise.
Pathophysiology/Pathogenetics
- Ewing sarcoma results from malignant transformation of stem and/or progenitor cells of mesoderm and neural crest lineages.
- EWS-ETS fusion proteins play a critical role in ES oncogenesis.
- Numerous targets of EWS-ETS–mediated transcriptional activity include a tumor suppressor gene (TGF beta type II receptor) and an inhibitor of insulin-like growth factor I (IGFBP-3).
- Non-transcriptional activity also is significant, with the proteins binding DNA across the genome.
- However, genetic approaches may not delineate the molecular basis of metastatic disease Ewing sarcoma.
Related Anatomy
- Primary sites of bone disease are:
- Lower extremity (41%)
- Pelvis (26%)
- Chest wall (16%)
- Upper extremity (9%)
- Spine (6%)
- Hand and foot (3%)
- Skull (2%)
- Most common soft-tissue sites are:
- Trunk (32%)
- Extremity (26%)
- Head and neck (18%)
- Retroperitoneum (16%)
- Other (9%)
Incidence
- The hand is affected in <0.2% of all (bone and soft-tissue) Ewing sarcoma.3
- Across all age groups in the United States, 1 case of ES occurs per 1 million people.
- About half of all cases occur between the ages of 10 and 20 years.
- ES occurs 9 times more often in whites than in African Americans.
- The gender division is 59% male and 41% female.
Differential Diagnosis
- Desmoplastic sarcoma
- Malignant lymphoma
- Neuroblastoma
- Poorly differentiated synovial sarcoma
- Rhabdomyosarcoma