From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation.

Publication/Presentation Date

5-1-2013

Abstract

The Ewing sarcoma family of tumors includes osseous Ewing sarcoma, extraskeletal Ewing sarcoma, primitive neuroectodermal tumor, and Askin tumor. They share a karyotype abnormality with translocation involving chromosomes 11 and 22. Histologically, these lesions demonstrate crowded sheets of small round blue cells. Imaging features of osseous Ewing sarcoma often suggest the diagnosis, with aggressive long-bone destruction in the metadiaphysis of an adolescent or young adult and an associated soft-tissue mass. Focal areas of cortical destruction are frequent, allowing continuity between the intraosseous and extraosseous components. This continuity is also commonly seen as subtle channels extending through the cortex at computed tomography or magnetic resonance (MR) imaging, a finding that reflects the underlying pathologic appearance. Extraskeletal Ewing sarcoma commonly demonstrates a nonspecific radiologic appearance of a large soft-tissue mass affecting the paraspinal region or lower extremity. Askin tumor represents extraskeletal Ewing sarcoma involving the chest wall. Imaging typically reveals a large pleural-based mass and associated pleural effusion. Treatment of these tumors is usually a combination of neoadjuvant chemotherapy followed by surgical resection, which may be supplemented with radiation therapy. Imaging, particularly MR, is also vital to evaluate response to neoadjuvant therapy, direct surgical resection, and detect local recurrence or metastatic disease.

Volume

33

Issue

3

First Page

803

Last Page

831

ISSN

1527-1323

Disciplines

Diagnosis | Medicine and Health Sciences | Other Analytical, Diagnostic and Therapeutic Techniques and Equipment | Radiology

PubMedID

23674776

Department(s)

Department of Radiology and Diagnostic Medical Imaging

Document Type

Article

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