From the radiologic pathology archives: Ewing sarcoma family of tumors: Radiologic-pathologic correlation

Mark D. Murphey, Lien T. Senchak, Pramod K. Mambalam, Chika I. Logie, Mary K. Klassen-Fischer, Mark J. Kransdorf

Research output: Contribution to journalArticle

74 Scopus citations

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 lesion s 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.

Original languageEnglish (US)
Pages (from-to)803-831
Number of pages29
JournalRadiographics
Volume33
Issue number3
DOIs
StatePublished - May 1 2013

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

Fingerprint Dive into the research topics of 'From the radiologic pathology archives: Ewing sarcoma family of tumors: Radiologic-pathologic correlation'. Together they form a unique fingerprint.

  • Cite this

    Murphey, M. D., Senchak, L. T., Mambalam, P. K., Logie, C. I., Klassen-Fischer, M. K., & Kransdorf, M. J. (2013). From the radiologic pathology archives: Ewing sarcoma family of tumors: Radiologic-pathologic correlation. Radiographics, 33(3), 803-831. https://doi.org/10.1148/rg.333135005