Two Cases of Spinal, Extraosseous, Intradural Ewing's sarcoma/Peripheral Neuroectodermal Tumor: Radiologic, Pathologic, and Molecular Analysis.

Authors

Stacey K. Mardekian, Department of Pathology, Thomas Jefferson University HospitalFollow
Ashish Gandhe, MD, Department of Radiology, Thomas Jefferson University HospitalFollow
Markku Miettinen, Department of Laboratory of Pathology, National Cancer InstituteFollow
Svetlana Pack, Department of Laboratory of Pathology, National Cancer Institute
Mark T. Curtis MD, PhD, Department of Pathology, Thomas Jefferson University HospitalFollow
Ziedulla Abdullaev, Department of Laboratory of Pathology, National Cancer Institute

Document Type

Article

Publication Date

1-30-2014

Comments

This article has been peer reviewed. It was published in: Journal of Clinical Imaging Science.

The published version is available at DOI: 10.4103/2156-7514.126050

Copyright © 2014 Mardekian SK.

Abstract

Extraosseous Ewing's sarcoma/peripheral neuroectodermal tumors (ES/PNETs) are rare neoplasms that account for approximately 10%-15% of soft tissue sarcomas in children and 5% of soft tissue sarcomas in adults. Primary spinal, extraosseous, intradural ES/PNETs are even less common. The diagnosis of ES/PNET is extremely challenging, because the tumor can have a nonspecific radiologic appearance, and the histologic features are shared by many other "small round cell tumors." Thus, ES/PNET should be included in the radiologic and pathologic differential diagnosis, even in older patients and in unusual tumor sites. We report two cases of spinal, extraosseous, intradural ES/PNETs in adults who presented with back pain. Magnetic resonance imaging revealed contrast enhancing, intradural lesions in the area of the conus medullaris. The tumor in Case 1 was partially intramedullary, while the tumor in Case 2 was exclusively extramedullary. In both cases, the radiologic and intraoperative surgical impression favored ependymoma. The diagnosis of ES/PNET was established in both cases by histopathologic, immunohistochemical, and molecular analysis.

Recommended Citation

Mardekian, Stacey K.; Gandhe, MD, Ashish; Miettinen, Markku; Pack, Svetlana; Curtis, Mark T. MD, PhD; and Abdullaev, Ziedulla, "Two Cases of Spinal, Extraosseous, Intradural Ewing's sarcoma/Peripheral Neuroectodermal Tumor: Radiologic, Pathologic, and Molecular Analysis." (2014). Department of Radiology Faculty Papers. Paper 36.
https://jdc.jefferson.edu/radiologyfp/36

PubMed ID

24678438