AJDRAJNR - American Journal of Neuroradiology

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Case Report
PEDIATRICS

Central Nervous System Extraosseous Ewing Sarcoma: Radiologic Manifestations of This Newly Defined Pathologic Entity

J.S. Pekalaa, S. Gururanganb, J.M. Provenzalea and S. Mukundan, Jr.a,c

a Department of Radiology and The Brain Tumor Center at Duke, Durham, NC
b Department of Pediatrics, Duke University Medical Center, Durham, NC
c Department of Biomedical Engineering, Duke University, Durham, NC

Address correspondence to Srinivasan Mukundan, Jr., PhD, MD, Duke University Medical Center, Durham, NC 27710

Summary: Although these entities are histologically similar, recent advances in molecular genetics have allowed the distinction of central nervous system extraosseous Ewing sarcoma (CNS-EES) from central primitive neuroectodermal tumors (c-PNET) including medulloblastoma and supratentorial PNET. We present 2 cases of pathologically confirmed CNS-EES. Knowledge of CNS-EES as a distinct entity enables the neuroradiologist to suggest the proper diagnosis and the need for special immuno-histochemical and molecular studies to confirm the diagnosis. Because treatment and prognosis are vastly different, the proper diagnosis of CNS-EES versus c-PNET is critical.




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M. E. Miller, L. Emerson, F. Clayton, B. G. Bentz, R. E. Data, K. L. Salzman, L. M. Smith, and M. K. Yu
Extraosseous Ewing's Sarcoma
J. Clin. Oncol., October 20, 2007; 25(30): 4845 - 4848.
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