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Spinal intradural extraosseous Ewing’s sarcoma | Mateen | Rare Tumors
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Spinal intradural extraosseous Ewing’s sarcoma

Farrah Mateen, Aziza Nassar, Aditya Bardia, Aminah Jatoi, Michael Haddock, Jan Buckner, Daniel Lachance

Abstract


Extraosseous Ewing’s sarcoma (EES) involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET) by identification of the chromosomal translocation (11;22)(q24;q12). We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970-2009). Reverse transcriptase polymerase chain reaction (RT-PCR) identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months) or death (4 years). This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.

Keywords


Chemotherapy, Ewing's Sarcoma, Neurology, Spinal Cord

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DOI: http://dx.doi.org/10.4081/rt.2011.e7
Submitted: 2010-07-17 01:42:35
Published: 2011-03-09 11:03:13
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Rare Tumors [ISSN 2036-3605, eISSN 2036-3613] is an Open Access, peer-reviewed journal published by PAGEPress, Pavia, Italy. All credits and honors to PKP for their OJS.

 

 

 

 
 
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