Spinal intradural extraosseous Ewing's sarcoma

Farrah J. Mateen, Aziza Nassar, Aditya Bardia, Aminah Jatoi, Michael G. Haddock, Jan C. Buckner, Daniel H. Lachance

Research output: Contribution to journalArticle

9 Scopus citations

Abstract

Extraosseous Ewing's sarcoma (EES) involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuro-ectodermal 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.

Original languageEnglish (US)
Pages (from-to)21-24
Number of pages4
JournalRare Tumors
Volume3
Issue number1
DOIs
StatePublished - Mar 14 2011

Keywords

  • Extraosseous ewing's sarcoma
  • Intradural
  • Meninges
  • Spinal cord

ASJC Scopus subject areas

  • Histology
  • Oncology

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