Spinal intradural extraosseous Ewing's sarcoma

Author:

Mateen Farrah J.1,Nassar Aziza2,Bardia Aditya3,Jatoi Aminah4,Haddock Michael G.5,Buckner Jan C.4,Lachance Daniel H.6

Affiliation:

1. Department of Neurology, Johns Hopkins Hospital, Baltimore, MD;

2. Department of Pathology, Mayo Clinic; Mayo Clinic, Rochester, MN, USA

3. Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University; Mayo Clinic, Rochester, MN, USA

4. Department of Oncology, Mayo Clinic; Mayo Clinic, Rochester, MN, USA

5. Radiation Oncology, Mayo Clinic; Mayo Clinic, Rochester, MN, USA

6. Department of Neurology, Mayo Clinic, Rochester, MN, USA

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.

Publisher

SAGE Publications

Subject

Oncology,Histology

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