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LETTER TO EDITOR
Year : 2011  |  Volume : 59  |  Issue : 6  |  Page : 931-933

Acute cauda equina syndrome due to primary Ewing's sarcoma of the spine


Department of Orthopedics, Division of Spine and Musculoskeletal Oncology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Date of Submission04-Sep-2011
Date of Decision13-Nov-2011
Date of Acceptance04-Sep-2011
Date of Web Publication2-Jan-2012

Correspondence Address:
R Krishnakumar
Department of Orthopedics, Division of Spine and Musculoskeletal Oncology, Amrita Institute of Medical Sciences, Kochi, Kerala
India
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DOI: 10.4103/0028-3886.91393

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How to cite this article:
Krishnakumar R, Renjitkumar J. Acute cauda equina syndrome due to primary Ewing's sarcoma of the spine. Neurol India 2011;59:931-3

How to cite this URL:
Krishnakumar R, Renjitkumar J. Acute cauda equina syndrome due to primary Ewing's sarcoma of the spine. Neurol India [serial online] 2011 [cited 2014 Sep 19];59:931-3. Available from: http://www.neurologyindia.com/text.asp?2011/59/6/931/91393


Sir,

Ewing's sarcoma family of tumors (ESFT) are a group of small round cell tumors of neuroectodermal origin and includes Ewing's sarcoma of the bone, extraskeletal Ewing's sarcoma, primitive neuroectodermal tumor, and Askin tumor. [1] Primary Ewing's sarcoma of the spine is rare. We report a case of primary Ewing's sarcoma of the spine presenting with acute cauda equina syndrome.

A 20-year-old man presented with acute onset of weakness of left lower limb and urinary retention. On examination he had motor weakness (Grade 3/5) of left lower limb with absent knee and ankle jerks and saddle hypoesthesia. Magnetic resonance imaging (MRI) of the spine showed a lesion involving the L3 vertebral body, left pedicle and facet with epidural mass severely compressing the thecal sac. The lesion showed irregular enhancement on contrast-enhanced T1-weighted images [Figure 1]a-c. Patient underwent L3 laminectomy and decompression of the spinal roots. Tumor mass was seen around the left L3 nerve root extending to the neural foramen. Histopathology of the tumor showed small round blue cells with positive Periodic acid-Schiff (PAS) staining [Figure 2]a and b. The immunohistochemical analysis revealed strong perimembranous staining with CD-99 (MIC2) [Figure 2]c. Reverse transcription-polymerase chain reaction analysis confirmed the presence of an EWS-FLI1 translocation, t (11; 22). The final diagnosis was primary Ewing's sarcoma of the spine. The patient underwent postoperative chemoradiation and his motor power improved to Grade 4/5 with return of normal bladder function.
Figure 1a: T2 sagittal MRI showing tumor mass compressing the spinal cord
Figure 1b: T2 axial MRI showing epidural mass severely compressing the spinal cord (arrows) with lateral extension to the facet and neural foramen
Figure 1c: T1 coronal MRI showing contrast enhancement (arrows)


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Figure 2a: The microphotograph of biopsy specimen showing small round cells with scanty cytoplasm (hematoxylin and eosin [H and E] stain)
Figure 2b: The microphotograph of biopsy specimen showing cells with PAS positivity
Figure 2c: The immunohistochemical analysis showing strong perimembranous staining with CD-99 (MIC2)


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Primary Ewing's sarcoma of the spine is rare and the use of multiagent chemotherapy combined with surgery or radiation therapy for definitive local control could increase disease-free survival rates to 50-80%. [2],[3],[4]

 
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1.Delattre O, Zucman J, Melot T, Garau XS, Zucker JM, Lenoir GM, et al. The Ewing family of tumors-a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med 1994;331:294-9.  Back to cited text no. 1
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2.Nesbit ME Jr, Gehan EA, Burgert EO Jr, Vietti TJ, Cangir A, Tefft M, et al. Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: A longterm follow-up of the First Intergroup study. J Clin Oncol 1990;8:1664-74.  Back to cited text no. 2
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3.Razek A, Perez CA, Tefft M, Nesbit M, Vietti T, Burgert EO Jr, et al. Intergroup Ewing's Sarcoma Study: Local control related to radiation dose, volume, and site of primary lesion in Ewing's sarcoma. Cancer 1980;46:516-21.  Back to cited text no. 3
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4.Rosen G, Caparros B, Nirenberg A, Marcove RC, Huvos AG, Kosloff C, et al. Ewing's sarcoma: Ten-year experience with adjuvant chemotherapy. Cancer 1981;47:2204-13.  Back to cited text no. 4
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