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CASE REPORT
Year : 2007  |  Volume : 2  |  Issue : 1  |  Page : 18-19
 

Acute presentation of epidural Ewing's sarcoma


1 Department of Neurology, Himalayan Institute of Medical Sciences, Dehradun (UA), India
2 Department of Neurosurgery, Himalayan Institute of Medical Sciences, Dehradun (UA), India
3 Department of Pathology, Himalayan Institute of Medical Sciences, Dehradun (UA), India

Correspondence Address:
D Goel
B-27, HIMS, Swami Ram Nagar, Dehradun (UA)
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.32001

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   Abstract 

Acute transverse myelitis is most common in postviral infections, post-immunization or is associated with multiple sclerosis. Primary central nervous system lymphoma very rarely involves spinal cord and its presentation as myelitis is more uncommon. This case is a rare presentation of a rare disease.


Keywords: Childhood, epidural cord compression, sarcoma


How to cite this article:
Goel D, Singhal A, Gupta C, Kishor S, Anuradha K. Acute presentation of epidural Ewing's sarcoma. J Pediatr Neurosci 2007;2:18-9

How to cite this URL:
Goel D, Singhal A, Gupta C, Kishor S, Anuradha K. Acute presentation of epidural Ewing's sarcoma. J Pediatr Neurosci [serial online] 2007 [cited 2020 Oct 25];2:18-9. Available from: https://www.pediatricneurosciences.com/text.asp?2007/2/1/18/32001



   Introduction Top


Epidural spinal cord compression (ESCC) refers to compression of spinal cord or cauda-equina, most commonly due to cancer as a primary cause. [1] Other benign etiologies like prolapsed intervertebral disc or epidural abscess present as acute neurological syndrome. On the other hand, presentation in neoplastic diseases is slowly progressive back pain followed by paraplegia and bladder dysfunction. [2] The commonest malignancies involving the spinal epidural space are metastatic lymphomas, nerve sheath tumor, meningiomas, hemangiomas and metastases from systemic cancers. [2] Rare round cell cancers which can present with ESCC are primary central nervous system lymphoma and granulocytic sarcoma and extra-osseous Ewing sarcoma (EOES). [2],[3],[4] Here we report this case of EOES presenting with uncommon acute-onset-myelitis-like illness. The rare presentation of this rare disease is described with literature review.


   Case Report Top


A 15-year-old female presented with abrupt onset of weakness in both lower limbs. She had fever for 10 days about a month prior to this episode, which responded to the symptomatic treatment. She also developed retention of urine and required urinary catheterization on admission. On examination, she had grade 0 power in both lower limbs with hypotonia and areflexia. There was transverse sensory level below nipple line and absent abdominal reflexes bilaterally. The magnetic resonance (MR) imaging findings demonstrated an epidural mass extending from D3 to D6 spines [Figure - 1], suggestive of an epidural abscess. Her total leukocyte count was 13,660 with 90% polymorphs and 10% lymphocytes. Her hemoglobin level was 9.2 gm% and the erythrocyte sedimentation rate was 64 mm/h. She underwent surgical decompression of the spinal cord by D3 to D6 laminectomy and complete excision of the necrotic mass. The histology showed a moderate sized round cell tumor with morphology consistent with Ewing's sarcoma [Figure - 2], which was periodic acid-Schiff (PAS) positive and also immuno-positive to Mic-2 and Chromogranin but negative for synoptophysin, favoring the diagnosis of Ewing's sarcoma. She was also treated with radiotherapy and chemotherapy postoperatively. However, she continued to deteriorate despite treatment and eventually died after 4 months.


   Discussion Top


EOES is an undifferentiated small round-to-oval cell tumor of uncertain origin. Clinical information about the disease is based on various case reports. EOES is very uncommon, and only 21 cases have been reported in the literature [4],[5],[6],[7],[8] so far. It differs in presentation from skeletal Ewing's sarcoma in several respects. The average age of occurrence is around 17 years (range 4-47 years), in contrast to 10 years for skeletal Ewing's sarcoma. EOES, like skeletal Ewing's sarcoma, occurs more commonly in males than females, with a ratio of 2:1. Symptomatology usually includes back pain and/or radicular pain (100%), paresis of one or both legs (83%), sensory disturbances and bladder and bowel dysfunction. Most of the patients present with insidious onset neurological deficits. Rarely acute presentation as in our case has been reported.

Lumbar spine is most commonly affected, and cervical involvement is rare. On MR these lesions commonly appear isointense to muscle on T1-weighted images, hyperintense on T2-weighted images and show moderate enhancement on contrast-enhanced T1-weighted images without bony involvement. Microscopically, the lesion is composed of sheets of fairly uniform round-to-oval cells. Usually the cells have relatively clear and indistinct cytoplasm but contain large quantities of PAS-positive, diastase-digestible material, indicating high concentrations of glycogen. The tumor cells show diffuse, intense membrane reactivity for CD99 (MIC 2) on immuno-histochemical staining. Wide surgical resection with follow-up local irradiation and chemotherapy offer the best chance for long-term disease-free survival.

Two types of chemotherapeutic treatment have been recommended - mesna, adriamycin, ifosfamide, dacarbazine [MAID] regimen; and cyclophosphamide, vincristine, adriamycin, dacarbazine [CYVADIC] regimen. Our case had unique features of very short history of 10 days and acute presentation with rapid deterioration and early death. Overall mean follow-up of these patients is available for 18 months, with the longest follow-up of 4 years of disease remission. About 50% of patients died in spite of combination therapy. Clinical, radiological or histological predictive features for poor prognosis are still unknown.

 
   References Top

1.Cavaliere R, Schiff D. Epidural spinal cord compression. Curr Treat Options Neurol 2004;6:285-95.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Kawasaki K, Wakabayashi K, Koizumi T, Tanaka R, Takahashi H. Spinal cord involvement of primary central nervous system lymphomas: Histopathological examination of 14 autopsy cases. Neuropathology 2002;22:13-8.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Kalayci M, Sumer M, Yenidunya S, Ozdolap S, Acikgoz B. Spinal granulocytic sarcoma (chloroma) presenting as acute cord compression in a non-leukemic patient. Neurol India 2005;53:221-3.  Back to cited text no. 3    
4.Shin JH, Lee HK, Rhim SC, Cho KJ, Choi CG, Suh DC. Spinal epidural extraskeletal Ewing sarcoma: MR findings in two cases. Am J Neuroradiol 2001;22:795-8.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Gandhi D, Goyal M, Belanger E, Modha A, Wolffe J, Miller W. Primary epidural Ewing's sarcoma: Case report and review of literature. Can Assoc Radiol J 2003;54:109-13.  Back to cited text no. 5    
6.Kadri PA, Mello PM, Olivera JG, Braga FM. Primary lumbar epidural Ewing's sarcoma: Case report. Arq Neuropsiquiatr 2002;60:145-9.   Back to cited text no. 6    
7.Allam K, Sze G. MR of primary extraosseous Ewing sarcoma. AJNR Am J Neuroradiol 1994;15:305-7.   Back to cited text no. 7    
8.Kaspers GJ, Kamphorst W, van de Graaff M, van Alphen HA, Veerman AJ. Primary spinal epidural extraosseous Ewing's sarcoma. Cancer 1991;68:648-54.  Back to cited text no. 8    


    Figures

  [Figure - 1], [Figure - 2]


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3 Spinal epidural metastasis from Ewingæs sarcoma on PET/CT: A case report
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