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Case Reports
. 2022 Jan 24;8(1):13.
doi: 10.1038/s41394-021-00474-7.

Primary extra-skeletal Ewing's sarcoma presenting as an epidural Soft Tissue Lesion causing cauda equina syndrome in an adolescent girl: a case report

Abhishek Soni  1 , Shaffaf Abdul Kareem  1 , Manoj Kumar Ramachandraiah  1 , Ajay Kumar Shetty Papanna  1 , Vidyadhara S  2
Affiliations
Case Reports

Primary extra-skeletal Ewing's sarcoma presenting as an epidural Soft Tissue Lesion causing cauda equina syndrome in an adolescent girl: a case report

Abhishek Soni et al. Spinal Cord Ser Cases. .

Abstract

Introduction: Primary epidural Ewing's sarcoma in the lumbar spinal canal is a rare condition and very few cases are reported in the literature.

Case report: A fifteen-year-old girl presented with low backache associated with sudden onset of weakness and radiculopathy of both lower limbs for 10 days, bowel and bladder involvement for 3 days. Physical examination revealed grade 0/5 power and absent sensations below L4 dermatomal level and perianal region (ASIA A). Plantar reflex was mute bilaterally. Magnetic resonance imaging revealed an extradural lesion within the spinal canal at the L3-L4 level. The patient underwent an emergency posterior decompression, extradural lesion excision and instrumented stabilization L3-L5. Based on histopathological examination of the tissue specimen, we diagnosed the lesion as Ewing sarcoma.

Discussion: Primary extra-skeletal Ewing's sarcoma presenting as an epidural lesion in the lumbar spine is a rare clinical entity that should be considered as a differential for spinal epidural lesions. Treatment for such cases is almost always an early surgical intervention due to its rapid onset and compressive neurological symptoms. Wide decompression with instrumented fusion and excision of the lesion followed by chemo and radiotherapy are recommended.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1. Radiographic findings.
Sagittal T2W (A) and mDixon (B) MRI images of the lumbar spine showing hypointense lesion at L3-L4 level causing significant canal compromise.
Fig. 2
Fig. 2. Radiographic findings.
Axial T2W (A), and Coronal (B) MRI images showing the lesion completely obliterating the spinal canal and displacing filum terminale and nerve roots anteriorly.
Fig. 3
Fig. 3. Histological findings.
H&E stain specimen showing atypical cells composed of small round cells having a high N:C ratio with moderate nuclear pleomorphism.
Fig. 4
Fig. 4. Histological findings.
Immunohistochemical staining shows neoplastic cells which are positive for CD99 (A), NKX2-2 (B), and FLI1 (C).
See this image and copyright information in PMC

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