open access

Vol 51, No 1 (2017)
Case reports
Submitted: 2015-11-22
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Intradural extramedullary Ewing's sarcoma: A case report and review of the literature

Konstantinos Paterakis, Alexandros Brotis, Anastasia Tasiou, Vasiliki Kotoula, Eftychia Kapsalaki, Marianna Vlychou
DOI: 10.1016/j.pjnns.2016.11.006
·
Neurol Neurochir Pol 2017;51(1):106-110.

open access

Vol 51, No 1 (2017)
Case reports
Submitted: 2015-11-22

Abstract

Introduction

Extra-skeletal Ewing's sarcomas are very rare lesions to the spine surgeon, with the intradural, extramedullary lesions being even rarer. Herein we present a patient with an intradural, extramedullary form of Ewing's sarcoma and review the relevant literature. The medical records, operative reports, radiographical studies and histological examinations of a single patient are retrospectively reviewed.

Case report

A 31-year old male presented with back-pain, right-leg progressive paraparesis, and inability to walk. Both motor and sensory disturbances were revealed on the right leg at the clinical examination. Lumbar MRI showed two lesions. The first one was an intradural, extramedullary lesion at the L2-L3 level, while the second was smaller, located at the bottom of the dural sac. The patient underwent gross total resection of the L2-L3 lesion after a bilateral laminectomy. Histological examination was compatible with Ewing's sarcoma, and was verified by molecular analysis. No other extra-skeletal or skeletal lesion was found. A chemotherapy scheme was tailored to the patients’ histological diagnosis. The patient presented with local recurrence and bone metastasis 2 years after his initial diagnosis. A second operation was performed and the follow up of the patient showed no disease progression 18 months after revision surgery.

Conclusion

The spine surgeon should be aware of the existence of such rare entities, in order to timely fulfill the staging process and institute the proper therapy. The management of patients with extra-skeletal Ewing's sarcomas involves professionals as members of a multidisciplinary team, all of which should co-operate for the patient's optimal outcome.

Abstract

Introduction

Extra-skeletal Ewing's sarcomas are very rare lesions to the spine surgeon, with the intradural, extramedullary lesions being even rarer. Herein we present a patient with an intradural, extramedullary form of Ewing's sarcoma and review the relevant literature. The medical records, operative reports, radiographical studies and histological examinations of a single patient are retrospectively reviewed.

Case report

A 31-year old male presented with back-pain, right-leg progressive paraparesis, and inability to walk. Both motor and sensory disturbances were revealed on the right leg at the clinical examination. Lumbar MRI showed two lesions. The first one was an intradural, extramedullary lesion at the L2-L3 level, while the second was smaller, located at the bottom of the dural sac. The patient underwent gross total resection of the L2-L3 lesion after a bilateral laminectomy. Histological examination was compatible with Ewing's sarcoma, and was verified by molecular analysis. No other extra-skeletal or skeletal lesion was found. A chemotherapy scheme was tailored to the patients’ histological diagnosis. The patient presented with local recurrence and bone metastasis 2 years after his initial diagnosis. A second operation was performed and the follow up of the patient showed no disease progression 18 months after revision surgery.

Conclusion

The spine surgeon should be aware of the existence of such rare entities, in order to timely fulfill the staging process and institute the proper therapy. The management of patients with extra-skeletal Ewing's sarcomas involves professionals as members of a multidisciplinary team, all of which should co-operate for the patient's optimal outcome.

Get Citation

Keywords

IEES, MRI, RT-PCR, GFAP, EMA, EWS/FLI1, RT

About this article
Title

Intradural extramedullary Ewing's sarcoma: A case report and review of the literature

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 51, No 1 (2017)

Pages

106-110

DOI

10.1016/j.pjnns.2016.11.006

Bibliographic record

Neurol Neurochir Pol 2017;51(1):106-110.

Keywords

IEES
MRI
RT-PCR
GFAP
EMA
EWS/FLI1
RT

Authors

Konstantinos Paterakis
Alexandros Brotis
Anastasia Tasiou
Vasiliki Kotoula
Eftychia Kapsalaki
Marianna Vlychou

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