open access
Intradural extramedullary Ewing's sarcoma: A case report and review of the literature
Affiliations- Department of Neurosurgery, University Hospital of Larissa, Larissa, Greece
- Department of Pathology, University Hospital “AHEPA”, Thessaloniki, Greece
- Department of Radiology, University Hospital of Larissa, Larissa, Greece
open access
Abstract
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 reportA 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.
ConclusionThe 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
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 reportA 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.
ConclusionThe 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.
Keywords
IEES, MRI, RT-PCR, GFAP, EMA, EWS/FLI1, RT
About this articleTitle
Intradural extramedullary Ewing's sarcoma: A case report and review of the literature
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
106-110
Page views
635
Article views/downloads
885
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
