Abstract
Purpose
To report on the clinical course and treatment of Ewing sarcoma of the thoracic epidural space in a 5-year-old girl.
Methods
We present the case of a 5-year-old girl who experienced back pain (day 1); on day 10, the pain had exacerbated and involuntary movements in the lower limbs occurred, and an MRI performed in her local hospital revealed a tumor lesion at the upper thoracic level.
Results
On day 13, emergency surgery was performed for partial resection of the tumor. Pathological examination of the resected tumor by immunostaining and gene testing revealed that it was MIC2 positive and an EWS-FLI 1 chimera, respectively, and Ewing sarcoma was diagnosed. The involuntary movements resolved immediately after the surgery. Three weeks after the operation, chemotherapy and radiation therapy were commenced. After 5 months, deep tendon reflexes recovered to normal. MRI showed that the tumor has not recurred at 29 months after surgery.
Conclusions
The majority of epidural patients undergo emergency surgery only after symptom exacerbation, which includes the development of neurological deficits. Thus, preoperative diagnosis of Ewing sarcoma of the epidural space is difficult and diagnosis is frequently made by a post-operative gene test. The resection area is limited to the intralesional margin area because a larger resection is difficult due to the characteristics of the affected region; thus, there is a higher possibility of recurrence and careful follow-up of the case is necessary.
References
Pizzo PA, Poplack DG (2002) Principles and practice of pediatric oncology 4th edn: 1193–1195
Klimo P Jr, Codd PJ, Grier H, Goumnerova LC (2009) Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr 4:222–229
Dogan S, Leković GP, Theodore N, Horn EM, Eschbacher J, Rekate HL (2009) Primary thoracolumbar Ewing’s sarcoma presenting as isolated epidural mass. Spine J 9:e9–e14
Song X, Choi J, Rao C, Nallu S, Nicastri AD (2008) Primary Ewing sarcoma of lumber spine with massive intraspinal extension. Pediatr Neurol 38:58–60
Kogawa M, Asazuma T, Iso K, Koike Y, Domoto H, Aida S, Fujikawa K (2004) Primary cervical spinal epidural extra-osseous Ewing’s sarcoma. Acta Neurochir 146:1051–1053
Mukhopadhyay P, Gairola M, Sharma M, Mukhopadhyay P, Gairola M, Sharma M (2001) Primary spinal epidural extraosseous Ewing’s sarcoma. Australas Radiol 45:372–379
Kaspers GJ, Kamphorst W, van de Graaff M, van Alphen HA, Veerman AJ (1991) Primary spinal epidural extraosseous Ewing’s sarcoma. Cancer 68:648–654
Machin Valtueña M, Garcia-Sagredo JM, Muñoz Villa A, Lozano Giménez C, Aparicio Meix JM (1987) 18q-syndrome and extraskeletal Ewing’s sarcoma. J Med Genet 24:426–428
Spaziante R, de Divitiis E, Giamundo A, Gambardella A, Di Prisco B (1983) Ewins’s sarcoma arising primary in the spinal epidural space: fifth case report. Neurosurgery 12:337–341
Kinsella TJ, Triche TJ, Dickman PS, Costa J, Tepper JE, Glaubiger D (1983) Extraskeletal Ewing’s sarcoma: results of combined modelity treatment. J Clin Oncol 1:489–495
Simonati A, Vio M, Iannucci AM, Bricolo A, Rizzuto N (1981) Lumbar epidural Ewing sarcoma. Light and electron microscopic investigation. J Neurol 225:67–72
Tefft M, Vawter GF, Mitus A (1969) A paravertebral “round cell” tumors in children. Radiology 92:1501–1509
Bacci G, Ferrari S, Bertoni F et al (2000) Prognostic factors in nonmetastatic Ewing’s sarcoma of bone treated with adjuvant chemotherapy: analysis of 359 patient at the Institute Ortopedico Rizzoli. J Clin Oncol 18:4–11
Paulussen M, Ahrens S, Dunst J et al (2001) Localized Ewing tumor of bone: final results of the cooperative Ewing’s Sarcoma Study CESS 86. J Clin Oncol 19:1818–1829
Cotterill SJ, Ahrens S, Paulussen M, Jürgens HF, Voûte PA, Gadner H, Craft AW (2000) Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European intergroup Cooperative Ewing’s Sarcoma study groups. Clin Oncol 18:3108–3114
Shankar AG, Ashley S, Craft AW, Pinkerton CR (2003) Outcome after relapse in an unselected cohort of children and adolescents with Ewing Sarcoma. Med Pediatr Oncol 40:141–147
Kasper B, Lehnert T, Bernd L, Mechtersheimer G, Goldschmidt H, Ho AD, Egerer G (2004) Autografting for sarcoma: high-dose chemotherapy with autologous peripheral blood stem cell transplantation for bone and soft-tissue sarcomas. Bone Marrow Transplant 34:37–41
Conflict of interest
None of the authors has any potential conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kobayashi, S., Takahashi, J., Sakashita, K. et al. Ewing sarcoma of the thoracic epidural space in a young child. Eur Spine J 22 (Suppl 3), 373–379 (2013). https://doi.org/10.1007/s00586-012-2481-y
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00586-012-2481-y