Ewing sarcoma of the thoracic epidural space in a young child.
OBJECTIVE
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.
Department of Orthopedic Surgery, School of Medicine, Shinshu University, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan.
Shinsuke
S
Kobayashi
Jun
J
Takahashi
Kazuo
K
Sakashita
Mana
M
Fukushima
Hiroyuki
H
Kato
D002675:Child, Preschool; D015174:Epidural Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D012512:Sarcoma, Ewing / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D013904:Thoracic Vertebrae
C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS