Primary Extraskeletal Ewing Sarcoma of the Thoracic Spinal Epidural Space: A Case Report

Yasuhiro Yamamoto,Makoto Kuroda,Masatake Matsubara,Daisuke Ishimura,Osuke Washimi,Mitsutosi Uchibori,Masahiro Yonekawa,Harumoto Yamada

doi:10.4137/cmo.s332

Abstract

Cases of extraskeletal Ewing sarcoma (EES) originating primarily within the spinal epidural space, are very rare and have a very poor prognosis. There is no standard therapy for this disease. We report the case of a 23-year-old man presenting with symptoms of back pain and numbness of both legs for 10 days. Imaging studies revealed a dorsal soft-tissue, extradural mass at the T8–9 vertebral level. The patient underwent a laminectomy and complete excision of the tumor. The EES diagnosis was confirmed by histologic analysis including immunohistochemistry and by presence of the EWS-ERG due to the t (21: 22) (q22: q12) chromosomal translocation by a reverse transcriptase-polymerase chain reaction (RT-PCR). This is the first report of spinal epidural EES with presence of the EWS-ERG fusion transcript. Post-operatively, the patient received aggressive adjuvant chemotherapy and radiotherapy. At 63 months after surgery, the patient is without clinical or radiological evidence of recurrent or metastatic disease. Early discovery of EES and a complete resection followed by the aggressive treatment with radiation and chemotherapy may improve disease-free and overall survival.

Highlights

Ewing sarcoma (ES) and peripheral primitive neuroectodermal tumor are closely related malignant, small, round cell tumors, and usually found in long bones
Primary spinal epidural extraskeletal Ewing sarcoma (EES) should be suspected whenever young patients present with back pain and/or radicular pain, have abnormal neurological findings and an extradural mass is detected on Magnetic resonance imaging (MRI)
A 23-year-old man complained of back pain which had lasted for 10 days followed by the onset of numbness in the anterior and posterior aspect of both legs for 2 days prior to presentation at our institution

Summary

Introduction

Ewing sarcoma (ES) and peripheral primitive neuroectodermal tumor (pPNET) are closely related malignant, small, round cell tumors, and usually found in long bones. Based upon reports in the literature, it is exceptionally rare to find them originating primarily within the spinal epidural space; only 34 such cases have been reported to date [1, 5, 7,8,9,10]. Primary spinal epidural EES should be suspected whenever young patients present with back pain and/or radicular pain, have abnormal neurological findings and an extradural mass is detected on MRI. In ES/pPNET, the detection of the chromosomal translocation by RT-PCR is useful for making an accurate diagnosis, in addition to the usual histochemical stains and immunohistochemistry

Case Report

Findings

Discussion