Primary spinal epidural extraosseous Ewing’s sarcoma: Report of five cases and literature review

Abstract

Ewing's sarcoma is the most common malignant bone tumour occurring in children and adolescents and exists in two different clinicopathological entities: osseous Ewing's sarcoma (OES) and extraosseous Ewing's sarcoma (EES). Five cases of primary epidural EES are described, which presented with non-specific symptoms leading to a long diagnostic delay. The median age at diagnosis was 22 years (range 13-36 years). The median diagnostic delay was 3 months. All patients had one or more neurological deficits. All underwent surgical exploration with a laminectomy and partial resection followed by adjuvant radiotherapy to a dose of 46-50 Gy and chemotherapy with VAC (vincristine, adriamycin and cyclophosphamide) alternating with ICE (ifosphamide, cisplatin and etoposide) for at least six cycles. The mean follow-up period is 21.2 months (range 11-32 months). Four of the five patients achieved a complete remission and are disease free at the time of writing this report. Two patients have a residual neurological deficit--both having presented with long history of neurological deficit. Primary spinal epidural EES should be suspected whenever young patients present with back pain and/or radicular pain, have abnormal neurology and an extradural mass is demonstrated on MRI. Surgical excision followed by adjuvant radiotherapy (50 Gy) and combination chemotherapy (VAC alternating with ICE) achieved local and systemic control in these patients. A greater number of patients and longer follow up are required to evolve a generally accepted treatment policy for this aggressive but potentially curable malignancy.

Pediatric Epidural Extra-Osseous Ewing’s Sarcoma - Extremely Rare Case Report and Review of the Literature

“…The male-female relationship is 1.5:1 in spinal epidural EES resembles that of bone (Figure 1-5). [17][18][19][20][21][22][23][24][25][26] The most common presentation is back pain, radicular pain, and paresis of unilateral or bilateral limbs, sensory disturbances, and bladder dysfunction. All these symptoms are related to compression of the cord, nerve roots and cauda equine as seen with other epidural tumors.…”

An undiagnosed chronic back pain may come out with severe morbidity in young patient, needs to be evaluated adequately: A case report and review of literature

“…Distant metastases occurred in nearly 40% of the cases. Lung, spine, and brain were the most frequent sites of metastasis [12][13][14][15] . Neurological deficit is most important determinant for deciding treatment of disease.…”

Ewing sarcoma of the thoracic epidural space in a young child

“…Persistent back pain and radiculopathy are common symptoms. We are only aware of 11 previous reports [2][3][4][5][6][7][8][9][10][11][12] in the literature of Ewing sarcoma of the epidural space in a child (Table 1), and the majority of these are reported in boys (seven cases) and at the lumbar vertebral level or thoraco-lumbar junction (eight cases). Our report of a 5-year-old girl with an upper thoracic lesion will be the first of its kind with regard to age, sex, and site of occurrence.…”