A case report on non‐metastatic Ewing sarcoma of the lumbar spine in a young patient

Abstract

Background: Ewing sarcoma (ES), the second most common malignant bone tumor after osteosarcoma in the second decade, occurs in 0.9% of cases as the primary non-sacral form.Case: A 20-years-old male presented with acute paraparesis of bilateral lower limb and numbness following initial back pain for the last 6 months. Magnetic resonance imaging (MRI) of the lumbar spine revealed a 4 cm enhancing soft tissue mass at the L4/L5 vertebra extending into the spinal canal with compression of the thecal sac.The computed tomography (CT) of the chest, abdomen, and pelvis revealed aggressive lytic lesions in the L4 spinous process with soft tissue extension into the spinal canal with no other site of distant metastasis. He was treated with IV steroids (Injection dexamethasone 10 mg IV followed by 4 mg tablet dexamethasone q6h; subsequently tapered off). A core needle biopsy showed a small, round blue cell neoplasm, (suggestive of a primitive neuroectodermal) stained positive for CD99 and vimentin stain. The diagnosis of ES lumbar spine was made which was treated with surgical resection with an appropriate margin measuring 8 Â 4.5 Â 2.5 cm with decompression and L4/5 laminectomies, which had a negative margin in the surgical pathology report. Concomitant local radiotherapy and chemotherapy [cycles of vincristine 2 mg/m 2 , adriamycin/doxorubicin 75 mg/m 2 , cyclophosphamide 1200 mg/m 2 (VDC) with mesna rescue alternating with cycles of ifosfamide 1800 mg/m 2 and etoposide 100 mg/m 2 (IE)] was started. The motor strength was regained gradually with preserved spine biomechanics and oncological control with no recurrence in 2-year follow-ups. Conclusions:The presentation of lumbar ES can vary from local pain and swelling to acute paraparesis. Timely diagnosis and treatment with multimodal therapy, namely, steroids for acute spinal cord compression and surgery with chemoradiotherapy for ES can improve spinal biomechanics and oncological control.

Post-Radiotherapy Complications in Ewing Sarcoma: A Case Report and Literature Review

“…Subsequently, the finding of the t(11; 22) translocation led to widespread suspicions of a neural origin. Finally, an epithelial origin was inferred from the erratic expression of cytokeratin and tight junction proteins [3,4,[7][8][9][10][11][12][13]. Additionally, spinal cord compression-related neurological impairments may cause patients to present later than expected.…”

“…It primarily impacts children and adolescents. With 95% of the population under 25, it is characterized by high rates of development and growth [1][2][3][4][5][6][7][8]. Males are 1.5 times more inclined than females to develop it [1,5,8].…”

“…The European Intergroup Cooperative Ewing Sarcoma Studies (EI-CESS) found that 54% of ES cases occurred in the axial skeleton, 42% in the appendicular skeleton, and the rest in other bones [2,7,9]. Furthermore, only 3.5% to 14.9% of all primary bone sarcomas are primary malignant sarcomas of the spine [2][3][4].…”

“…Additionally, ES of the sacral spine, which is exceedingly aggressive, has a bad prognosis and is less sensitive to treatment. It is more common than ES of the non-sacral spine, which is incredibly uncommon and makes up only 0.9% of all instances [3,5,7,11].…”

“…Additionally, aside from the signs and symptoms of a neurological deficit caused by spinal cord compression, individuals with lesions involving the spine or sacrum may also experience radiculopathy or back pain. In young patients, this delay in onset has been more common than an acute, rapid progression of the lesion [2,7,10]. The Ewing group of cancers has a defective ES gene (EWSR1) allele.…”