Department of Neurosurgery, 48435Shiraz University of Medical Sciences, Shiraz, Iran.
Shiraz Neurosciences Research Center, Department of Neurosurgery, 48435Shiraz University of Medical Sciences, Shiraz, Iran.
J Int Med Res. 2022 Aug;50(8):3000605221108095. doi: 10.1177/03000605221108095.
Ewing sarcoma (ES) is a highly aggressive bone and soft tissue tumor that occurs mainly in young children and adolescents and is associated with primary and metastatic disease. Intramedullary ES (either primary or secondary) is rare, and the ideal management remains inconclusive. We herein report intramedullary and extramedullary metastatic ES in a single patient. A 46-year-old woman was referred to our outpatient clinic from the oncology clinic with progressive paraparesis and paresthesia for 1 week prior to presentation. She had developed left clavicular ES 2 years earlier for which surgery and chemoradiotherapy had been performed. At the present evaluation, she was diagnosed with intramedullary thoracic and lumbar extradural masses. Thoracic surgery was performed, and a biopsy of the lesion was obtained. The diagnosis of ES was confirmed histopathologically, and she underwent adjuvant chemotherapy. Her neurological status did not improve after surgery, and she underwent rehabilitation and physical therapy. The lumbar lesion resolved with chemotherapy. Metastasis of ES to the spinal cord, especially intramedullary lesions, is extremely rare, and there is no standard management guideline. However, surgical decompression and adjuvant chemotherapy are the main treatments in these cases.
尤因肉瘤(ES)是一种高度侵袭性的骨和软组织肿瘤,主要发生在儿童和青少年,与原发性和转移性疾病有关。髓内 ES(原发性或继发性)很少见,理想的治疗方法仍不确定。我们在此报告一例单一患者的髓内和髓外转移性 ES。一名 46 岁女性因进行性截瘫和感觉异常 1 周而从肿瘤科门诊转至我们的门诊就诊,在就诊前 1 周出现上述症状。她在 2 年前被诊断出左侧锁骨 ES,已接受手术和放化疗。在本次评估中,她被诊断为胸腰椎髓外硬膜内肿块。进行了胸部手术,并对病变进行了活检。组织病理学证实了 ES 的诊断,她接受了辅助化疗。手术后她的神经状况没有改善,她接受了康复和物理治疗。腰椎病变通过化疗得到缓解。ES 转移至脊髓,特别是髓内病变,极其罕见,且没有标准的治疗指南。然而,在这些情况下,手术减压和辅助化疗是主要的治疗方法。