Kutty Raja K, Peethambaran Anilkumar, Sunilkumar Balakrishnan S, Balachandran Nair Krishna G, Korde Paresh, Jain Sourabh Kumar
Department of Neurosurgery, Government Medical College, Thiruvananthapuram, Kerala, India.
Department of Neurosurgery, Government Medical College, Thiruvananthapuram, Kerala, India.
World Neurosurg. 2017 Nov;107:1046.e9-1046.e15. doi: 10.1016/j.wneu.2017.07.182. Epub 2017 Aug 7.
Ewing sarcoma (ES) is among the most frequented extremity osseous tumor in childhood. It was first described by James Ewing as diffuse endotheliomas in 1921. The name Ewing sarcoma was coined by Oberling in 1928 as a tribute to the legend who described this disease. ES exists in osseous and extraosseous forms. It shares much of its molecular typing with primitive neuroectodermal tumor (PNET); hence, they are regarded as different ends of the same molecular spectrum. ES, extraosseous ES, PNET, and Askins tumor are the other members of this family termed the Ewing sarcoma family of tumors. Extraosseous ES has been described in various locations of the spine, but its occurrence in the cervical epidural region is uncommon.
A 12-year-old child presented to us with neck pain and progressive weakness of all 4 limbs. She was investigated and found to have a highly vascular lesion in the cervical epidural region extending from C2 to the C4 region. Embolization therapy was unsuccessful. She deteriorated and eventually had to undergo emergency surgery to decompress the spinal cord. The lesion was biopsied and diagnosed as extraosseous ES. After radiotherapy and chemotherapy, the patient regained most of the power in her limbs.
Extraosseous ES is a rare tumor of the cervical cord in this age group. We report this case to highlight the difficulties encountered in the management of this variant.
尤因肉瘤（ES）是儿童期最常见的四肢骨肿瘤之一。1921年詹姆斯·尤因首次将其描述为弥漫性内皮瘤。1928年奥伯林将其命名为尤因肉瘤，以向描述这种疾病的传奇人物致敬。ES有骨内和骨外两种形式。它与原始神经外胚层肿瘤（PNET）有许多分子分型相同；因此，它们被视为同一分子谱系的不同端点。ES、骨外ES、PNET和阿斯金斯瘤是这个家族的其他成员，统称为尤因肉瘤家族肿瘤。骨外ES已在脊柱的各个部位被描述，但其发生在颈椎硬膜外区域并不常见。
一名12岁儿童因颈部疼痛和四肢进行性无力前来就诊。经检查发现其颈椎硬膜外区域有一个从C2延伸至C4区域的高血管性病变。栓塞治疗未成功。她病情恶化，最终不得不接受紧急手术以减压脊髓。对病变进行活检，诊断为骨外ES。经过放疗和化疗后，患者四肢的大部分力量得以恢复。
骨外ES在该年龄组中是一种罕见的颈髓肿瘤。我们报告此病例以突出在处理这种变体时遇到的困难。
