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成人颈椎原发性哑铃形尤因肉瘤:4例报告及文献复习

Primary dumbbell-shaped Ewing's sarcoma of the cervical vertebra in adults: Four case reports and literature review.

作者信息

Zhu Qing, Zhang Jisheng, Xiao Jianru

机构信息

Department of Orthopedics, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai 86200, P.R. China.

出版信息

Oncol Lett. 2012 Mar;3(3):721-725. doi: 10.3892/ol.2012.550. Epub 2012 Jan 3.

Abstract

Ewing's sarcoma is the second most common malignant bone tumor in children and adolescents. The 4 cases described in this study were diagnosed with dumbbell-shaped intraspinal and extraspinal Ewing's sarcomas. The incidence of dumbbell-shaped tumors of this type in the spine is 17.5%. These tumors are often misdiagnosed as neurogenic tumors (schwannoma, neurofibromatosis) or giant cell tumors based on imaging. Radiculopathy is more common than spinal cord compression in Ewing's sarcoma. Preoperative biopsy is strongly recommended. As soon as Ewing's sarcoma is diagnosed by pathology, the treatment should begin with 2-3 cycles of neoadjuvant chemotherapy. Anterior-posterior and posterolateral approaches are both recommended for exposing this tumor. Following surgery, chemotherapy is critical to lessen the rate of recurrence and metastasis and to prolong the survival time. However, radiotherapy should be used with caution, as the spinal cord is sensitive to radiation; local irradiation is suggested. The tumor is difficult to remove en bloc in the cervical spine. It has a high rate of recurrence and metastasis. Therefore, the prognosis of Ewing's sarcoma in the cervical region is poorer compared to that in the thoracic and lumbosacral regions.

摘要

尤因肉瘤是儿童和青少年中第二常见的恶性骨肿瘤。本研究中描述的4例患者被诊断为哑铃形椎管内和椎管外尤因肉瘤。脊柱中这种类型的哑铃形肿瘤的发生率为17.5%。基于影像学,这些肿瘤常被误诊为神经源性肿瘤(神经鞘瘤、神经纤维瘤病)或巨细胞瘤。在尤因肉瘤中,神经根病比脊髓压迫更常见。强烈建议进行术前活检。一旦通过病理诊断为尤因肉瘤,治疗应从2 - 3个周期的新辅助化疗开始。推荐采用前后路和后外侧入路来显露该肿瘤。手术后,化疗对于降低复发和转移率以及延长生存时间至关重要。然而,放疗应谨慎使用,因为脊髓对辐射敏感;建议进行局部照射。该肿瘤在颈椎难以整块切除。其复发和转移率高。因此,与胸段和腰骶段相比,颈椎尤因肉瘤的预后较差。

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