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胸腰椎原发性硬脊膜内髓外骨外尤文肉瘤/外周原始神经外胚层肿瘤(PIEES/PNET):病例报告及文献综述

Primary intradural extramedullary extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumor (PIEES/PNET) of the thoracolumbar spine: A case report and literature review.

作者信息

Pu Feifei, Liu Jianxiang, Zhang Zhicai, Guo Tao, Shao Zengwu

机构信息

Department of Orthopedics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.

Department of Pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.

出版信息

Open Med (Wars). 2021 Oct 21;16(1):1591-1596. doi: 10.1515/med-2021-0377. eCollection 2021.

Abstract

We present a rare case of a primary intradural extramedullary Ewing's sarcoma/peripheral primitive neuroectodermal tumor (PIEES/PNET) in the thoracolumbar spine and review the current literature. We describe the imaging manifestations, pathological features, surgical methods, and patient survival to shed light on the clinical management of this rare tumor. A 32-year-old man experienced progressive low back pain for more than 1 month. An intradural extramedullary tumor from T12 to L2 was detected on magnetic resonance imaging. He underwent a thoracolumbar laminotomy for decompression, complete excision of the intradural extramedullary tumor, and internal fixation with pedicle screws. A histopathological examination confirmed that the tumor was a PIEES/PNET via an immunohistochemical study of the surgically resected tissues. Postoperatively, the patient received chemotherapy and radiotherapy. No recurrence, metastasis, or failure of internal fixation were noted at a 17-month post-surgery radiographic examination. PIEES/PNET of the thoracolumbar spine is extremely rare. Treatment is difficult because the current literature is sparse and cases are rare. Complete resection combined with chemotherapy and radiotherapy effectively reduces recurrence and metastasis.

摘要

我们报告了一例罕见的胸腰椎原发性硬脊膜内髓外尤因肉瘤/外周原始神经外胚层肿瘤(PIEES/PNET)病例,并对当前文献进行了综述。我们描述了其影像学表现、病理特征、手术方法及患者生存情况,以阐明这种罕见肿瘤的临床管理。一名32岁男性经历了超过1个月的进行性下背痛。磁共振成像检测到T12至L2水平存在硬脊膜内髓外肿瘤。他接受了胸腰椎椎板切开减压术、硬脊膜内髓外肿瘤全切术及椎弓根螺钉内固定术。通过对手术切除组织进行免疫组织化学研究,组织病理学检查证实该肿瘤为PIEES/PNET。术后,患者接受了化疗和放疗。术后17个月的影像学检查未发现复发、转移或内固定失败情况。胸腰椎PIEES/PNET极为罕见。由于当前文献稀少且病例罕见,治疗颇具难度。完整切除联合化疗和放疗可有效降低复发和转移风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f149/8532567/6954648725df/j_med-2021-0377-fig001.jpg

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