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原发性脊髓内间叶性软骨肉瘤:一例报告及文献复习

Primary intraspinal mesenchymal chondrosarcoma: a case report and literature review.

作者信息

Huang Kuo-Feng, Tzaan Wen-Ching, Lin Chin-Yew

机构信息

Department of Neurosurgery, Chang Gung Memorial Hospital, Taipei.

出版信息

Chang Gung Med J. 2003 May;26(5):370-6.

Abstract

Mesenchymal chondrosarcomas are rare malignant tumors of the bone and soft tissue. Extraskeletal mesenchymal chondrosarcomas, especially those that arise in the central nervous system, are even rarer. Most of those described were intracranially located, with only a very few cases having been reported in an intraspinal region. Therapeutic experience with primary spinal mesenchymal chondrosarcomas is also extremely limited. We report on a case of a 21-year-old man with back pain and bilateral progressive weakness and numbness of the lower extremities. A T1-weighted magnetic resonance image revealed a hypointense tumor located at the T8 level. The tumor was completely excised through a posterior approach. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. Spinal irradiation and chemotherapy were also administered for prevention of local recurrence and metastasis. The patient has been symptom-free for 1 year after surgery. Herein, we review the 22 cases of primary intraspinal mesenchymal chondrosarcomas in the literature and discuss their clinical presentations, pathology, imaging studies, treatments, and outcomes.

摘要

间叶性软骨肉瘤是一种罕见的骨与软组织恶性肿瘤。骨外间叶性软骨肉瘤,尤其是发生于中枢神经系统的,更为罕见。已报道的大多数病例位于颅内,仅有极少数病例发生于脊髓区域。原发性脊髓间叶性软骨肉瘤的治疗经验也极为有限。我们报告一例21岁男性患者,有背痛及双下肢进行性无力和麻木症状。T1加权磁共振成像显示一个低信号肿瘤位于T8水平。通过后路手术将肿瘤完整切除。显微镜检查及免疫组化研究确诊为间叶性软骨肉瘤。还进行了脊柱放疗及化疗以预防局部复发和转移。患者术后1年无症状。在此,我们回顾文献中22例原发性脊髓间叶性软骨肉瘤病例,并讨论其临床表现、病理、影像学检查、治疗及预后。

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