Department of Neurosurgery, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Chuo, Japan.
Department of Neurosurgery, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Chuo, Japan.
World Neurosurg. 2021 Jan;145:376-380. doi: 10.1016/j.wneu.2020.10.007. Epub 2020 Oct 10.
Among chondrosarcomas arising from bones and soft tissues, mesenchymal chondrosarcoma (MCS), especially primary spinal intradural extramedullary MCS, is extremely rare, and only 18 cases have been reported to date. We report an adult case of this rare condition mimicking meningioma.
A 42-year-old woman presented with paraplegia and sensory disturbance associated with right-sided Brown-Séquard syndrome following back pain. Magnetic resonance imaging showed an intradural mass in the right dorsal spinal canal with homogeneous enhancement and dural tail sign at the T8 level. Computed tomography demonstrated a calcified portion in the mass. Following T7-8 laminectomies, an intradural extramedullary tumor was completely removed after detaching the tumor from the dura mater. The histopathologic diagnosis was MCS, and positron emission tomography showed no metastatic lesions at other sites. The patient did not receive adjuvant therapy, and magnetic resonance imaging revealed no evidence of recurrence during 2-year follow-up.
Primary spinal intradural extramedullary MCS has been reported to have a better prognosis than MCS occurring in other regions. In a case with early complete surgical resection, adjuvant therapy should be considered at the time of recurrence.
在源于骨骼和软组织的软骨肉瘤中,间叶性软骨肉瘤(MCS),特别是原发性脊柱髓外硬脊膜内 MCS,极为罕见,迄今为止仅报告了 18 例。我们报告了一例罕见的脑膜瘤样病例。
一名 42 岁女性因背痛后出现右侧 Brown-Séquard 综合征伴截瘫和感觉障碍。磁共振成像显示 T8 水平硬脊膜内椎管内有一个均质强化的肿块,伴有硬脊膜尾征。计算机断层扫描显示肿块中有钙化部分。在 T7-8 椎板切除术之后,在从硬脑膜上分离肿瘤后,完全切除了硬脊膜外髓内肿瘤。组织病理学诊断为 MCS,正电子发射断层扫描显示在其他部位没有转移病灶。该患者未接受辅助治疗,在 2 年的随访中磁共振成像未显示复发迹象。
与其他部位发生的 MCS 相比,原发性脊柱髓外硬脊膜内 MCS 的预后较好。对于早期完全手术切除的病例,在复发时应考虑辅助治疗。