Department of Orthopedics, National Taiwan University College of Medicine and National Taiwan University Hospital, No.7, Chungshan South Road, 10002, Taipei, Taiwan.
Department of Orthopedics, National Taiwan University Hospital Hsin Chu Branch, Hsin Chu, Taiwan.
BMC Musculoskelet Disord. 2019 Sep 4;20(1):408. doi: 10.1186/s12891-019-2799-2.
Mesenchymal chondrosarcoma (MCS) is a rare malignant variant of chondrosarcoma with a high tendency of recurrence and metastasis. Intradural extramedullary spinal MCS is exceedingly rare and usually found in pediatric patients. Herein, we present an elderly patient with primary intradural extramedullary spinal MCS. Relevant literatures are reviewed to disclose characteristics of intradural extramedullary spinal MCS.
A 64-year-old female presented with urinary difficulty and tightness of upper back preceding progressive weakness of right lower extremity. Magnetic resonance imaging revealed an intradural extramedullary tumor at the level of 3rd thoracic vertebra. This patient underwent total tumor resection and then received adjuvant radiotherapy. Histopathological examination showed that the tumor composed of spindle and round cells with high nucleocytoplasmic ratio accompanied by scattered eosinophilic chondroid matrix. Along with immunohistochemical findings and the existence of HEY1-NCOA2 fusion transcript, the diagnosis of MCS was confirmed. Neurologic deficit recovered nearly completely after surgery. No evidence of local recurrence or distant metastasis was found 5 years after treatments. Including the current case, a total of 18 cases have been reported in the literature with only one case with local recurrence and one case of mortality. The current case was the eldest patient diagnosed with primary intraspinal MCS in the literature.
MCS rarely appears in the intradural space of the spine. In contrast to classic MCS, treatment outcome of primary intradural extramedullary spinal MCS is usually excellent as total tumor resection is commonly achievable. Adjuvant radiotherapy may reduce local recurrence and chemotherapy may be associated with fewer recurrences especially for unresectable tumors.
间叶性软骨肉瘤（MCS）是一种罕见的软骨肉瘤恶性变体，具有较高的复发和转移倾向。硬脊膜外髓内脊髓 MCS 极为罕见，通常发生在儿童患者中。本文报告了一例老年患者的原发性硬脊膜外髓内脊髓 MCS。回顾相关文献，揭示硬脊膜外髓内脊髓 MCS 的特征。
一名 64 岁女性因上背部紧绷和排尿困难，逐渐出现右下肢无力前来就诊。磁共振成像显示第 3 胸椎水平存在硬脊膜外髓内肿瘤。该患者接受了全肿瘤切除术，然后接受了辅助放疗。组织病理学检查显示肿瘤由纺锤形和圆形细胞组成，核浆比较高，伴有散在的嗜酸性软骨样基质。结合免疫组织化学结果和 HEY1-NCOA2 融合转录本的存在，诊断为 MCS。手术后神经功能缺损几乎完全恢复。治疗 5 年后，未发现局部复发或远处转移的证据。包括本病例在内，文献中总共报告了 18 例，仅有 1 例局部复发和 1 例死亡。本病例是文献中诊断为原发性脊柱内 MCS 的最年长患者。
MCS 很少出现在脊柱的硬脊膜内。与经典 MCS 不同，原发性硬脊膜外髓内脊髓 MCS 的治疗结果通常较好，因为通常可以实现全肿瘤切除术。辅助放疗可能会降低局部复发的风险，化疗可能与较少的复发相关，尤其是对于不可切除的肿瘤。
