颅内软骨肉瘤的诊断与治疗（附17例报告）

Diagnosis and treatment of intracranial chondrosarcomas： A report of 17 cases

目的总结颅内软骨肉瘤的临床特点和诊疗经验，为其临床治疗提供参考依据。方法回顾性分析2007年6月至2016年12月中国医科大学附属第一医院神经外科收治的17例颅内软骨肉瘤患者的临床资料。所有患者均行手术治疗。9例术后行放射治疗。结果17例患者中，9例达到肿瘤全切除，8例为次全切除。术后病理学结果：普通型软骨肉瘤13例，黏液型软骨肉瘤3例，间叶型软骨肉瘤1例。术前误诊比例为14／17。术后新发脑神经功能障碍3例，脑脊液漏2例，颅内感染2例，出现精神症状1例。16例患者获得随访。术后随访时间为11～113个月，平均（55．9±23．1）个月。患者2年、5年的无进展生存率分别为81．3％和75．0％。随访期间，行次全切除的患者中，2例死亡，5例病情进展。结论颅内软骨肉瘤为颅内罕见的恶性肿瘤，临床表现和影像学表现缺乏特异性，术前易误诊。其治疗方法首选手术切除，肿瘤全切除的患者可获得较好的预后。

Objective To summarize the clinical characteristics, diagnosis and treatment of intracranial chondrosarcomas and to provide the reference for the clinical treatment of intracranial chondrosarcomas. Methods The clinical data of 17 patients of intracranial choudrosarcoma undergoing surgical operation at Department of Neurosurgery, the First Hospital of China Medical University from June 2007 to December 2016 were retrospectively analyzed. Nine cases underwent postoperative radiotherapy. Results Gross total resection of tumor was achieved in 9 cases and subtotal resection in 8. Thirteen cases were pathologically diagnosed as classic chondrosarcoma, 3 as myxoid chondrosarcoma and 1 as mesenchymal chondrosarcoma. Preoperative misdiagnosis rate was 14/17. Postoperative newly developed cranial nerve palsy occurred in 3 cases and cerebrospinal fluid leakage in 2. Intracranial infection occurred in 2 patients and psychiatric disorder in 1. Sixteen patients were followed up for 11 - 113 months with an average of 55.9 ＋23. 1 months. The 2 - and 5 -year progression-free survival rates were 81.3% and 75. 0% respectively. Among the patients with subtotal resection, 5 cases progressed and 2 died. Conclusions Intracranial chondrosarcoma is a rare intracranial malignant tumor, Due to lack of specific clinical and imaging features, the preoperative misdiagnosis rate may be high. Sm＇gery should be given priority and patients with total resection could obtain relatively good prognosis.