摘要：

目的 总结颅底骨源性肿瘤的临床诊断、手术切除及综合治疗相关经验。方法与结果 2006年9月至2018年3月共对76例颅底骨源性肿瘤患者施行外科手术治疗，手术入路包括经鼻、经口腭、上颌骨掀翻、耳后颅颈联合、Derome、额颞和耳前-颞下窝入路；神经内镜手术者24例、经颅显微镜手术者52例。肿瘤全切除者38例、次全切除22例、部分切除14例、经活检确诊2例。组织病理类型分别为脊索瘤（28例）、骨肉瘤（19例）、软骨肉瘤（11例）、骨纤维异常增殖症（8例）、骨巨细胞瘤（5例）、骨母细胞瘤（3例）、骨软骨瘤（1例）和软骨黏液样纤维瘤（1例）。术后并发症包括神经功能障碍（19例次）、偏瘫（3例次）、脑脊液鼻漏（12例次）、颅内感染（8例次）、肺部感染（4例次）和切口愈合不良（4例次）。术后随访20～158个月，11例失访、死亡21例、肿瘤无进展生存34例、肿瘤进展带瘤生存10例。结论 虽然颅底骨源性肿瘤发病率较低，但生物学特性以恶性者居多。因此，术前需根据患者症状与体征以及辅助检查结果进行综合评估，以提高定性诊断之准确率并明确手术目的，从而达到合理选择手术入路、最大程度切除病灶之目的，同时术后辅以规范化综合治疗，有望提高肿瘤治愈率。

关键词: 颅底肿瘤, 肿瘤,骨组织, 精准医学, 神经外科手术

Abstract:

Objective To review experience of diagnosis, surgical procedures and comprehensive therapeutic strategies for skull base bone tumors. Methods and Results Clinical data of 76 patients with skull base bone tumors were retrospectively analyzed from September 2006 to March 2018. After preoperative assessment, different kinds of surgical approaches including endonasal, intraoral, maxillary swing, craniocervical transjugular, Derome, frontotemporal and infratemporal approaches were chosen according to clinical and radiographic findings. In comparison with 24 patients receiving the endoscopic surgery, other 52 patients were operated on under microscopic visualization. Total resection was achieved in 38 cases, and 22 patients underwent subtotal resection. In addition, 14 cases received partial resection while biopsies were performed in 2 cases to confirm histological findings. Tumor pathology included chordoma (28 cases), osteosarcoma (19 cases), chondrosarcoma (11 cases), fibrous dysplasia (8 cases), giant cell tumor of bone (5 cases), osteoblastoma (3 cases), osteochondroma (1 case), and chondromyxiod fibroma (1 case). Complications included cranial nerve deficits (19 cases), hemiparesis (3 cases), cerebrospinal fluid leakage (12 cases), intracranial infection (8 cases), pneumonitis (4 cases), and wound dehiscence (4 cases). During a 20 to 158 months follow-up, 11 cases were lost and 21 patients died in the study period. The disease-free survival was achieved in 34 cases while 10 patients experienced tumor progression. Conclusions Malignant bone tumors invading the skull base is much more common comparing with the benign lesions. Multidisciplinary consultation may improve diagnostic reliability and clarify the therapeutic purpose. Radical resection using the individualized approaches accompanied by comprehensive therapy should be considered to improve the outcomes of patients.

Key words: Skull base neoplasms, Neoplasms,bone tissue, Precision medicine, Neurosurgical procedures