【作者】 赵雷；

【导师】 张勋；

【作者基本信息】 河北医科大学 ， 耳鼻咽喉科学， 2009， 硕士

【摘要】 脊索瘤是一种起源于脊索残余或异位脊索的骨肿瘤,亦有人认为由良性脊索细胞瘤恶性转化而来,其具有局部浸润、易复发、远处转移倾向,被认为是一种恶性骨肿瘤,其好发于中轴骨的两端(颅底和骶尾部),尤其是颅底部脊索瘤,因其所在位置解剖结构复杂,毗邻重要结构,其手术治疗难度大,危险性高,一直是学者们关注的焦点。以往颅底脊索瘤的治疗,主要是神经外科采用颅内入路或是颅面联合入路,不仅危险性较高,而且损伤较大,往往造成较大的颅面缺损。随着人们对颅底解剖结构的逐渐熟悉及医疗技术的进一步发展,采用颅外入路越来越备受关注,尤其是随着耳鼻咽喉-头颈外科学的发展,鼻内窥镜手术技术日益成熟,其应用在减小手术创伤的同时,有效地减少了手术所致的结构缺损,更为有效地扩大了手术视野,使得术中瘤体的切除更加彻底,对周围重要解剖结构的保护更加完善。颅底脊索瘤因其瘤体多样化及其颅底复杂的解剖结构,依据肿瘤扩散方式或临床表现及其部位等不同的标准,被人们分成不同的类型,其手术治疗难度也较大,依据不同的肿瘤类型并结合具体情况,往往采用不同的术式,甚至是两种或两种以上术式的联合,以尽可能彻底完整地切除肿瘤减少复发为目的,目前采用的术式主要包括:经口入路、经上颌骨-蝶骨入路、Le Fort I入路和扩大Le Fort I入路、经髁入路、经颞部入路、经额下或额底入路、经上颌骨翻转入路以及经鼻内窥镜引导手术等。随着鼻内窥镜技术的日益成熟和发展,其在颅底脊索瘤的手术治疗中的应用将更为广泛,其优越性必将日益凸现。手术切除是治疗颅底脊索瘤的主要方法,术后辅以放射治疗,则可更为有效地降低肿瘤复发率。传统观念认为脊索瘤对常规放疗剂量不敏感,因此应适当增加放疗剂量。随着手术技术的日益成熟和放疗技术的逐步发展,颅底脊索瘤的局部控制将逐渐改善,复发率必将逐渐减小,患者的生活质量亦会得到很大提高。更多还原

【Abstract】 Chordomas are rare malignant bone tumors,believed to arise from vestigial or ectopic notochordal tissue,However, recent studies suggest the possibility that chordomas arise from benign notochordal cell tumors.They were considered as mali- gnant tumors, with local invasiveness, a tendency for recurrence, and, in rare circumstances, the potential to metastasize. The most common locations include the sacrococcygeal region and skull base. The management of the skull base chordomas is particularly challenging as they lie adjacent to vital anatomic structures.Traditionally the management of skull base chordomas has been a formidable challenge for neurosurgeons,it’s risky to acc- ess the locations via intracranial or cranial-facial,and cranial- facial defects are common. Trans-extracranial to access to the skull base has been paied much more attention to with the study of the skull base structure and the development of the medical equipments ,especially the development of the Otorhinolaryngo- lgy-Head and Neck Surgery.The nasal endoscopic surgery make it possible to diminish the surgical trauma and structure defect, even enlarge the visual field of operation,besides,the resection of tumor would be completely,and avoid the damage of the important structures.Because of the diversification of tumors and the complex- ity of the cranial base structures,the chordomas could be divided into different categories,according to the diffusion types of tumor、clinical manifestation or the locations,many surgical access were adopted to management chordomas according to the different categories and the special conditions.sometimes it’s necessary for surgeons to adopt two or more accesses to resect the tumor completely.recently,the main accesses contains: trans- oral approach、transsuperior maxillary bone-sphenoid bone app- roach、Le Fort I and enlarged Le Fort I approach、transcondylus approach、transtemporal approach、transsuperior maxillary bone turnover approach and with the guiding of the nasal endoscopic operation,and so on.with the development and the maturity of Nasal Endoscopic Surgery(NES),the use of the NES will be ext- ensively and superiority of it will be showed in the managem- ent of chordoma.Surgical resection followed by radiation therapy is the rational choice for the patients suffered from chordomas. Chord- omas were considered relatively resistant to conventional radio- therapy,so it’s neccesary to increase the dose of radiotherapy. with the development of radiation therapy and the maturity of surgical technical day by day,the local control muse be impro- ved,the relapse rate must be diminished,besides,the quality of life must be improved.更多还原