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。
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.
引文
1 Kaye AH,Laws ER(editors). Chordomas and chondrosar- coma of the cranial base . Brain Tumors (edition),1996,777-794
2 Robbie D,Lowery. A Case Report:Maxillotomy for Rem- oval of a Clival Chordoma. Journal of Neuroscience Nursing,1999,31,5
3 Mary L. McMaster,Alisa M.Goldstein, Christina M. Bro- mley. Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes and Control ,2001,12: 1-11
4 Salisbury JR . The pathology of the human notochord. J Pathol,1993,171: 253-25
5 Ho KL. Ecchordosis physaliphora and chordoma: a compar- ative ultrastructural study. Clin Neuropathol ,1985,4: 77- 86
6 Takehiko Yamaguchi, Hiroaki Watanabe-Ishiiwa, Seiichiro Suzuki, et al. Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors. Modern Pathology ,2005,18, 1005-1010
7 Yamaguchi T, Yamato M, Saotome K. First histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion: differential diagnosis of benign and malignant notochordal lesions. Skeletal Radiol ,2002, 31:413-418
8 T Yamaguchi, S Suzuki, H Ishiiwa ,et al. Intraosseous benign notochordal cell tumours: overlooked precursors of classic chordomas? . Histopathology,2004, 44, 597-602
9 Takehiko Yamaguchi, Seiichiro Suzuki, Hiroaki Ishiiwa, et al. Benign Notochordal Cell Tumors:A Comparative Hist- ological Study of Benign Notochordal Cell Tumors, Classic Chordomas, and Notochordal Vestiges of Fetal Interverteb- ral Discs. Am J Surg Pathol ,2004,28:756-761
10 Anson KM, Byrne PO, Robertson ID, et al.. Radical excision of sacrococcygeal tumours. Br J Surg,1994, 81: 460-461
11 Dorfman H, Czerniak B(editors). Chordoma and related lesions. Bone tumors,St Louis: Mosby,1998,974-1008
12 Menezes AH, Traynelis VC. Tumors of the craniovertebral junction, in Youmans JR (ed): Neurological Surgery, ed 4. Philadelphia:WB Saunders, 1996, 4, 3041-3072
13 13、Murphy JM, Wallis F, Toland J, et al. CT and MRI appearances of a thoracic chordoma. Eur Radiol 1998, 8:1677-1679.
14 Kenani. Arnautovic, Ossamaal-mefty. Surgical seeding of chordomas. J Neurosurg, 2001,95,798-803
15 Veronique benk,Norbert J. Liebsch,Johne. Munzenrider,et al. Base of skull and cervical spine chordomas in children treated by high-dose irradiation. Int. J. Radiation Oncology Biol. Phys, 1995,31,3: 577-581.
16 Radiation therapy for chordomas of the base of skull and cervical spine: patterns of failure and outcome after relapse. Int J Radiat Oncol Biol Phys 1995,33:579-584
17 Benedictoo.colli. et al Chordomas of the craniocervical junction: follow-up review and prognostic factors . J Neur- osurg, 2001,95:933-943
18 Georges noel, Jean-louis habrand, Hamid mammar,et al. Combination of photon and proton radiation therapy for chordoms and chondrosarcomas of the skull base: The center de protonthe’rapie D’orsay experience. Int. J. Rad- iation Oncology Biol. Phys, 2001,51, 2:392-398
19 Franz J. Wippold 111,Kelly K. Koeller,James G. Smirnio- topoulos. Clinical and Imaging Features of Cervical Chor- doma. AJR 1999,172,423-1426
20邱吉庆,许海洋,于洪泉,等.颅底脊索瘤的诊断、分型与手术入路选择.中国微侵袭神经外科杂志,2007, 12( 7):296-298
21张辉,张大良,孙彦,等.颅底及颈部脊索瘤11例临床分析.临床耳鼻咽喉科杂志,2006,3,20(6):251-253
22 Ossamaal-Mefty,Luis A.B.Borba. Skull base chordomas: a management challenge. J Neurosurg, 1997,86:182-189
23 Georges No?l, Lo?c Feuvret, Régis Ferrand . Radiotherap-eutic factors in the management of cervical-basal chordo- mas and chondrosarcomas. Neurosurgery, 2004,55: 1252- 1262
24 Luis A.B.Borba,Ossamaal-Mefty,F.A.C.S.,et al. Cranial chordomas in children and adolescents.J Neurosurg , 1996, 84:584-591
25王天铎颅底肿瘤及类肿瘤临床耳鼻咽喉头颈外科杂志,2007,5,21(9):385-389
26吴彦桥,杨伟炎,周定标,等.颅底脊索瘤临床分期及手术治疗.中华耳鼻咽喉科杂志,2003,10,38(5):358-362
27 Kaibara T,Hurlbert RJ. Transoral resection of axial lesions augmented by intraoperative magnetic resonance imaging. Report of three cases. J Neurosurg ,2001,95,2,239-242
28 Mazhar husain, Manu rastogi,Bal krishna ojha,et al. Endos- copic transoral surgery for craniovertebral junction anoma- lies. J Neurosurg Spine, 2006,5:367-373
29 Taro kaibara,R. john hurlbert,Garnette R.Sutherland,et al. Intraoperative magnetic resonance imaging-augmented transoral resection of axial disease. Neurosurg. Focus , 2001,10,2,1-4
30 Hasan Caglar Ugur,Gokmen Kahilogullari,Ayhan Attar,et al. Neuronavigation-Assisted Transoral-Transpharyngeal App- roach for Basilar Invagination-Two Case Reports. Neurol Med Chir(Tokyo) 2006,46,306-308
31 Ossamaal-Mefty,Luis A.B.Borba, Nobuo aoki. The transco- ndylar approach to extradural nonneoplastic lesions of thecraniovertebral junction. J Neurosurg , 1996,84:1-6
32 Todd T. Kingdom, Russ P. Nockls, and Michael J. Kaplan. Transoral-transpharyngeal approach to the craniocervical junction. Otolarygol head neck surg,1995,113:393-400
33 Vougioukas VI,Hubbe U,Schipper J,et al. Navigated transoral approach to the cranial base and the craniocervical junction:Technical note.Neurosurgery ,2003,52:247-251
34 Bernardo fraioli,Vincenzo Esposito, Antonio Santoro,et al. Transmaxillosphenoidal approach to tumors invading the medial compartment of the cavernous sinus. J. Neurosurg, 1995,1,82,63-69
35 Robbie D,Lowery. A Case Report:Maxillotomy for Removal of a Clival Chordoma. Journal of Neuroscience Nursing,1999,10,31,5
36 George J,Kaptain,David D,et al. Transsphenoidal Appr- oaches for the Extracapsular Resection of Midline Suprasellar and Anterior Cranial Base Lesions. Neuro- surgery,2001,7,49,1
37骆纯,卢亦成,孙克华,等.扩大经蝶入路显微手术治疗蝶斜区肿瘤.中国耳鼻咽喉颅底外科杂志,2007,8,13(4):256-258
38张亚卓,王忠诚,赵德安,等.内镜经鼻蝶手术治疗颅底脊索瘤.中华神经外科杂志,2007,3,23(3):163-166
39韩林,舒凯,徐钰,等.经蝶入路颅底脊索瘤的显微手术治疗(附15例分析).中国微侵袭神经外科杂志,2008,13(9) 401-403
40 Michael P Colreavy;Tim Baker;Matthew Campbell;et al. The safety and effectiveness of the Le Fort I approach to removing central skull base lesions. Ear,Nose&Throat Journal,2001,5,80,5
41吕胜青,杨辉,陈立华.颅底脊索瘤的临床诊治进展中国耳鼻咽喉颅底外科杂志,2005,10,11(5):366-368
42郭京,张黎,张思迅,等.经上颌骨翻转入路手术切除斜坡脊索瘤.中华医学杂志2005,6,85(20):1379-1381
43韩德民,周兵.鼻内窥镜手术学,第一版,101-102、177-179
44覃道芬,高下,周平,等.鼻内镜经鼻入路手术治疗颅底脊索瘤.现代肿瘤医学,2008,5,16(5):730-732
45李长青,张秋航,严波,等.经鼻内镜切除颅底软骨肉瘤1例.中国耳鼻咽喉颅底外科杂志2008,2,14(1):75-76
46张秋航,孔锋,严波,等.经鼻内镜斜坡脊索瘤和脊索肉瘤的外科治疗.中国微侵袭神经外科杂志, 2006, 11( 10)438-440
47李光宇,朱冬冬,姜晓丹,等.上颌窦脊索瘤1例.临床耳鼻咽喉头颈外科杂志,2007,3, 21,(5)237
48骆献阳,赵德安,周毅,等.颧颞入路联合耳前颞下窝入路切除巨大咽旁颅中窝脊索瘤1例.临床耳鼻咽喉科杂志Oct 2003 ,10,17 ,(10)624
49 Uggowitzer MM, Kugler C, Groell R,et al . Drop metastases in a patient with a chondroid chordoma of the clivus. Neuroradiology,1999,41:504-507
50 Nancy J. Fischbein, Michael J. Kaplan, Roy A. Holliday, et al. Dillon Recurrence of Clival Chordoma along the Surg-ical Pathway . Am J Neuroradiol,2000,3,21:578-583
51 Marcio A. Facxjndes, Eugen B. Hug, Norbert J. Liebsch,et al. Radiation therapy for chordoms of the base of skull and cervical spine:patterns of failure and outcome after relapse. lnt. J. Radiation Oncology Biol. Phys, I995,33,3,579-584
52 M.M.Uggowitzer , C. Kugler , R.Groell , et al. Drop metastases in a patient with a chondroid chordoma of the clivus. Neuroradiology ,1999,41: 504-507
53 Toshifumi Ozaki, Axel Hillmann, Winfird Winkelmann. Surgical Treatment of Sacrococcygeal Chordoma. Journal of Surgical Oncology, 1997,64:274-279
54刘庆坤带蒂胸大肌肌皮瓣修复口腔颌面部肿瘤术后缺损22例中国现代医生,2008,3,46(7) :66
55许君武,徐杰,郑昆华,等.前臂皮瓣联合腓骨肌瓣修复上颌骨大型缺损.现代肿瘤医学2004,12,12(6):539-540
56潘孟雄,唐际存,马祥伟,等.前臂桡侧游离皮瓣修复口腔颌面部软组织缺损15例临床分析华夏医学,2007,8,20(4)798-799
57南杰,米玉录.胸大肌肌皮瓣在头颈部手术缺损修复中的应用.肿瘤研究与临床,2006,7,18(7):472-473.
58 58、Sunil Krishnan, Robert L. Foote, Paul D. Brown. Radio surgery for cranial base chordomas and chondorosarcomas- Neurosurgery ,2005,56:777-784
59 Jurgen Debus, Daniela Schulz-ertner, Lothar Schad,et al. Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int. J. RadiationOncology Biol. Phys,2000,47,3,. 591-596
60 Georges noel,Jean-louis Habrand,Hamd Mammar,et al. Combination of photon and proton radiation therapy for chordomas and chondrosarcomas of the skull base:the center de protonthe’rapied’orsay experience. Int. J. Radiation Oncology Biol. Phys,2001,51,2, 392-398
61 Krayenbuhl, H., Yasargil, M. G. Cranial chordomas. Progr.Neurol. Surg.,1975,6:380-434
62 Natarajan Muthukumar, Douglas Kondziolka,L. Dade Lunsford,et al. Stereotactic radiosurgery for chordoma and chondrosarcoma:future experiences. Int. J. Radiation Onco- logy Biol. Phys,1998,41,2, 387-392
63 Eugen B. Hug, Markus M. Fitzek, Norbert J. Liebsch,et al. Locally challenging osteo-and chondrogenic tumors of the axial skeleton:results of conbined proton and photon radia- tion therapy using three-dimensional treatment planning. Int. J. Radiation Oncology Biol. Phys, 1995,31,3, 467-476
64 Ikeda H, Honjo J, Sakurai H, et al. Dedifferentiated chordoma arising in irradiated sacral chordoma. Radiat Med,1997,15:109-111
65 Atsuro teranara,Andrzej Niemierko,Michael Goitein,et al. Analysis of the relationship between tumor dose inhomo- geneity,and local control in patients with skull base chord- oma. Int. J. Radiation Oncology Biol. Phys,1999,45,2, 351 -358
66 Forsyth PA, Cascino TL, Shaw EG, et al: Intracranialchordomas:a clinicopathological and prognostic study of 51 cases. J Neurosurg,1993,78:741-747
67 Borba LA, Al-Mefty O, Mrak RE, et al: Cranial chordomas in children and adolescents. J Neurosurg,1996,84:584-591
68 Heffelfinger MJ, Dahlin DC, MacCarty CS, et al. Chordomas and cartilaginous tumors at the skull base. Cancer,1973,32:410-420