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Tumor-biology and current treatment of skull-base chordomas

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Advances and Technical Standards in Neurosurgery

Part of the book series: Advances and Technical Standards in Neurosurgery ((NEUROSURGERY,volume 33))

Abstract

Chordomas are rare, slow growing tumors of the axial skeleton, which derive from the remnants of the fetal notochord. They can be encountered anywhere along the axial skeleton, most commonly in the sacral area, skull base and less commonly in the spine. Chordomas have a benign histopathology but exhibit malignant clinical behavior with invasive, destructive and metastatic potential. Genetic and molecular pathology studies on oncogenesis of chordomas are very limited and there is little known on mechanisms governing the disease. Chordomas most commonly present with headaches and diplopia and can be readily diagnosed by current neuroradiological methods. There are 3 pathological subtypes of chordomas: classic, chondroid and dedifferentiated chordomas. Differential diagnosis from chondrosarcomas by radiology or pathology may at times be difficult.

Skull base chordomas are very challenging to treat. Clinically there are at least two subsets of chordoma patients with distinct behaviors: some with a benign course and another group with an aggressive and rapidly progressive disease. There is no standard treatment for chordomas. Surgical resection and high dose radiation treatment are the mainstays of current treatment. Nevertheless, a significant percentage of skull base chordomas recur despite treatment. The outcome is dictated primarily by the intrinsic biology of the tumor and treatment seems only to have a secondary impact. To date we only have a limited understanding this biology; however better understanding is likely to improve treatment outcome.

Hereby we present a review of the current knowledge and experience on the tumor biology, diagnosis and treatment of chordomas.

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References

  1. (2000) Proton therapy for base of skull chordoma: a report for the Royal College of Radiologists. The Proton Therapy Working Party. Clin Oncol (R Coll Radiol) 12: 75–79

    Google Scholar 

  2. Abenoza P, Sibley RK (1986) Chordoma: an immunohistologic study. Hum Pathol 17: 744–747

    PubMed  CAS  Google Scholar 

  3. Akimoto J, Takeda H, Hashimoto T, Haraoka J, Ito H (1996) A surgical case of ecchordosis physaliphora. No Shinkei Geka 24: 1021–1025

    PubMed  CAS  Google Scholar 

  4. Al-Mefty O, Fox JL, Smith RR (1988) Petrosal approach for petroclival meningiomas. Neurosurgery 22: 510–517

    PubMed  CAS  Google Scholar 

  5. Al-Mefty O, Borba LA (1997) Skull base chordomas: a management challenge. J Neurosurg 86: 182–189

    PubMed  CAS  Google Scholar 

  6. Alezais MM, Peyron A (1914) Contrubution a letude des chordomes; chordomes de la region occipitale, Paris

    Google Scholar 

  7. Alezais MM, Peyron A (1914) Sur une tendence evolutive remarquable de certainc chordomes-passage de l’epitheliome au sarcome polymorphe, Paris

    Google Scholar 

  8. Alonso WA, Black P, Connor GH, Uematsu S (1971) Transoral transpalatal approach for resection of clival chordoma. Laryngoscope 81: 1626–1631

    PubMed  CAS  Google Scholar 

  9. Arbit E, Patterson RH Jr (1981) Combined transoral and median labiomandibular glossotomy approach to the upper cervical spine. Neurosurgery 8: 672–674

    PubMed  CAS  Google Scholar 

  10. Archer DJ, Young S, Uttley D (1987) Basilar aneurysms: a new transclival approach via maxillotomy. J Neurosurg 67: 54–58

    PubMed  CAS  Google Scholar 

  11. Arnautovic KI, Al-Mefty O (2001) Surgical seeding of chordomas. Neurosurg Focus 10: E7

    PubMed  CAS  Google Scholar 

  12. Arnold H, Herrmann HD (1986) Skull base chordoma with cavernous sinus involvement. Partial or radical tumour-removal? Acta Neurochir (Wien) 83: 31–37

    CAS  Google Scholar 

  13. Aszodi A, Chan D, Hunziker E, Bateman JF, Fassler R (1998) Collagen II is essential for the removal of the notochord and the formation of intervertebral discs. J Cell Biol 143: 1399–1412

    PubMed  CAS  Google Scholar 

  14. Auger M, Raney B, Callender D, Eifel P, Ordonez NG (1994) Metastatic intracranial chordoma in a child with massive pulmonary tumor emboli. Pediatr Pathol 14: 763–770

    PubMed  CAS  Google Scholar 

  15. Austin-Seymour M, Munzenrider J, Goitein M, Verhey L, Urie M, Gentry R, Birnbaum S, Ruotolo D, McManus P, Skates S, et al (1989) Fractionated proton radiation therapy of chordoma and low-grade chondrosarcoma of the base of the skull. J Neurosurg 70: 13–17

    PubMed  CAS  Google Scholar 

  16. Aydin P, Ozek M, Kansu T (1994) Intermittent diplopia in chordoma. Ann Ophthalmol 26: 20–22

    PubMed  CAS  Google Scholar 

  17. Azzarelli A, Quagliuolo V, Cerasoli S, Zucali R, Bignami P, Mazzaferro V, Dossena G, Gennari L (1988) Chordoma: natural history and treatment results in 33 cases. J Surg Oncol 37: 185–191

    PubMed  CAS  Google Scholar 

  18. Babu RP, Sekhar LN, Wright DC (1994) Extreme lateral transcondylar approach: technical improvements and lessons learned. J Neurosurg 81: 49–59

    PubMed  CAS  Google Scholar 

  19. Bailey SM, Murnane JP (2006) Telomeres, chromosome instability and cancer. Nucleic Acids Res 34: 2408–2417

    PubMed  CAS  Google Scholar 

  20. Bayrakli F, Guney I, Kilic T, Ozek M, Pamir MN (2007) New candidate chromosomal regions for chordoma development. Surg Neurol 68(4): 425–430

    PubMed  Google Scholar 

  21. Bayrakli FGI, Kilic T, Ozek MM, Pamir MN (accepted 1 November 2006) New candidate chromosomal regions for chordoma development. Surg Neurol (article in print)

    Google Scholar 

  22. Beck DW, Menezes AH (1987) Lesions in Meckel’s cave: variable presentation and pathology. J Neurosurg 67: 684–689

    PubMed  CAS  Google Scholar 

  23. Becker D, Ammirati M, Black K, Canalis R, Andrews J (1991) Transzygomatic approach to tumours of the parasellar region. Technical note. Acta Neurochir Suppl 53: 89–91

    CAS  Google Scholar 

  24. Bejjani GK, Sekhar LN, Riedel CJ (2000) Occipitocervical fusion following the extreme lateral transcondylar approach. Surg Neurol 54: 109–115; discussion 115–116

    PubMed  CAS  Google Scholar 

  25. Benk V, Liebsch NJ, Munzenrider JE, Efird J, McManus P, Suit H (1995) Base of skull and cervical spine chordomas in children treated by high-dose irradiation. Int J Radiat Oncol Biol Phys 31: 577–581

    PubMed  CAS  Google Scholar 

  26. Bergh P, Kindblom LG, Gunterberg B, Remotti F, Ryd W, Meis-Kindblom JM (2000) Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer 88: 2122–2134

    PubMed  CAS  Google Scholar 

  27. Bernstein M, Gutin PH (1985) Interstitial irradiation of skull base tumours. Can J Neurol Sci 12: 366–370

    PubMed  CAS  Google Scholar 

  28. Berson AM, Castro JR, Petti P, Phillips TL, Gauger GE, Gutin P, Collier JM, Henderson SD, Baken K (1988) Charged particle irradiation of chordoma and chondrosarcoma of the base of skull and cervical spine: the Lawrence Berkeley Laboratory experience. Int J Radiat Oncol Biol Phys 15: 559–565

    PubMed  CAS  Google Scholar 

  29. Bootz F, Keiner S, Schulz T, Scheffler B, Seifert V (2001) Magnetic resonance imaging — guided biopsies of the petrous apex and petroclival region. Otol Neurotol 22: 383–388

    PubMed  CAS  Google Scholar 

  30. Borba L, Colli B, Al-Mefty O (2001) Skull base chordomas. Neurosurg Quart 11: 124–139

    Google Scholar 

  31. Borba LA, Al-Mefty O, Mrak RE, Suen J (1996) Cranial chordomas in children and adolescents. J Neurosurg 84: 584–591

    PubMed  CAS  Google Scholar 

  32. Borgel J, Olschewski H, Reuter T, Miterski B, Epplen JT (2001) Does the tuberous sclerosis complex include clivus chordoma? A case report. Eur J Pediatr 160: 138

    PubMed  CAS  Google Scholar 

  33. Bouropoulou V, Kontogeorgos G, Papamichales G, Bosse A, Roessner A, Vollmer E (1987) Differential diagnosis of chordoma immunohistochemical aspects. Arch Anat Cytol Pathol 35: 35–40

    PubMed  CAS  Google Scholar 

  34. Bouvier D, Raghuveer CV (2001) Aspiration cytology of metastatic chordoma to the orbit. Am J Ophthalmol 131: 279–280

    PubMed  CAS  Google Scholar 

  35. Bridge JA, Pickering D, Neff JR (1994) Cytogenetic and molecular cytogenetic analysis of sacral chordoma. Cancer Genet Cytogenet 75: 23–25

    PubMed  CAS  Google Scholar 

  36. Brooks JJ, LiVolsi VA, Trojanowski JQ (1987) Does chondroid chordoma exist? Acta Neuropathol 72: 229–235

    PubMed  CAS  Google Scholar 

  37. Brooks LJ, Afshani E, Hidalgo C, Fisher J (1981) Clivus chorodoma with pulmonary metastases appearing as failure to thrive. Am J Dis Child 135: 713–715

    PubMed  CAS  Google Scholar 

  38. Brown RV, Sage MR, Brophy BP (1990) CT and MR findings in patients with chordomas of the petrous apex. Am J Neuroradiol 11: 121–124

    PubMed  CAS  Google Scholar 

  39. Buchfelder M, Fahlbusch R, Ganslandt O, Stefan H, Nimsky C (2002) Use of intraoperative magnetic resonance imaging in tailored temporal lobe surgeries for epilepsy. Epilepsia 43: 864–873

    PubMed  Google Scholar 

  40. Buonamici L, Roncaroli F, Fioravanti A, Losi L, Van den Berghe H, Calbucci F, Dal Cin P (1999) Cytogenetic investigation of chordomas of the skull. Cancer Genet Cytogenet 112: 49–52

    PubMed  CAS  Google Scholar 

  41. Butler MG, Dahir GA, Hedges LK, Juliao HS, Sciadini M, Schwartz HS (1995) Cytogenetic, telomere, and telomerase studies in five surgically managed lumbosacral chordomas. Cancer Genet Cytogenet 85: 51–57

    PubMed  CAS  Google Scholar 

  42. Butler MG, Sciadini M, Hedges LK, Schwartz HS (1996) Chromosome telomere integrity of human solid neoplasms. Cancer Genet Cytogenet 86: 50–53

    PubMed  CAS  Google Scholar 

  43. Byrd SE, Harwood-Nash DC, Barry JF, Fitz CR, Boldt DW (1977) Coronal computed tomography of the skull and brain in infants and children. Part II. Clinical value. Radiology 124: 710–714

    PubMed  CAS  Google Scholar 

  44. Cappabianca P, Cavallo LM, Colao A, Del Basso De Caro M, Esposito F, Cirillo S, Lombardi G, de Divitiis E (2002) Endoscopic endonasal transsphenoidal approach: outcome analysis of 100 consecutive procedures. Minim Invasive Neurosurg 45: 193–200

    PubMed  CAS  Google Scholar 

  45. Casali PG, Messina A, Stacchiotti S, Tamborini E, Crippa F, Gronchi A, Orlandi R, Ripamonti C, Spreafico C, Bertieri R, Bertulli R, Colecchia M, Fumagalli E, Greco A, Grosso F, Olmi P, Pierotti MA, Pilotti S (2004) Imatinib mesylate in chordoma. Cancer 101: 2086–2097

    PubMed  CAS  Google Scholar 

  46. Casson PR, Bananno PC, Converse JM (1974) The midface degloving procedure. Plast Reconst Surg 53: 102–103

    PubMed  CAS  Google Scholar 

  47. Castro JR, Linstadt DE, Bahary JP, Petti PL, Daftari I, Collier JM, Gutin PH, Gauger G, Phillips TL (1994) Experience in charged particle irradiation of tumors of the skull base: 1977–1992. Int J Radiat Oncol Biol Phys 29: 647–655

    PubMed  CAS  Google Scholar 

  48. Catton C, O’Sullivan B, Bell R, Laperriere N, Cummings B, Fornasier V, Wunder J (1996) Chordoma: long-term follow-up after radical photon irradiation. RadiotherOncol 41: 67–72

    CAS  Google Scholar 

  49. Cha ST, Jarrahy R, Yong WH, Eby T, Shahinian HK (2002) A rare symptomatic presentation of ecchordosis physaliphora and unique endoscope-assisted surgical management. Minim Invasive Neurosurg 45: 36–40

    PubMed  CAS  Google Scholar 

  50. Chadduck WM (1973) Unusual lesions involving the sella turcica. South Med J 66: 948–955

    PubMed  CAS  Google Scholar 

  51. Chadduck WM, Boop FA, Sawyer JR (1991) Cytogenetic studies of pediatric brain and spinal cord tumors. Pediatr Neurosurg 17: 57–65

    PubMed  Google Scholar 

  52. Chambers PW, Schwinn CP (1979) Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 72: 765–776

    PubMed  CAS  Google Scholar 

  53. Chandler JP, Pelzer HJ, Bendok BB, Hunt Batjer H, Salehi SA (2005) Advances in surgical management of malignancies of the cranial base: the extended transbasal approach. J Neurooncol 73: 145–152

    PubMed  Google Scholar 

  54. Chang S, Khoo C, DePinho RA (2001) Modeling chromosomal instability and epithelial carcinogenesis in the telomerase-deficient mouse. Semin Cancer Biol 11: 227–239

    PubMed  CAS  Google Scholar 

  55. Chetty R, Levin CV, Kalan MR (1991) Chordoma: a 20-year clinicopathologic review of the experience at Groote Schuur Hospital, Cape Town. J Surg Oncol 46: 261–264

    PubMed  CAS  Google Scholar 

  56. Chugh R, Dunn R, Zalupski MM, Biermann JS, Sondak VK, Mace JR, Leu KM, Chandler WF, Baker LH (2005) Phase II study of 9-nitro-camptothecin in patients with advanced chordoma or soft tissue sarcoma. J Clin Oncol 23: 3597–3604

    PubMed  CAS  Google Scholar 

  57. Claus EB, Horlacher A, Hsu L, Schwartz RB, Dello-Iacono D, Talos F, Jolesz FA, Black PM (2005) Survival rates in patients with low-grade glioma after intraoperative magnetic resonance image guidance. Cancer 103: 1227–1233

    PubMed  Google Scholar 

  58. Coffin CM, Swanson PE, Wick MR, Dehner LP (1993) Chordoma in childhood and adolescence. A clinicopathologic analysis of 12 cases. Arch Pathol Lab Med 117: 927–933

    PubMed  CAS  Google Scholar 

  59. Colli B, Al-Mefty O (2001) Chordomas of the craniocervical junction: follow-up review and prognostic factors. J Neurosurg 95: 933–943

    PubMed  CAS  Google Scholar 

  60. Colli BO, Al-Mefty O (2001) Chordomas of the skull base: follow-up review and prognostic factors. Neurosurg Focus 10: Article 1

    Google Scholar 

  61. Congdon CC (1952) Proliferative lesions resembling chordoma following puncture of nucleus pulposus in rabbits. J Nat Cancer Inst 12: 893–907

    PubMed  CAS  Google Scholar 

  62. Couldwell WT, Sabit I, Weiss MH, Giannotta SL, Rice D (1997) Transmaxillary approach to the anterior cavernous sinus: a microanatomic study. Neurosurgery 40: 1307–1311

    PubMed  CAS  Google Scholar 

  63. Couldwell WT, Weiss MH, Rabb C, Liu JK, Apfelbaum RI, Fukushima T (2004) Variations on the standard transsphenoidal approach to the sellar region, with emphasis on the extended approaches and parasellar approaches: surgical experience in 105 cases. Neurosurgery 55: 539–547; discussion 547–550

    PubMed  Google Scholar 

  64. Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF (2001) Chordoma: a cytologic study with histologic and radiologic correlation. Cancer 93: 40–51

    PubMed  CAS  Google Scholar 

  65. Crockard A (1996) Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 38: 420

    PubMed  CAS  Google Scholar 

  66. Crockard A, Macaulay E, Plowman PN (1999) Stereotactic radiosurgery. VI. Posterior displacement of the brainstem facilitates safer high dose radiosurgery for clival chordoma. Br J Neurosurg 13: 65–70

    PubMed  CAS  Google Scholar 

  67. Crockard HA, Calder I, Ransford AO (1990) One-stage transoral decompression and posterior fixation in rheumatoid atlanto-axial subluxation. J Bone Joint Surg Br 72: 682–685

    PubMed  CAS  Google Scholar 

  68. Crockard HA, Cheeseman A, Steel T, Revesz T, Holton JL, Plowman N, Singh A, Crossman J (2001) A multidisciplinary team approach to skull base chondrosarcomas. J Neurosurg 95: 184–189

    PubMed  CAS  Google Scholar 

  69. Crockard HA, Steel T, Plowman N, Singh A, Crossman J, Revesz T, Holton JL, Cheeseman A (2001) A multidisciplinary team approach to skull base chordomas. J Neurosurg 95: 175–183

    PubMed  CAS  Google Scholar 

  70. Crumley RL, Gutin PH (1989) Surgical access for clivus chordoma. The University of California, San Francisco, experience. Arch Otolaryngol Head Neck Surg 115: 295–300

    PubMed  CAS  Google Scholar 

  71. Cummings BJ, Hodson DI, Bush RS (1983) Chordoma: the results of megavoltage radiation therapy. Int J Radiat Oncol Biol Phys 9: 633–642

    PubMed  CAS  Google Scholar 

  72. Cushing H (1912) The pituitary body and its disorders, clinical states produced by disorders of the hypophysis cerebri. J. B. Lippincott Company, Philadelphia, London

    Google Scholar 

  73. Dalpra L, Malgara R, Miozzo M, Riva P, Volonte M, Larizza L, Fuhrman Conti AM (1999) First cytogenetic study of a recurrent familial chordoma of the clivus. Int J Cancer 81: 24–30

    PubMed  CAS  Google Scholar 

  74. de Divitiis E, Cappabianca P, Cavallo LM (2002) Endoscopic transsphenoidal approach: adaptability of the procedure to different sellar lesions. Neurosurgery 51: 699–705; discussion 705–707

    PubMed  Google Scholar 

  75. DeBoer JM, Neff JR, Bridge JA (1992) Cytogenetics of sacral chordoma. Cancer Genet Cytogenet 64: 95–96

    PubMed  CAS  Google Scholar 

  76. Debus J, Hug EB, Liebsch NJ, O’Farrel D, Finkelstein D, Efird J, Munzenrider JE (1997) Brainstem tolerance to conformal radiotherapy of skull base tumors. Int J Radiat Oncol Biol Phys 39: 967–975

    PubMed  CAS  Google Scholar 

  77. Debus J, Schulz-Ertner D, Schad L, Essig M, Rhein B, Thillmann CO, Wannenmacher M (2000) Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int J Radiat Oncol Biol Phys 47: 591–596

    PubMed  CAS  Google Scholar 

  78. Delgado TE, Garrido E, Harwick RD (1981) Labiomandibular, transoral approach to chordomas in the clivus and upper cervical spine. Neurosurgery 8: 675–679

    PubMed  CAS  Google Scholar 

  79. Deniz ML, Kilic T, Almaata I, Kurtkaya O, Sav A, Pamir MN (2002) Expression of growth factors and structural proteins in chordomas: basic fibroblast growth factor, transforming growth factor alpha, and fibronectin are correlated with recurrence. Neurosurgery 51: 753–760; discussion 760

    PubMed  Google Scholar 

  80. Derome P (1972) Les tumeurs spheno-ethmoidales. Possibilites d’exerese et de reparation chirurgicale Neurochirurgie 18: 1–164

    Google Scholar 

  81. Derome PJ (1985) Surgical management of tumours invading the skull base. Can J Neurol Sci 12: 345–347

    PubMed  CAS  Google Scholar 

  82. Dolenc VV, Skrap M, Sustersic J, Skrbec M, Morina A (1987) A transcavernous-transsellar approach to the basilar tip aneurysms. Br J Neurosurg 1: 251–259

    PubMed  CAS  Google Scholar 

  83. Dolenc VV (1994) Frontotemporal epidural approach to trigeminal neurinomas. Acta Neurochir (Wien) 130: 55–65

    CAS  Google Scholar 

  84. Dolenc VV (1997) Transcranial epidural approach to pituitary tumors extending beyond the sella. Neurosurgery 41: 542–550; discussion 551–552

    PubMed  CAS  Google Scholar 

  85. Dorfman HD, Czerniak B (1995) Bone cancers. Cancer 75: 203–210

    PubMed  CAS  Google Scholar 

  86. Dort JC, Sutherland GR (2001) Intraoperative magnetic resonance imaging for skull base surgery. Laryngoscope 111: 1570–1575

    PubMed  CAS  Google Scholar 

  87. Doucet V, Peretti-Viton P, Figarella-Branger D, Manera L, Salamon G (1997) MRI of intracranial chordomas. Extent of tumour and contrast enhancement: criteria for differential diagnosis. Neuroradiology 39: 571–576

    PubMed  CAS  Google Scholar 

  88. Dow GR, Robson DK, Jaspan T, Punt JA (2003) Intradural cerebellar chordoma in a child: a case report and review of the literature. Childs Nerv Syst 19: 188–191

    PubMed  Google Scholar 

  89. Dreghorn CR, Newman RJ, Hardy GJ, Dickson RA (1990) Primary tumors of the axial skeleton. Experience of the leeds regional bone tumor registry. Spine 15: 137–140

    PubMed  CAS  Google Scholar 

  90. Eisenberg MB, Woloschak M, Sen C, Wolfe D (1997) Loss of heterozygosity in the retinoblastoma tumor suppressor gene in skull base chordomas and chondrosarcomas. Surg Neurol 47: 156–160; discussion 160–161

    PubMed  CAS  Google Scholar 

  91. Ekinci G, Akpinar IN, Baltacioglu F, Erzen C, Kilic T, Elmaci I, Pamir MN (2003) Earlypostoperative magnetic resonance imaging in glial tumors: prediction of tumor regrowth and recurrence. Eur J Radiol 45: 99–107

    PubMed  Google Scholar 

  92. El-Kalliny M, van Loveren H, Keller JT, Tew JM Jr (1992) Tumors of the lateral wall of the cavernous sinus. J Neurosurg 77: 508–514

    PubMed  CAS  Google Scholar 

  93. Enin IP (1964) Chordoma of the nasopharynx in 2 members of a family. Vestn Otorinolaringol 26: 88–90

    PubMed  CAS  Google Scholar 

  94. Erbengi A, Tekkok IH, Acikgoz B (1991) Posterior fossa chordomas — with special reference to transoral surgery. Neurosurg Rev 14: 23–28

    PubMed  CAS  Google Scholar 

  95. Erdem E, Angtuaco EC, Van Hemert R, Park JS, Al-Mefty O (2003) Comprehensive review of intracranial chordoma. Radiographics 23: 995–1009

    PubMed  Google Scholar 

  96. Eriksson B, Gunterberg B, Kindblom LG (1981) Chordoma. A clinicopathologic and prognostic study of a Swedish national series. Acta Orthop Scand 52: 49–58

    PubMed  CAS  Google Scholar 

  97. Fagundes MA, Hug EB, Liebsch NJ, Daly W, Efird J, Munzenrider JE (1995) 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 33: 579–584

    PubMed  CAS  Google Scholar 

  98. Fahlbusch R, Ganslandt O, Buchfelder M, Schott W, Nimsky C (2001) Intraoperative magnetic resonance imaging during transsphenoidal surgery. J Neurosurg 95: 381–390

    PubMed  CAS  Google Scholar 

  99. Falconer MA, Bailey IC, Duchen LW (1968) Surgical treatment of chordoma and chondroma of the skull base. J Neurosurg 29: 261–275

    PubMed  CAS  Google Scholar 

  100. Feigl GC, Bundschuh O, Gharabaghi A, Safavi-Abassi S, El Shawarby A, Samii M, Horstmann GA (2005) Evaluation of a new concept for the management of skull base chordomas and chondrosarcomas. J Neurosurg Suppl 102: 165–170

    Google Scholar 

  101. Ferry AP, Haddad HM, Goldman JL (1981) Orbital invasion by an intracranial chordoma. Am J Ophthalmol 92: 7–12

    PubMed  CAS  Google Scholar 

  102. Fisch U (1978) Infratemporal fossa approach to tumours of the temporal bone and base of the skull. J Laryngol Otol 92: 949–967

    PubMed  CAS  Google Scholar 

  103. Fischbein NJ, Kaplan MJ, Holliday RA, Dillon WP (2000) Recurrence of clival chordoma along the surgical pathway. Am J Neuroradiol 21: 578–583

    PubMed  CAS  Google Scholar 

  104. Fleming GF, Heimann PS, Stephens JK, Simon MA, Ferguson MK, Benjamin RS, Samuels BL (1993) Dedifferentiated chordoma. Response to aggressive chemotherapy in two cases. Cancer 72: 714–718

    PubMed  CAS  Google Scholar 

  105. Foote RF, Ablin G, Hall WW (1958) Chordoma in siblings. Calif Med 88: 383–386

    PubMed  CAS  Google Scholar 

  106. Forsyth PA, Cascino TL, Shaw EG, Scheithauer BW, O’Fallon JR, Dozier JC, Piepgras DG (1993) Intracranial chordomas: a clinicopathological and prognostic study of 51 cases. J Neurosurg 78: 741–747

    PubMed  CAS  Google Scholar 

  107. Fowler J (1989) The linear quadratic formula and progress in fractionated radiotherapy. Br J Radiol 62: 679–694

    PubMed  CAS  Google Scholar 

  108. Fraioli B, Esposito V, Santoro A, Iannetti G, Giuffre R, Cantore G (1995) Transmaxillosphenoidal approach to tumors invading the medial compartment of the cavernous sinus. J Neurosurg 82: 63–69

    PubMed  CAS  Google Scholar 

  109. Frank G, Sciarretta V, Calbucci F, Farneti G, Mazzatenta D, Pasquini E (2006) The endoscopic transnasal transsphenoidal approach for the treatment of cranial base chordomas and chondrosarcomas. Neurosurgery 59: ONS50–ONS57; discussion ONS50–ONS57

    PubMed  Google Scholar 

  110. Franquemont DW, Katsetos CD, Ross GW (1989) Fatal acute pontocerebellar hemorrhage due to an unsuspected spheno-occipital chordoma. Arch Pathol Lab Med 113: 1075–1078

    PubMed  CAS  Google Scholar 

  111. Fujita N, Miyamoto T, Imai J, Hosogane N, Suzuki T, Yagi M, Morita K, Ninomiya K, Miyamoto K, Takaishi H, Matsumoto M, Morioka H, Yabe H, Chiba K, Watanabe S, Toyama Y, Suda T (2005) CD24 is expressed specifically in the nucleus pulposus of intervertebral discs. Biochem Biophys Res Commun 338: 1890–1896

    PubMed  CAS  Google Scholar 

  112. Fuller DB, Bloom JG (1988) Radiotherapy for chordoma. Int J Radiat Oncol Biol Phys 15: 331–339

    PubMed  CAS  Google Scholar 

  113. Gadwal SR, Fanburg-Smith JC, Gannon FH, Thompson LD (2000) Primary chondrosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 14 cases with a review of the literature. Cancer 88: 2181–2188

    PubMed  CAS  Google Scholar 

  114. Gay E, Sekhar LN, Rubinstein E, Wright DC, Sen C, Janecka IP, Snyderman CH (1995) Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36: 887–896; discussion 896–897

    PubMed  CAS  Google Scholar 

  115. Gehanne C, Delpierre I, Damry N, Devroede B, Brihaye P, Christophe C (2005) Skull base chordoma: CT and MRI features. Jbr-Btr 88: 325–327

    PubMed  CAS  Google Scholar 

  116. George B, Dematons C, Cophignon J (1988) Lateral approach to the anterior portion of the foramen magnum. Application to surgical removal of 14 benign tumors: technical note. Surg Neurol 29: 484–490

    PubMed  CAS  Google Scholar 

  117. George B, Ferrario CA, Blanquet A, Kolb F (2003) Cavernous sinus exenteration for invasive cranial base tumors. Neurosurgery 52: 772–780; discussion 780–782

    PubMed  Google Scholar 

  118. Gibas Z, Miettinen M, Sandberg AA (1992) Chromosomal abnormalities in two chordomas. Cancer Genet Cytogenet 58: 169–173

    PubMed  CAS  Google Scholar 

  119. Gil Z, Fliss DM, Voskoboinik N, Leider-Trejo L, Spektor S, Yaron Y, Orr-Urtreger A (2004) Cytogenetic analysis of three variants of clival chordoma. Cancer Genet Cytogen 154: 124–130

    CAS  Google Scholar 

  120. Glosser G, McManus P, Munzenrider J, Austin-Seymour M, Fullerton B, Adams J, Urie MM (1997) Neuropsychological function in adults after high dose fractionated radiation therapy of skull base tumors. Int J Radiat Oncol Biol Phys 38: 231–239

    PubMed  CAS  Google Scholar 

  121. Goel A (1995) Extended middle fossa approach for petroclival lesions. Acta Neurochir (Wien) 135: 78–83

    CAS  Google Scholar 

  122. Goel A (1995) Chordoma and chondrosarcoma: relationship to the internal carotid artery. Acta Neurochir (Wien) 133: 30–35

    CAS  Google Scholar 

  123. Goel A, Muzumdar DP, Nitta J (2001) Surgery on lesions involving cavernous sinus. J Clin Neurosci 8Suppl 1: 71–77

    PubMed  Google Scholar 

  124. Gollin SM, Janecka IP (1994) Cytogenetics of cranial base tumors. J Neurooncol 20: 241–254

    PubMed  CAS  Google Scholar 

  125. Gormley WB, Beckman ME, Ho KL, Boyd SB, Rock JP (1995) Primary craniofacial chordoma: case report. Neurosurgery 36: 1196–1199

    PubMed  CAS  Google Scholar 

  126. Gottschalk D, Fehn M, Patt S, Saeger W, Kirchner T, Aigner T (2001) Matrix gene expression analysis and cellular phenotyping in chordoma reveals focal differentiation pattern of neoplastic cells mimicking nucleus pulposus development [see comment]. Am J Pathol 158: 1571–1578

    PubMed  CAS  Google Scholar 

  127. Gutin PH, Leibel SA, Hosobuchi Y, Crumley RL, Edwards MS, Wilson CB, Lamb S, Weaver KA (1987) Brachytherapy of recurrent tumors of the skull base and spine with iodine-125 sources. Neurosurgery 20: 938–945

    PubMed  CAS  Google Scholar 

  128. Gwak HS, Yoo HJ, Youn SM, Chang U, Lee DH, Yoo SY, Rhee CH (2005) Hypofractionated stereotactic radiation therapy for skull base and upper cervical chordoma and chondrosarcoma: preliminary results. Stereotact Funct Neurosurg 83: 233–243

    PubMed  Google Scholar 

  129. Habrand JL, Mammar H, Ferrand R, Pontvert D, Bondiau PY, Kalifa C, Zucker JM (1999) Proton beam therapy (PT) in the management of CNS tumors in childhood. Strahlenther Onkol 175(Suppl 2): 91–94

    PubMed  Google Scholar 

  130. Haeckel C, Krueger S, Kuester D, Ostertag H, Samii M, Buehling F, Broemme D, Czerniak B, Roessner A (2000) Expression of cathepsin K in chordoma. Hum Pathol 31: 834–840

    PubMed  CAS  Google Scholar 

  131. Hakuba A, Nishimura S, Inoue Y (1985) Transpetrosal-transtentorial approach and its application in the therapy of retrochiasmatic craniopharyngiomas. Surg Neurol 24: 405–415

    PubMed  CAS  Google Scholar 

  132. Hakuba A, Tanaka K, Suzuki T, Nishimura S (1989) A comined orbitozygomatic infratemporal epidural and subdural approach for lesions involving the entire cavernous sinus’. J Neurosurg 71: 699–704

    PubMed  CAS  Google Scholar 

  133. Halperin EC (1997) Why is female sex an independent predictor of shortened overall survival after proton/photon radiation therapy for skull base chordomas? Int J Radiat Oncol Biol Phys 38: 225–230

    PubMed  CAS  Google Scholar 

  134. Handa J, Suzuki F, Nioka H, Koyama T (1987) Clivus chordoma in childhood. Surg Neurol 28: 58–62

    PubMed  CAS  Google Scholar 

  135. Hara T, Kawahara N, Tsuboi K, Shibahara J, Ushiku T, Kirino T (2006) Sarcomatous transformation of clival chordoma after charged-particle radiotherapy. Report of two cases. J Neurosurg 105: 136–141

    PubMed  Google Scholar 

  136. Hardy J, Vezina JL (1976) Transsphenoidal neurosurgery of intracranial neoplasm. Adv Neurol 15: 261–273

    PubMed  CAS  Google Scholar 

  137. Harsh G, Ojemann R, Varvares M, Swearingen B, Cheney M, Joseph M (2001) Pedicled rhinotomy for clival chordomas invaginating the brainstem. Neurosurg Focus 10: E8

    PubMed  CAS  Google Scholar 

  138. Harvey WF, Dawson EK (1941) Chordoma. Edinburgh Med J 48: 713–721

    Google Scholar 

  139. Hasse H (1857) Ein neuer Fall von Schleimgeschwulst am Clivus. Virch Arch 11

    Google Scholar 

  140. Hazarika D, Kumar RV, Muniyappa GD, Mukherjee G, Rao CR, Narasimhamurthy NK, Shenoy AM, Nanjundappa (1995) Diagnosis of clival chordoma by fine needle aspiration of an oropharyngeal mass. A case report. Acta Cytol 39: 507–510

    PubMed  CAS  Google Scholar 

  141. Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW (1973) Chordomas and cartilaginous tumors at the skull base. Cancer 32: 410–420

    PubMed  CAS  Google Scholar 

  142. Higinbotham NL, Phillips RF, Farr HW, Hustu HO (1967) Chordoma. Thirty-five-year study at Memorial Hospital. Cancer 20: 1841–1850

    PubMed  CAS  Google Scholar 

  143. Ho KL (1985) Ecchordosis physaliphora and chordoma: a comparative ultrastructural study. Clin Neuropathol 4: 77–86

    PubMed  CAS  Google Scholar 

  144. Hoch BL, Nielsen GP, Liebsch NJ, Rosenberg AE (2006) Base of skull chordomas in children and adolescents: a clinicopathologic study of 73 cases. Am J Surg Pathol 30: 811–818

    PubMed  Google Scholar 

  145. Honeybul S, Neil-Dwyer D, Lang DA, Evans BT (2001) Extended transbasal approach with preservation of olfaction: ananatomical study. Br J Oral Maxillofac Surg 39: 149–157

    PubMed  CAS  Google Scholar 

  146. Horiguchi H, Sano T, Qian ZR, Hirokawa M, Kagawa N, Yamaguchi T, Hirose T, Nagahiro S (2004) Expression of cell adhesion molecules in chordomas: an immunohistochemical study of 16 cases. Acta Neuropathol (Berl) 107: 91–96

    PubMed  CAS  Google Scholar 

  147. Horten BC, Montague SR (1976) In vitro characteristics of a sacrococcygeal chordoma maintained in tissue and organ culture systems. Acta Neuropathologica 35: 13–25

    PubMed  CAS  Google Scholar 

  148. Horten BC, Montague SR (1976) Human ecchordosis physaliphora and chick embryonic notochord. A comparative electron microscopic study. Virchows Arch A Pathol Anat Histol 371: 295–303

    PubMed  CAS  Google Scholar 

  149. House WF, Shelton C (1992) Middle fossa approach for acoustic tumor removal. Otolaryngol Clin 25: 347–359

    CAS  Google Scholar 

  150. Hruban RH, Traganos F, Reuter VE, Huvos AG (1990) Chordomas with malignant spindle cell components. A DNA flow cytometric and immunohistochemical study with histogenetic implications. Am J Pathol 137: 435–447

    PubMed  CAS  Google Scholar 

  151. Hug EB, Loredo LN, Slater JD, DeVries A, Grove RI, Schaefer RA, Rosenberg AE, Slater JM (1999) Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 91: 432–439

    PubMed  CAS  Google Scholar 

  152. Hug EB (2001) Review of skull base chordomas: prognostic factors and long-term results of proton-beam radiotherapy. Neurosurg Focus 10: E11

    PubMed  CAS  Google Scholar 

  153. Hug EB, Sweeney RA, Nurre PM, Holloway KC, Slater JD, Munzenrider JE (2002) Proton radiotherapy in management of pediatric base of skull tumors. Int J Radiat Oncol Biol Phys 52: 1017–1024

    PubMed  Google Scholar 

  154. Igaki H, Tokuuye K, Okumura T, Sugahara S, Kagei K, Hata M, Ohara K, Hashimoto T, Tsuboi K, Takano S, Matsumura A, Akine Y (2004) Clinical results of proton beam therapy for skull base chordoma. Int J Radiat Oncol Biol Phys 60: 1120–1126

    PubMed  Google Scholar 

  155. Jackson IT (1990) Craniofacial osteotomies to facilitate the resection of the tumors of the skull base. McGraw-Hill, Health Professions Division, New York

    Google Scholar 

  156. Jeffrey PB, Davis RL, Biava C, Rosenblum M (1993) Microtubule aggregates in a clival chordoma. Arch Pathol Lab Med 117: 1055–1057

    PubMed  CAS  Google Scholar 

  157. Jeffrey PB, Biava CG, Davis RL (1995) Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation. Am J Clin Pathol 103: 271–279

    PubMed  CAS  Google Scholar 

  158. Jho HD, Carrau RL, McLaughlin ML, Somaza SC (1996) Endoscopic transsphenoidal resection of a large chordoma in the posterior fossa. Case report. Neurosurg Focus 1: e3; discussion 1p following e3

    PubMed  CAS  Google Scholar 

  159. Jho HD, Carrau RL (1997) Endoscopic endonasal transsphenoidal surgery: experience with 50 patients. J Neurosurg 87: 44–51

    PubMed  CAS  Google Scholar 

  160. Jho HD, Carrau RL, McLaughlin MR, Somaza SC (1997) Endoscopic transsphenoidal resection of a large chordoma in the posterior fossa. Acta Neurochir (Wien) 139: 343–347; discussion 347–348

    CAS  Google Scholar 

  161. Kakuno Y, Yamada T, Hirano H, Mori H, Narabayashi I (2002) Chordoma in the sella turcica. Neurol Med Chir (Tokyo) 42: 305–308

    Google Scholar 

  162. Kamrin RP, Potanos JN, Pool JL (1964) An evaluation of the diagnosis and treatment of chordoma. J Neurol Neurosurg Psychiatry 27: 157–165

    Google Scholar 

  163. Kassam A, Snyderman CH, Mintz A, Gardner P, Carrau RL (2005) Expanded endonasal approach: the rostrocaudal axis. Part II. Posterior clinoids to the foramen magnum. Neurosurg Focus 19: E4

    PubMed  Google Scholar 

  164. Kawase T (1990) Skull base approaches for clival tumors. No Shinkei Geka 18: 121–128

    PubMed  CAS  Google Scholar 

  165. Kawase T (1997) Anatomical strategy of parasellar tumor surgery. No Shinkei Geka 25: 681–688

    PubMed  CAS  Google Scholar 

  166. Kelley MJ, Korczak JF, Sheridan E, Yang X, Goldstein AM, Parry DM (2001) Familial chordoma, a tumor of notochordal remnants, is linked to chromosome 7q33. Am J Hum Genet 69: 454–460

    PubMed  CAS  Google Scholar 

  167. Kerr WA, Allen KL, Haynes DR, Sellars SL (1975) Letter: familial nasopharyngeal chordoma. S Afr Med J 49: 1584

    PubMed  CAS  Google Scholar 

  168. Kilgore S, Prayson RA (2002) Apoptotic and proliferative markers in chordomas: a study of 26 tumors. Ann Diagn Pathol 6: 222–228

    PubMed  Google Scholar 

  169. Kilic T, Ekinci G, Seker A, Elmaci I, Erzen C, Pamir MN (2001) Determining optimal MRI follow-up after transsphenoidal surgery for pituitary adenoma: scan at 24 hours postsurgery provides reliable information. Acta Neurochir (Wien) 143: 1103–1126

    CAS  Google Scholar 

  170. Kimura F, Kim KS, Friedman H, Russell EJ, Breit R (1990) MR imaging of normal and abnormal clivus. Am J Neuroradiol 11: 1015–1021

    PubMed  CAS  Google Scholar 

  171. Kitano M, Taneda M (2001) Extended transsphenoidal approach with submucosal posterior ethmoidectomy for parasellar tumors: technical note. J Neurosurg 94: 999–1004

    PubMed  CAS  Google Scholar 

  172. Klebs E (1864) Ein Fall von Ecchondrosis spheno-occipitalise amylacea. Virchows Arch Path Anat 31: 396–399

    Google Scholar 

  173. Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK (2002) The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol 61: 215–225; discussion 226–229

    PubMed  Google Scholar 

  174. Klingler L, Shooks J, Fiedler PN, Marney A, Butler MG, Schwartz HS (2000) Microsatellite instability in sacral chordoma. J Surg Oncol 73: 100–103

    PubMed  CAS  Google Scholar 

  175. Knisely JP, Linskey ME (2006) Less common indications for stereotactic radiosurgery or fractionated radiotherapy for patients with benign brain tumors. Neurosurg Clin N Am 17: 149–167, vii

    PubMed  Google Scholar 

  176. Knutsen T, Gobu V, Knaus R, Padilla-Nash H, Augustus M, Strausberg RL, Kirsch IR, Sirotkin K, Ried T (2005) The interactive online SKY=M-FISH & CGH database and the Entrez cancer chromosomes search database: linkage of chromosomal aberrations with the genome sequence. Genes Chromosomes Cancer 44: 52–64

    PubMed  CAS  Google Scholar 

  177. Kölliker A (1860) Über die Beziehungen der Chorda dorsalis zur Bildung der Wirbel der Selachier und einiger andern Fische. Verhandl. phys. med. Gesellsch. Würzburg 10: 193–242

    Google Scholar 

  178. Kombogiorgas D, St George EJ, Chapman S, English M, Solanki GA (2006) Infantile clivus chordoma without clivus involvement: case report and review of the literature. Childs Nerv Syst 22(10): 1369–1374

    PubMed  CAS  Google Scholar 

  179. Kondziolka D, Lunsford LD, Flickinger JC (1991) The role of radiosurgery in the management of chordoma and chondrosarcoma of the cranial base. Neurosurgery 29: 38–45; discussion 45–46

    PubMed  CAS  Google Scholar 

  180. Kouri JG, Chen MY, Watson JC, Oldfield EH (2000) Resection of suprasellar tumors by using a modified transsphenoidal approach. Report of four cases. J Neurosurg 92: 1028–1035

    PubMed  CAS  Google Scholar 

  181. Koybasioglu F, Simsek GG, Onal BU, Han U, Adabag A (2005) Oropharyngeal chordoma diagnosed by fine needle aspiration: a case report. Acta Cytol 49: 173–176

    PubMed  Google Scholar 

  182. Krayenbühl H, Yasargil MG (1975) Cranial chordomas. Progr Neurol Surg 6: 380–434

    Google Scholar 

  183. Krishnan S, Foote RL, Brown PD, Pollock BE, Link MJ, Garces YI (2005) Radiosurgery for cranial base chordomas and chondrosarcomas. Neurosurgery 56: 777–784; discussion 777–784

    PubMed  Google Scholar 

  184. Kumar PP, Good RR, Leibrock LG, Mawk JR, Yonkers AJ, Ogren FP (1988) High activity iodine 125 endocurietherapy for recurrent skull base tumors. Cancer 61: 1518–1527

    PubMed  CAS  Google Scholar 

  185. Kumar PP, Good RR, Skultety FM, Leibrock LG (1988) Local control of recurrent clival and sacral chordoma after interstitial irradiation with iodine-125: new techniques for treatment of recurrent or unresectable chordomas. Neurosurgery 22: 479–483

    PubMed  CAS  Google Scholar 

  186. Kurokawa H, Miura S, Goto T (1988) Ecchordosis physaliphora arising from the cervical vertebra, the CT and MRI appearance. Neuroradiology 30: 81–83

    PubMed  CAS  Google Scholar 

  187. Kurtsoy A, Menku A, Tucer B, Suat Oktem I, Akdemir H, Kemal Koc R (2004) Transbasal approaches: surgical details, pitfalls and avoidances. Neurosurg Rev 27: 267–273

    PubMed  Google Scholar 

  188. Kuzniacka A, Mertens F, Strombeck B, Wiegant J, Mandahl N (2004) Combined binary ratio labeling fluorescence in situ hybridization analysis of chordoma. Cancer Genet Cytogenet 151: 178–181

    PubMed  CAS  Google Scholar 

  189. Kyoshima K, Matsuo K, Kushima H, Oikawa S, Idomari K, Kobayashi S (2002) Degloving transfacial approach with Le Fort I and nasomaxillary osteotomies: alternative transfacial approach. Neurosurgery 50: 813–821

    PubMed  Google Scholar 

  190. Lalwani AK, Kaplan MJ, Gutin PH (1992) The transsphenoethmoid approach to the sphenoid sinus and clivus. Neurosurgery 31: 1008–1014; discussion 1014

    PubMed  CAS  Google Scholar 

  191. Lam R (1990) The nature of cytoplasmic vacuoles in chordoma cells. A correlative enzyme and electron microscopic histochemical study. Pathol Res Pract 186: 642–650

    PubMed  CAS  Google Scholar 

  192. Lanzino G, Hirsch WL, Pomonis S, Sen CN, Sekhar LN (1992) Cavernous sinus tumors: neuroradiologic and neurosurgical considerations on 150 operated cases. J Neurosurg Sci 36: 183–196

    PubMed  CAS  Google Scholar 

  193. Lanzino G, Sekhar LN, Hirsch WL, Sen CN, Pomonis S, Snyderman CH (1993) Chordomas and chondrosarcomas involving the cavernous sinus: review of surgical treatment and outcome in 31 patients. Surg Neurol 40: 359–371

    PubMed  CAS  Google Scholar 

  194. Lanzino G, Dumont AS, Lopes MB, Laws ER Jr (2001) Skull base chordomas: overview of disease, management options, and outcome. Neurosurg Focus 10: E12

    PubMed  CAS  Google Scholar 

  195. Larson TC 3rd, Houser OW, Laws ER Jr (1987) Imaging of cranial chordomas. Mayo Clin Proc 62: 886–893

    PubMed  Google Scholar 

  196. Laws ER Jr, Trautmann JC, Hollenhorst RW Jr (1977) Transsphenoidal decompression of the optic nerve and chiasm. Visual results in 62 patients. J Neurosurg 46: 717–722

    PubMed  Google Scholar 

  197. Laws ER Jr (1984) Transsphenoidal surgery for tumors of the clivus. Otolaryngol Head Neck Surg 92: 100–101

    PubMed  Google Scholar 

  198. Laws ER Jr, Fode NC, Redmond MJ (1985) Transsphenoidal surgery following unsuccessful prior therapy. An assessment of benefits and risks in 158 patients. J Neurosurg 63: 823–829

    PubMed  Google Scholar 

  199. Laws ER Jr (2001) Skull base chordomas: overview of disease, management options, and outcome. Neurosurg Focus 10(3): Article 12

    Google Scholar 

  200. Layfield LJ, Liu K, Dodd LG, Olatidoye BA (1998) “Dedifferentiated” chordoma: a case report of the cytomorphologic findings on fine-needle aspiration. Diagn Cytopathol 19: 378–381

    PubMed  CAS  Google Scholar 

  201. Lee-Jones L, Aligianis I, Davies PA, Puga A, Farndon PA, Stemmer-Rachamimov A, Ramesh V, Sampson JR (2004) Sacrococcygeal chordomas in patients with tuberous sclerosis complex show somatic loss of TSC1 or TSC2. Genes Chromosomes Cancer 41: 80–85

    PubMed  CAS  Google Scholar 

  202. Lee HJ, Kalnin AJ, Holodny AI, Schulder M, Grigorian A, Sharer LR (1998) Hemorrhagic chondroid chordoma mimicking pituitary apoplexy. Neuroradiology 40: 720–723

    PubMed  CAS  Google Scholar 

  203. Leproux F, de Toffol B, Aesch B, Cotty P (1993) MRI of cranial chordomas: the value of gadolinium. Neuroradiology 35: 543–545

    PubMed  CAS  Google Scholar 

  204. Lim GH (1975) Clivus chordoma with unusual bone sclerosis and brainsteminvasion. A case report with review of the radiology of cranial chordomas. Australas Radiol 19: 242–250

    PubMed  CAS  Google Scholar 

  205. Linck (1909) Chordoma malignum. Zieglers Beitr 46

    Google Scholar 

  206. Linsenmayer TF, Gibney E, Schmid TM (1986) Segmental appearance of type X collagen in the developing avian notochord. Dev Biol 113: 467–473

    PubMed  CAS  Google Scholar 

  207. Losi L, Tomasich G (1996) Dedifferentiated (sarcomatoid) chordoma in the base of the skull. Report of a case. Pathologica 88: 514–518

    PubMed  CAS  Google Scholar 

  208. Luschka H (1857) Über gallertartige Auswüchse am Clivus Blumenbachii. Virch Arch 11: 8–12

    Google Scholar 

  209. Mabrey R (1935) Chordoma: a study of 150 cases. Am J Cancer 25: 501–517

    Google Scholar 

  210. Macdonald RL, Deck JH (1990) Immunohistochemistry of ecchordosis physaliphora and chordoma. Can J Neurol Sci 17: 420–423

    PubMed  CAS  Google Scholar 

  211. Magrini SM, Papi MG, Marletta F, Tomaselli S, Cellai E, Mungai V, Biti G (1992) Chordomanatural history, treatment and prognosis. The Florence Radiotherapy Department experience (1956–1990) and a critical review of the literature. Acta Oncol 31: 847–851

    PubMed  CAS  Google Scholar 

  212. Maira G, Pallini R, Anile C, Fernandez E, Salvinelli F, La Rocca LM, Rossi GF (1996) Surgical treatment of clival chordomas: the transsphenoidal approach revisited. J Neurosurg 85: 784–792

    PubMed  CAS  Google Scholar 

  213. Makhmudov UB, Tcherekayev VA, Tanyashin SV (1992) Transoral approach to tumors of the clivus: report of two cases. J Craniofac Surg 3: 35–38

    PubMed  CAS  Google Scholar 

  214. Mandahl N, Heim S, Arheden K, Rydholm A, Willen H, Mitelman F (1990) Chromosomal rearrangements in chondromatous tumors. Cancer 65: 242–248

    PubMed  CAS  Google Scholar 

  215. Mapstone TB, Kaufman B, Ratcheson RA (1983) Intradural chordoma without bone involvement: nuclear magnetic resonance (NMR) appearance. Case report. J Neurosurg 59: 535–537

    PubMed  CAS  Google Scholar 

  216. Marshman LA, Gunasekera L, Rose PE, Olney JS (2001) Primary intracerebral mesenchymal chondrosarcoma with rhabdomyosarcomatous differentiation: case report and literature review. Br J Neurosurg 15: 419–424

    PubMed  CAS  Google Scholar 

  217. Matsumoto J, Towbin RB, Ball WS Jr (1989) Cranial chordomas in infancy and childhood. A report of two cases and review of the literature. Pediatr Radiol 20: 28–32

    PubMed  CAS  Google Scholar 

  218. Matsumoto K, Tamesa N, Tomita S, Tamiya T, Furuta T, Ohmoto T (1997) Stereotactic brachytherapy for clival chordoma. No Shinkei Geka 25: 919–925

    PubMed  CAS  Google Scholar 

  219. McDermott MW, Gutin PH (1996) Image-guided surgery for skull base neoplasms using the ISG viewing wand. Anatomic and technical considerations. Neurosurg Clin N Am 7: 285–295

    PubMed  CAS  Google Scholar 

  220. McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM (2001) Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 12: 1–11

    PubMed  CAS  Google Scholar 

  221. Mehnert F, Beschorner R, Kuker W, Hahn U, Nagele T (2004) Retroclival ecchordosis physaliphora: MR imaging and review of the literature. Am J Neuroradiol 25: 1851–1855

    PubMed  Google Scholar 

  222. Meis JM, Raymond AK, Evans HL, Charles RE, Giraldo AA (1987) “Dedifferentiated” chordoma. A clinicopathologic and immunohistochemical study of three cases. Am J Surg Pathol 11: 516–52

    PubMed  CAS  Google Scholar 

  223. Meis JM, Giraldo AA (1988) Chordoma. An immunohistochemical study of 20 cases. Arch Pathol Lab Med 112: 553–556

    PubMed  CAS  Google Scholar 

  224. Menezes AH, VanGilder JC (1988) Transoral-transpharyngeal approach to the anterior craniocervical junction. Ten-year experience with 72 patients. J Neurosurg 69: 895–903

    PubMed  CAS  Google Scholar 

  225. Menezes AH, Gantz BJ, Traynelis VC, McCulloch TM(1997) Cranial base chordomas. Clin Neurosurg 44: 491–509

    PubMed  CAS  Google Scholar 

  226. Mertens F, Kreicbergs A, Rydholm A, Willen H, Carlen B, Mitelman F, Mandahl N (1994) Clonal chromosome aberrations in three sacral chordomas. Cancer Genet Cytogenet 73: 147–151

    PubMed  CAS  Google Scholar 

  227. Meyer JE, Oot RF, Lindfors KK (1986) CT appearance of clival chordomas. J Comput Assist Tomogr 10: 34–38

    PubMed  CAS  Google Scholar 

  228. Meyers SP, Hirsch WL Jr, Curtin HD, Barnes L, Sekhar LN, Sen C (1992) Chondrosarcomas of the skull base: MR imaging features. Radiology 184: 103–108

    PubMed  CAS  Google Scholar 

  229. Meyers SP, Hirsch WL Jr, Curtin HD, Barnes L, Sekhar LN, Sen C (1992) Chordomas of the skull base: MR features. Am J Neuroradiol 13: 1627–1636

    PubMed  CAS  Google Scholar 

  230. Mills RP (1984) Chordomas of the skull base. J R Soc Med 77: 10–16

    PubMed  CAS  Google Scholar 

  231. Miozzo M, Dalpra L, Riva P, Volonta M, Macciardi F, Pericotti S, Tibiletti MG, Cerati M, Rohde K, Larizza L, Fuhrman Conti AM (2000) A tumor suppressor locus in familial and sporadic chordoma maps to 1p36. Int J Cancer 87: 68–72

    PubMed  CAS  Google Scholar 

  232. Mitchell A, Scheithauer BW, Unni KK, Forsyth PJ, Wold LE, McGivney DJ (1993) Chordoma and chondroid neoplasms of the spheno-occiput. An immunohistochemical study of 41 cases with prognostic and nosologic implications. Cancer 72: 2943–2949

    PubMed  CAS  Google Scholar 

  233. Miyagi A, Maeda K, Sugawara T (1998) Usefulness of neuroendoscopy and a neuronavigator for removal of clival chordoma. No Shinkei Geka 26: 169–175

    PubMed  CAS  Google Scholar 

  234. Mondal A, Mukherjee PK (1988) Cytological and cytochemical diagnosis of chordoma by fine needle aspiration biopsy — a case report. Indian J Pathol Microbiol 31: 64–66

    PubMed  CAS  Google Scholar 

  235. Moreira-Gonzalez A, Pieper DR, Balaguer Cambra J, Simman R, Jackson IT (2005) Skull base tumors: a comprehensive review of transfacial swing osteotomy approaches. Plast Reconstr Surg 115: 711–720

    PubMed  CAS  Google Scholar 

  236. Mortini P, Roberti F, Kalavakonda C, Nadel A, Sekhar LN (2003) Endoscopic and microscopic extended subfrontal approach to the clivus: a comparative anatomical study. Skull Base 13: 139–147

    PubMed  Google Scholar 

  237. Müller H (1858) Ü Uber das Vorkommen von Resten des Chorda dorsalis bei Menschen nach der Geburt und über ihr Verhältniss zu den Gallertgeschwülesten am Clivus. Z Ration Med 2: 202–229

    Google Scholar 

  238. Munzenrider JE, Liebsch NJ (1999) Proton therapy for tumors of the skull base. Strahlenther Onkol 175Suppl 2: 57–63

    PubMed  Google Scholar 

  239. Muthukumar N, Kondziolka D, Lunsford LD, Flickinger JC (1998) Stereotactic radiosurgery for chordoma and chondrosarcoma: further experiences. Int J Radiat Oncol Biol Phys 41: 387–392

    PubMed  CAS  Google Scholar 

  240. Nagib MG, Wisiol ES, Simonton SC, Levinson RM (1990) Transoral labiomandibular approach to basiocciput chordomas in childhood. Childs Nerv Syst 6: 126–130

    PubMed  CAS  Google Scholar 

  241. Naka T, Boltze C, Samii A, Herold C, Ostertag H, Iwamoto Y, Oda Y, Tsuneyoshi M, Kuester D, Roessner A (2003) Skull base and nonskull base chordomas: clinicopathologic and immunohistochemical study with special reference to nuclear pleomorphism and proliferative ability. Cancer 98: 1934–1941

    PubMed  Google Scholar 

  242. Naka T, Boltze C, Kuester D, Schulz TO, Samii A, Herold C, Ostertag H, Roessner A (2004) Expression of matrix metalloproteinase (MMP)-1, MMP-2, MMP-9, cathepsin B, and urokinase plasminogen activator in non-skull base chordoma. Am J Clin Pathol 122: 926–930

    PubMed  CAS  Google Scholar 

  243. Naka T, Boltze C, Kuester D, Samii A, Herold C, Ostertag H, Iwamoto Y, Oda Y, Tsuneyoshi M, Roessner A (2005) Intralesional fibrous septum in chordoma: a clinicopathologic and immunohistochemical study of 122 lesions. Am J Clin Pathol 124: 288–294

    PubMed  Google Scholar 

  244. Naka T, Boltze C, Kuester D, Schulz T, Schneider-Stock R, Kellner A, Samii A, Herold C, Ostertag H, Roessner A (2005) Alterations of G1-S checkpoint in chordoma: the prognostic impact of p53 overexpression. Cancer 104: 1255–1263

    PubMed  CAS  Google Scholar 

  245. Ng SH, Ko SF, Wan YL, Tang LM, Ho YS (1998) Cervical ecchordosis physaliphora: CT and MR features. Br J Radiol 71: 329–331

    PubMed  CAS  Google Scholar 

  246. Nijhawan VS, Rajwanshi A, Das A, Jayaram N, Gupta SK (1989) Fine-needle aspiration cytology of sacrococcygeal chordoma. Diagn Cytopathol 5: 404–407

    PubMed  CAS  Google Scholar 

  247. Nimsky C, Ganslandt O, Von Keller B, Romstock J, Fahlbusch R (2004) Intraoperative high-field-strength MR imaging: implementation and experience in 200 patients. Radiology 233: 67–78

    PubMed  Google Scholar 

  248. Nimsky C, Ganslandt O, Buchfelder M, Fahlbusch R (2006) Intraoperative visualization for resection of gliomas: the role of functional neuronavigation and intraoperative 1.5 T MRI. Neurol Res 28: 482–487

    PubMed  Google Scholar 

  249. Noel G, Habrand JL, Jauffret E, de Crevoisier R, Dederke S, Mammar H, Haie-Meder C, Pontvert D, Hasboun D, Ferrand R, Boisserie G, Beaudre A, Gaboriaud G, Guedea F, Petriz L, Mazeron JJ (2003) Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine. Prognostic factors and patterns of failure. Strahlenther Onkol 179: 241–248

    PubMed  Google Scholar 

  250. Noel G, Feuvret L, Calugaru V, Dhermain F, Mammar H, Haie-Meder C, Ponvert D, Hasboun D, Ferrand R, Nauraye C, Boisserie G, Beaudre A, Gaboriaud G, Mazal A, Habrand JL, Mazeron JJ (2005) Chordomas of the base of the skull and upper cervical spine. One hundred patients irradiated by a 3D conformal technique combining photon and proton beams. Acta Oncol 44: 700–708

    PubMed  Google Scholar 

  251. O’Neill P, Bell BA, Miller JD, Jacobson I, Guthrie W (1985) Fifty years of experience with chordomas in southeast Scotland. Neurosurgery 16: 166–170

    PubMed  CAS  Google Scholar 

  252. Ogi H, Kiryu H, Hori Y, Fukui M (1995) Cutaneous metastasis of CNS chordoma. Am J Dermatopathol 17: 599–602

    PubMed  CAS  Google Scholar 

  253. Oikawa S, Kyoshima K, Goto T, Iwashita T, Takizawa T, Kobayashi S, Ito M (2001) Histological study on local invasiveness of clival chordoma. Case report of autopsy. Acta Neurochir (Wien) 143: 1065–1069

    CAS  Google Scholar 

  254. Okada Y, Aoki S, Barkovich AJ, et al (1989) Cranial bone marrow in children: assessment of normal development with mr imaging. Radiology 171: 161–164

    PubMed  CAS  Google Scholar 

  255. Oot RF, Melville GE, New PF, Austin-Seymour M, Munzenrider J, Pile-Spellman J, Spagnoli M, Shoukimas GM, Momose KJ, Carroll R, et al (1988) The role of MR and CT in evaluating clival chordomas and chondrosarcomas. AJR Am J Roentgenol 151: 567–575

    PubMed  CAS  Google Scholar 

  256. Paavolainen P, Teppo L (1976) Chordoma in Finland. Acta Orthop Scand 47: 46–51

    PubMed  CAS  Google Scholar 

  257. Pai HH, Thornton A, Katznelson L, Finkelstein DM, Adams JA, Fullerton BC, Loeffler JS, Leibsch NJ, Klibanski A, Munzenrider JE (2001) Hypothalamic/pituitary function following high-dose conformal radiotherapy to the base of skull: demonstration of a dose-effect relationship using dose-volume histogramanalysis. Int J Radiat Oncol Biol Phys 49: 1079–1092

    PubMed  CAS  Google Scholar 

  258. Pallini R, Maira G, Pierconti F, Falchetti ML, Alvino E, Cimino-Reale G, Fernandez E, D’Ambrosio E, Larocca LM (2003) Chordoma of the skull base: predictors of tumor recurrence. J Neurosurg 98: 812–822

    PubMed  Google Scholar 

  259. Pamir MN, Kilic T, Ture U, Ozek MM (2004) Multimodality management of 26 skull-base chordomas with 4-year mean follow-up: experience at a single institution. Acta Neurochir (Wien) 146: 343–354; discusion 354

    CAS  Google Scholar 

  260. Pamir MN, Kilic T, Ozek MM, Ozduman K, Ture U (2006) Non-meningeal tumours of the cavernous sinus: a surgical analysis. J Clin Neurosci 13: 626–635

    PubMed  Google Scholar 

  261. Pamir MN, Ozduman K (2006) Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas. Eur J Radiol 58: 461–470

    PubMed  Google Scholar 

  262. Pamir MN, Peker S, Ozek MM, Dincer A (2006) Intraoperative MR imaging: preliminary results with 3 tesla MR system. Acta Neurochir Suppl 98: 97–100

    PubMed  CAS  Google Scholar 

  263. Parkinson D (2000) Extradural neural axis compartment. J Neurosurg 92: 585–588

    PubMed  CAS  Google Scholar 

  264. Patten BM (1968) Human ebryology. McGraw Hill, New York, pp 430–431

    Google Scholar 

  265. Pearlman AW, Friedman M (1970) Radical radiation therapy of chordoma. Am J Roentgenol Radium Ther Nucl Med 108: 332–341

    PubMed  CAS  Google Scholar 

  266. Peker S, Kurtkaya-Yapicier O, Kilic T, Pamir MN (2005) Microsurgical anatomy of the lateral walls of the pituitary fossa. Acta Neurochir (Wien) 147: 641–648; discussion 649

    CAS  Google Scholar 

  267. Persons DL, Bridge JA, Neff JR (1991) Cytogenetic analysis of two sacral chordomas. Cancer Genet Cytogenet 56: 197–201

    PubMed  CAS  Google Scholar 

  268. Petti PL (1996) Evaluation of a pencil-beam dose calculation technique for charged particle radiotherapy. Int J Radiat Oncol Biol Phys 35: 1049–1057

    PubMed  CAS  Google Scholar 

  269. Placzek M (1995) The role of the notochord and floor plate in inductive interactions. Curr Opin Genet Dev 5: 499–506

    PubMed  CAS  Google Scholar 

  270. Price CH, Jeffree GM (1977) Incidence of bone sarcoma in SW England, 1946–74, in relation to age, sex, tumour site and histology. Br J Cancer 36: 511–522

    PubMed  CAS  Google Scholar 

  271. Probst EN, Zanella FE, Vortmeyer AO (1993) Congenital clivus chordoma. Am J Neuroradiol 14: 537–539

    PubMed  CAS  Google Scholar 

  272. Rabadan A, Conesa H (1992) Transmaxillary-transnasal approach to the anterior clivus: a microsurgical anatomical model. Neurosurgery 30: 473–481; discussion 482

    PubMed  CAS  Google Scholar 

  273. Radner H, Katenkamp D, Reifenberger G, Deckert M, Pietsch T, Wiestler OD (2001) New developments in the pathology of skull base tumors. Virchows Arch 438: 321–335

    PubMed  CAS  Google Scholar 

  274. Raffel C, Wright DC, Gutin PH, Wilson CB (1985) Cranial chordomas: clinical presentation and results of operative and radiation therapy in twenty-six patients. Neurosurgery 17: 703–710

    PubMed  CAS  Google Scholar 

  275. Raso JL, Gusmao S (2006) Transbasal approach to skull base tumors: evaluation and proposal of classification. Surg Neurol 65(Suppl 1) S1: 33-1:37; discussion 1: 37-1:38

    PubMed  Google Scholar 

  276. Reifenberger G, Weber T, Weber RG, Wolter M, Brandis A, Kuchelmeister K, Pilz P, Reusche E, Lichter P, Wiestler OD (1999) Chordoid glioma of the third ventricle: immunohistochemical and molecular genetic characterization of a novel tumor entity. Brain Pathol 9: 617–626

    PubMed  CAS  Google Scholar 

  277. Rengachary SS, Grotte DA, Swanson PE (1997) Extradural ecchordosis physaliphora of the thoracic spine: case report. Neurosurgery 41: 1198–1201; discussion 1201–1202

    PubMed  CAS  Google Scholar 

  278. Rhomberg W, Eiter H, Bohler F, Dertinger S (2006) Combined radiotherapy and razoxane in the treatment of chondrosarcomas and chordomas. Anticancer Res 26: 2407–2411

    PubMed  CAS  Google Scholar 

  279. Ribbert H (1895) Über die experimentelle Erzeugung einer Ecchondrosis physalifora. Verhandl. d. XIII. Kongr f inn Med 13: 455–464

    Google Scholar 

  280. Ribbert H (1904) Geschwülstlehre für Arzte und studierende, Bonn

    Google Scholar 

  281. Ribbert H, Virchow R (1959) Chordoma

    Google Scholar 

  282. Rich TA, Schiller A, Suit HD, Mankin HJ (1985) Clinical and pathologic review of 48 cases of chordoma. Cancer 56: 182–187

    PubMed  CAS  Google Scholar 

  283. Richardson MS (2001) Pathology of skull base tumors. Otolaryngol Clin North Am 34: 1025–1042

    PubMed  CAS  Google Scholar 

  284. Riva P, Crosti F, Orzan F, Dalpra L, Mortini P, Parafioriti A, Pollo B, Fuhrman Conti AM, Miozzo M, Larizza L (2003) Mapping of candidate region for chordoma development to 1p36.13 by LOH analysis. Int J Cancer 107: 493–497

    PubMed  CAS  Google Scholar 

  285. Roche PH, Malca SA, Payan MJ, Pellet W (1995) Chondrosarcoma of the skull base. Apropos of sphenotemporal localization and review of the literature. Neurochirurgie 41: 353–358

    PubMed  CAS  Google Scholar 

  286. Rodriguez L, Colina J, Lopez J, Molina O, Cardozo J (1999) Intradural prepontine growth: giant ecchordosis physaliphora or extraosseous chordoma? Neuropathology 19: 336–340

    Google Scholar 

  287. Romero J, Cardenes H, la Torre A, Valcarcel F, Magallon R, Regueiro C, Aragon G (1993) Chordoma: results of radiation therapy in eighteen patients. Radiother Oncol 29: 27–32

    PubMed  CAS  Google Scholar 

  288. Rone R, Ramzy I, Duncan D (1986) Anaplastic sacrococcygeal chordoma. Fine needle aspiration cytologic findings and embryologic considerations. Acta Cytol 30: 183–188

    PubMed  CAS  Google Scholar 

  289. Rosenberg AE, Brown GA, Bhan AK, Lee JM (1994) Chondroid chordoma — a variant of chordoma. A morphologic and immunohistochemical study. Am J Clin Pathol 101: 36–41

    PubMed  CAS  Google Scholar 

  290. Rosenberg AE, Nielsen GP, Keel SB, Renard LG, Fitzek MM, Munzenrider JE, Liebsch NJ (1999) Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma. Am J Surg Pathol 23: 1370–1378

    PubMed  CAS  Google Scholar 

  291. Rosenberg AE, Nielsen GP, Keel SB (2000) Lipoid chordoma. Mod Pathol 13: 15a

    Google Scholar 

  292. Saad AG, Collins MH (2005) Prognostic value of MIB-1, E-cadherin, and CD44 in pediatric chordomas. Pediatr Dev Pathol 8: 362–368

    PubMed  CAS  Google Scholar 

  293. Sabit I, Schaefer SD, Couldwell WT (2000) Extradural extranasal combined transmaxillary transsphenoidal approach to the cavernous sinus: a minimally invasive microsurgical model. Laryngoscope 110: 286–291

    PubMed  CAS  Google Scholar 

  294. Saito A, Hasegawa T, Shimoda T, Toda G, Hirohashi S, Tajima G, Moriya Y (1998) Dedifferentiated chordoma: a case report. Jpn J Clin Oncol 28: 766–771

    PubMed  CAS  Google Scholar 

  295. Salisbury JR, Isaacson PG (1985) Demonstration of cytokeratins and an epithelial membrane antigen in chordomas and human fetal notochord. Am J Surg Pathol 9: 791–797

    PubMed  CAS  Google Scholar 

  296. Salisbury JR (1993) The pathology of the human notochord. J Pathol 171: 253–255

    PubMed  CAS  Google Scholar 

  297. Salisbury JR, Deverell MH, Cookson MJ, Whimster WF (1993) Three-dimensional reconstruction of human embryonic notochords: clue to the pathogenesis of chordoma. J Pathol 171: 59–62

    PubMed  CAS  Google Scholar 

  298. Samii M, Ammirati M (1988) The combined supra-infratentorial pre-sigmoid sinus avenue to the petro-clival region. Surgical technique and clinical applications. Acta Neurochir (Wien) 95: 6–12

    CAS  Google Scholar 

  299. Sandberg AA, Bridge JA (2003) Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: chondrosarcoma and other cartilaginous neoplasms. Cancer Genet Cytogenet 143: 1–31

    PubMed  CAS  Google Scholar 

  300. Santoni R, Liebsch N, Finkelstein DM, Hug E, Hanssens P, Goitein M, Smith AR, O’Farrell D, Efird JT, Fullerton B, Munzenrider JE (1998) Temporal lobe (TL) damage following surgery and high-dose photon and proton irradiation in 96 patients affected by chordomas and chondrosarcomas of the base of the skull. Int J Radiat Oncol Biol Phys 41: 59–68

    PubMed  CAS  Google Scholar 

  301. Sautner D, Saeger W, Ludecke DK (1993) Tumors of the sellar region mimicking pituitary adenomas. Exp Clin Endocrinol 101: 283–289

    PubMed  CAS  Google Scholar 

  302. Sawamura Y, Terasaka S, Fukushima T (1999) Extended transsphenoidal approach with sigma-shape osteotomy of the maxilla: technical note. Skull Base Surg 9: 119–125

    PubMed  CAS  Google Scholar 

  303. Sawyer JR, Husain M, Al-Mefty O (2001) Identification of isochromosome 1q as a recurring chromosome aberration in skull base chordomas: a new marker for aggressive tumors? Neurosurg Focus 10: E6

    PubMed  CAS  Google Scholar 

  304. Scheil S, Bruderlein S, Liehr T, Starke H, Herms J, Schulte M, Moller P (2001) Genomewide analysis of sixteen chordomas by comparative genomic hybridization and cytogenetics of the first human chordoma cell line, U-CH1. Genes Chromosomes Cancer 32: 203–211

    PubMed  CAS  Google Scholar 

  305. Schisano G, Tovi D (1962) Clivus chordomas. Neurochirurgia (Stuttg) 5: 99–120

    PubMed  CAS  Google Scholar 

  306. Schonegger K, Gelpi E, Prayer D, Dieckmann K, Matula C, Hassler M, Hainfellner JA, Marosi C (2005) Recurrent and metastatic clivus chordoma: systemic palliative therapy retards disease progression. Anticancer Drugs 16: 1139–1143

    PubMed  Google Scholar 

  307. Schulz-Ertner D, Haberer T, Jakel O, Thilmann C, Kramer M, Enghardt W, Kraft G, Wannenmacher M, Debus J (2002) Radiotherapy for chordomas and low-grade chondrosarcomas of the skull base with carbon ions. Int J Radiat Oncol Biol Phys 53: 36–42

    PubMed  Google Scholar 

  308. Schulz-Ertner D, Nikoghosyan A, Thilmann C, Haberer T, Jakel O, Karger C, Scholz M, Kraft G, Wannenmacher M, Debus J (2003) Carbon ion radiotherapy for chordomas and low-grade chondrosarcomas of the skull base. Results in 67 patients. Strahlenther Onkol 179: 598–605

    PubMed  Google Scholar 

  309. Sefiani S, Amarti A, Maher M, Benkiran L, Saidi A (2002) Dedifferentiated chordoma of the skull base. A case report. Neurochirurgie 48: 436–439

    PubMed  CAS  Google Scholar 

  310. Seifert G, Donath K (1998) [Cranial and cervical chordomas. A differential diagnostic problem]. Mund Kiefer Gesichtschir 2: 153–159

    PubMed  CAS  Google Scholar 

  311. Seifert V, Laszig R (1991) Transoral transpalatal removal of a giant premesencephalic clivus chordoma. Acta Neurochir (Wien) 112: 141–146

    CAS  Google Scholar 

  312. Sekhar LN, Moller AR (1986) Operative management of tumors involving the cavernous sinus. J Neurosurg 64: 879–889

    PubMed  CAS  Google Scholar 

  313. Sekhar LN, Nanda A, Sen CN, Snyderman CN, Janecka IP (1992) The extended frontal approach to tumors of the anterior, middle, and posterior skull base. JNeurosurg 76: 198–206

    CAS  Google Scholar 

  314. Sekhar LN, Janecka IP (1993) Surgery of cranial base tumors. Raven Press, New York

    Google Scholar 

  315. Sekhar LN, Pranatartiharan R, Chanda A, Wright DC (2001) Chordomas and chondrosarcomas of the skull base: results and complications of surgical management. Neurosurg Focus 10: E2

    PubMed  CAS  Google Scholar 

  316. Sen C, Triana A (2001) Cranial chordomas: results of radical excision. Neurosurg Focus 10: E3

    PubMed  CAS  Google Scholar 

  317. Sen CN, Sekhar LN, Schramm VL, Janecka IP (1989) Chordoma and chondrosarcoma of the cranial base: an 8-year experience. Neurosurgery 25: 931–940; discussion 940–941

    PubMed  CAS  Google Scholar 

  318. Sepehrnia A, Samii M, Tatagiba M (1991) Management of intracavernous tumours: an 11-year experience. Acta Neurochir Suppl 53: 122–126

    CAS  Google Scholar 

  319. Shugar JM, Som PM, Krespi YP, Arnold LM, Som ML (1980) Primary chordoma of the maxillary sinus. Laryngoscope 90: 1825–1830

    PubMed  CAS  Google Scholar 

  320. Sibley RK, Day DL, Dehner LP, Trueworthy RC (1987) Metastasizing chordoma in early childhood: a pathological and immunohistochemical study with review of the literature. Pediatr Pathol 7: 287–301

    PubMed  CAS  Google Scholar 

  321. Slater JM, Slater JD, Archambeau JO (1995) Proton therapy for cranial base tumors. J Craniofac Surg 6: 24–26

    PubMed  CAS  Google Scholar 

  322. Spallone A, Tcherekarjev VA, Korshunov AG (1996) Chondrosarcoma of the anterior cranial fossa. Report of two cases. J Neurosurg Sci 40: 115–120

    PubMed  CAS  Google Scholar 

  323. Spetzler RF, Herman JM, Beals S, Joganic E, Milligan J (1993) Preservation of olfaction in anterior craniofacial approaches. J Neurosurg 79: 48–52

    PubMed  CAS  Google Scholar 

  324. Spiro RH, Gerold FP, Strong EW (1981) Mandibular “swing” approach for oral and oropharyngeal tumors. Head Neck Surg 3: 37

    Google Scholar 

  325. Steiner and Fischer (1907) Über ein malignes chordom der Schaedel-Rückgradshöhle. Beitr Path Anat 40: 109–119

    Google Scholar 

  326. Stemple DL (2005) Structure and function of the notochord: an essential organ for chordate development. Development 132: 2503–2512

    PubMed  CAS  Google Scholar 

  327. Stepanek J, Cataldo SA, Ebersold MJ, Lindor NM, Jenkins RB, Unni K, Weinshenker BG, Rubenstein RL (1998) Familial chordoma with probable autosomal dominant inheritance. Am J Med Genet 75: 335–336

    PubMed  CAS  Google Scholar 

  328. Stewart M, Morin J (1926) Chordoma: a review, with report of a new sacrococcygeal case. J Pathol Bacterial 29: 41–60

    Google Scholar 

  329. Stough DR, Hartzog JT, Fisher RG (1971) Unusual intradural spinal metastasis of a cranial chordoma. Case report. J Neurosurg 34: 560–562

    PubMed  CAS  Google Scholar 

  330. Sundaresan N (1986) Chordomas. Clin Orthop Relat Res: 135–142

    Google Scholar 

  331. Sweet MB, Thonar EJ, Berson SD, Skikne MI, Immelman AR, Kerr WA (1979) Biochemical studies of the matrix of craniovertebral chordoma and a metastasis. Cancer 44: 652–660

    PubMed  CAS  Google Scholar 

  332. Sze G, Uichanco LS 3rd, Brant-Zawadzki MN, Davis RL, Gutin PH, Wilson CB, Norman D, Newton TH (1988) Chordomas: MR imaging. Radiology 166: 187–191

    PubMed  CAS  Google Scholar 

  333. Tai PT, Craighead P, Bagdon F (1995) Optimization of radiotherapy for patients with cranial chordoma. A review of dose-response ratios for photon techniques. Cancer 75: 749–756

    PubMed  CAS  Google Scholar 

  334. Tallini G, Dorfman H, Brys P, Dal Cin P, De Wever I, Fletcher CD, Jonson K, Mandahl N, Mertens F, Mitelman F, Rosai J, Rydholm A, Samson I, Sciot R, Van den Berghe H, Vanni R, Willen H (2002) Correlation between clinicopathological features and karyotype in 100 cartilaginous and chordoid tumours. A report from the Chromosomes and Morphology (CHAMP) Collaborative Study Group. J Pathol 196: 194–203

    PubMed  Google Scholar 

  335. Tamaki N, Nagashima T, Ehara K, Motooka Y, Barua KK (2001) Surgical approaches and strategies for skull base chordomas. Neurosurg Focus 10: E9

    PubMed  CAS  Google Scholar 

  336. Taniguchi K, Tateishi A, Higaki S, Igarashi M, Hayashi V, Inoue S, Shoji H (1984) Type of collagen in chordoma. Nippon Seikeigeka Gakkai Zasshi 58: 829–834

    PubMed  CAS  Google Scholar 

  337. Terahara A, Niemierko A, Goitein M, Finkelstein D, Hug E, Liebsch N, O’Farrell D, Lyons S, Munzenrider J (1999) Analysis of the relationship between tumor dose inhomogeneity and local control in patients with skull base chordoma. Int J Radiat Oncol Biol Phys 45: 351–358

    PubMed  CAS  Google Scholar 

  338. Tewfik HH, McGinnis WL, Nordstrom DG, Latourette HB (1977) Chordoma: evaluation of clinical behavior and treatment modalities. Int J Radiat Oncol Biol Phys 2: 959–962

    PubMed  CAS  Google Scholar 

  339. Thodou E, Kontogeorgos G, Scheithauer BW, Lekka I, Tzanis S, Mariatos P, Laws ER Jr (2000) Intrasellar chordomas mimicking pituitary adenoma. J Neurosurg 92: 976–982

    PubMed  CAS  Google Scholar 

  340. Toda H, Kondo A, Iwasaki K (1998) Neuroradiological characteristics of ecchordosis physaliphora. Case report and review of the literature. J Neurosurg 89: 830–834

    PubMed  CAS  Google Scholar 

  341. Tomlinson FH, Scheithauer BW, Forsythe PA, Unni KK, Meyer FB (1992) Sarcomatous transformation in cranial chordoma. Neurosurgery 31: 13–18

    PubMed  CAS  Google Scholar 

  342. Tsai EC, Santoreneos S, Rutka JT (2002) Tumors of the skull base in children: review of tumor types and management strategies. Neurosurg Focus 12: e1

    PubMed  Google Scholar 

  343. Tuite GF, Veres R, Crockard HA (1996) Pediatric transoral surgery: indications, complications, and long-term outcome. J Neurosurg 84: 573–583

    PubMed  CAS  Google Scholar 

  344. Ture U, Pamir MN (2002) Extreme lateral-transatlas approach for resection of the dens of the axis. J Neurosurg 96: 73–82

    PubMed  Google Scholar 

  345. Ture U, Pamir MN (2004) Small petrosal approach to the middle portion of the mediobasal temporal region: technical case report. Surg Neurol 61(1): 60–67

    PubMed  Google Scholar 

  346. Tzortzidis F, Elahi F, Wright D, Natarajan SK, Sekhar LN (2006) Patient outcome at longterm follow-up after aggressive microsurgical resection of cranial base chordomas. Neurosurgery 59: 230–237; discussion 230–237

    PubMed  Google Scholar 

  347. Tzortzidis F, Elahi F, Wright DC, Temkin N, Natarajan SK, Sekhar LN (2006) Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chondrosarcomas. Neurosurgery 58: 1090–1098; discussion 1090–1098

    PubMed  Google Scholar 

  348. Uggowitzer MM, Kugler C, Groell R, Lindbichler F, Radner H, Sutter B, Ranner G (1999) Drop metastases in a patient with a chondroid chordoma of the clivus. Neuroradiology 41: 504–507

    PubMed  CAS  Google Scholar 

  349. Ulich TR, Mirra JM (1982) Ecchordosis physaliphora vertebralis. Clin Orthop Relat Res 163: 282–289

    PubMed  Google Scholar 

  350. Unni KK (1996) Dahlin’s bone tumors: general aspects and data on 11087 cases, 5 edn. Lippincot-Raven, Phhiladelphia, PA

    Google Scholar 

  351. Urie MM, Fullerton B, Tatsuzaki H, Birnbaum S, Suit HD, Convery K, Skates S, Goitein M (1992) A dose response analysis of injury to cranial nerves and/or nuclei following proton beam radiation therapy. Int J Radiat Oncol Biol Phys 23: 27–39

    PubMed  CAS  Google Scholar 

  352. Utne JR, Pugh DG (1955) The roentgenologic aspects of chordoma. Am J Roentgenol Radium Ther Nucl Med 74: 593–608

    PubMed  CAS  Google Scholar 

  353. Uttley D, Moore A, Archer DJ (1989) Surgical management of midline skull-base tumors: a new approach. J Neurosurg 71: 705–710

    PubMed  CAS  Google Scholar 

  354. Valderrama E, Kahn LB, Lipper S, Marc J (1983) Chondroid chordoma. Electronmicroscopic study of two cases. Am J Surg Pathol 7: 625–632

    PubMed  CAS  Google Scholar 

  355. Van Loveren HR, Fernandez PM, Keller JT, Tew JM Jr, Shumrick K (1994) Neurosurgical applications of Le Fort I-type osteotomy. Clin Neurosurg 41: 425–443

    PubMed  Google Scholar 

  356. Verma K, Murthy L, Kapila K (1986) Cytologic diagnosis of chordoma by fine needle aspiration. Indian J Pathol Microbiol 29: 189–191

    PubMed  CAS  Google Scholar 

  357. Virchow RLK (1857) Untersuchungen über die Entwickelung des Schädelgrundes im gesunden und krankhaften Zustande, und über den Einfluss derselben auf Schädelform, Gesichtsbildung und Gehirnbau. G. Reimer, Berlin

    Google Scholar 

  358. Volpe NJ, Liebsch NJ, Munzenrider JE, Lessell S (1993) Neuro-ophthalmologic findings in chordoma and chondrosarcoma of the skull base. Am J Ophthalmol 115: 97–104

    PubMed  CAS  Google Scholar 

  359. Volpe R, Mazabraud A (1983) A clinicopathologic review of 25 cases of chordoma (a pleomorphic and metastasizing neoplasm). Am J Surg Pathol 7: 161–170

    PubMed  CAS  Google Scholar 

  360. Vougioukas VI, Hubbe U, Schipper J, Spetzger U (2003) Navigated transoral approach to the cranial base and the craniocervical junction: technical note. Neurosurgery 52: 247–250; discussion 251

    PubMed  Google Scholar 

  361. Walaas L, Kindblom LG (1991) Fine-needle aspiration biopsy in the preoperative diagnosis of chordoma: a study of 17 cases with application of electron microscopic, histochemical, and immunocytochemical examination. Hum Pathol 22: 22–28

    PubMed  CAS  Google Scholar 

  362. Watanabe A, Yanagita M, Ishii R, Shirabe T (1994) Magnetic resonance imaging of ecchordosis physaliphora — case report. Neurol Med Chir (Tokyo) 34: 448–450

    CAS  Google Scholar 

  363. Watkins L, Khudados ES, Kaleoglu M, et al. (1993) Skull base chordomas: a review of 38 patients, 1958–1988. Br J Neurosurg 7: 241–248

    PubMed  CAS  Google Scholar 

  364. Weber AL, Liebsch NJ, Sanchez R, Sweriduk ST Jr (1994) Chordomas of the skull base. Radiologic and clinical evaluation. Neuroimaging Clin N Am 4: 515–527

    PubMed  CAS  Google Scholar 

  365. Weber DC, Rutz HP, Pedroni ES, Bolsi A, Timmermann B, Verwey J, Lomax AJ, Goitein G (2005) Results of spot-scanning proton radiation therapy for chordoma and chondrosarcoma of the skull base: the Paul Scherrer Institut experience. Int J Radiat Oncol Biol Phys 63: 401–409

    PubMed  Google Scholar 

  366. Weinberger P, Yu Z, Kowalski D, Joe J, Manger P, Psyrri A, Sasaki C (2005) Differential expression of epidermal growth factor receptor, c-Met, and HER2/neu in chordoma compared with 17 other malignancies. Arch Otolaryngol Head Neck Surg 131: 707–711

    PubMed  Google Scholar 

  367. Wojno KJ, Hruban RH, Garin-Chesa P, Huvos AG (1992) Chondroid chordomas and low-grade chondrosarcomas of the craniospinal axis. An immunohistochemical analysis of 17 cases. Am J Surg Pathol 16: 1144–1152

    PubMed  CAS  Google Scholar 

  368. Wold LE, Laws ER Jr (1983) Cranial chordomas in children and young adults. J Neurosurg 59: 1043–1047

    PubMed  CAS  Google Scholar 

  369. Wolfe JT 3rd, Scheithauer BW (1987) “Intradural chordoma” or “giant ecchordosis physaliphora”? Report of two cases. Clin Neuropathol 6: 98–103

    PubMed  Google Scholar 

  370. Wyatt RB, Schochet SS Jr, McCormick WF (1971) Ecchordosis physaliphora. An electron microscopic study. J Neurosurg 34: 672–677

    PubMed  CAS  Google Scholar 

  371. Yamada T, Placzek M, Tanaka H, Dodd J, Jessell TM (1991) Control of cell pattern in the developing nervous system: polarizing activity of the floor plate and notochord. Cell 64: 635–647

    PubMed  CAS  Google Scholar 

  372. Yamaguchi T, Yamato M, Saotome K (2002) 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 31: 413–418

    PubMed  Google Scholar 

  373. Yamaguchi T, Suzuki S, Ishiiwa H, Shimizu K, Ueda Y (2004) Benign notochordal cell tumors: a comparative histological study of benign notochordal cell tumors, classic chordomas, and notochordal vestiges of fetal intervertebral discs. Am J Surg Pathol 28: 756–761

    PubMed  Google Scholar 

  374. Yamaguchi T, Suzuki S, Ishiiwa H, Ueda Y (2004) Intraosseous benign notochordal cell tumours: overlooked precursors of classic chordomas? Histopathology 44: 597–602

    PubMed  CAS  Google Scholar 

  375. Yamaguchi T, Watanabe-Ishiiwa H, Suzuki S, Igarashi Y, Ueda Y (2005) Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors. Mod Pathol 18: 1005–1010

    PubMed  Google Scholar 

  376. Yang XR, Beerman M, Bergen AW, Parry DM, Sheridan E, Liebsch NJ, Kelley MJ, Chanock S, Goldstein AM (2005) Corroboration of a familial chordoma locus on chromosome 7q and evidence of genetic heterogeneity using single nucleotide polymorphisms (SNPs). Int J Cancer 116: 487–491

    PubMed  CAS  Google Scholar 

  377. Yasargil MG (1996) Microneurosurgery. Thieme Verlag, Stuttgart, New York

    Google Scholar 

  378. Zenker FA (1857) Ueber die gallertgeschwülste des Clivus Blumenbachii (Ecchondrosis prolifera, Virchow). Virch Arch 12: 108–110

    Google Scholar 

  379. Zhou L, Guo H, Li S, Ji Y, Huang F (1995) An extensive subfrontal approach to the lesions involving the skull base. Chin Med J (Engl) 108: 407–412

    PubMed  CAS  Google Scholar 

  380. Zorlu F, Gurkaynak M, Yildiz F, Oge K, Atahan IL (2000) Conventional external radiotherapy in the management of clivus chordomas with overt residual disease. Neurol Sci 21: 203–207

    PubMed  CAS  Google Scholar 

  381. Zülch KJ, Christensen E (1956) Pathologische Anatomie der raumbeengenden intrakraniellen Prozesse. Springer, Berlin, Heidelberg New York

    Google Scholar 

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Authors and Affiliations

  1. Marmara University Faculty of Medicine, Department of Neurosurgery, Marmara University Institute of Neurological Sciences, Istanbul, Turkey

    M. N. Pamir & K. Özduman

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  1. M. N. Pamir
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  2. K. Özduman
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Editors and Affiliations

  1. Cambridge

    J. D. Pickard

  2. Ankara

    N. Akalan

  3. Roma

    C. Di Rocco

  4. Ljubljana

    V. V. Dolenc

  5. Lisbon

    J. Lobo Antunes

  6. Groningen

    J. J. A. Mooij

  7. Bonn

    J. Schramm

  8. Lyon

    M. Sindou

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Pamir, M.N., Özduman, K. (2008). Tumor-biology and current treatment of skull-base chordomas. In: Pickard, J.D., et al. Advances and Technical Standards in Neurosurgery. Advances and Technical Standards in Neurosurgery, vol 33. Springer, Vienna. https://doi.org/10.1007/978-3-211-72283-1_2

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  • DOI: https://doi.org/10.1007/978-3-211-72283-1_2

  • Publisher Name: Springer, Vienna

  • Print ISBN: 978-3-211-72282-4

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