Chordomas of the craniocervical junction: follow-up review and prognostic factors

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Object. Chordomas are rare tumors that arise from the remnants of the notochord. Because of their deep location, local infiltrative nature, and involvement of surrounding bone, treatment of chordomas is a challenge. In this study the authors analyze the data and prognostic factors obtained during the follow-up period (range 1–150 months, median 38 months) in 53 patients with craniocervical junction chordomas and 10 patients with chondrosarcomas.

Methods. Several surgical approaches were used, and some tumor excisions required staged procedures. Survival was calculated according the Kaplan—Meier method. Statistical analysis was performed using Fisher exact, log rank and Kruskal—Wallis tests. Radical/subtotal resection was achieved in 77.8% of the patients. The mortality rate during the follow-up period was 14.3%. In patients harboring chondrosarcoma better 5-year recurrence-free survival (RFS) rates were demonstrated than in those with chordoma (100% and 50.7%, respectively). Histological patterns (typical or chondroid chordomas) and patient age at onset of symptoms had no effect on the RFS rates. Radical/subtotal resections were associated with better RFS rates than partial resection. Adjuvant proton-beam therapy was shown to increase the RFS rates compared with conventional radiotherapy (90.9% and 19.4%, respectively, at 4 years posttreatment). Karyotypically abnormal tumors were associated with the worst RFS rates compared with karyotypically normal lesions (44.5% and 90.3%, respectively, at 3 years). Cases of cranial nerve palsy followed by those with cerebrospinal fluid leakages were the most frequent postoperative complications. Permanent postoperative neurological deficit was observed in 28.6% of the patients.

Conclusions. A better prognosis was observed in patients with chondrosarcoma compared with those harboring chordoma. Histological pattern and patient age at symptom onset were not factors that influenced prognosis in cases of chordoma. Extensive resection and possibly adjuvant proton-beam therapy provided better prognoses for these patients.

Article Information

Address reprint requests to: Benedicto O. Colli, M.D., Departmento de Cirurgia, HCFMRP, Campus Universitário USP, 14048–900 Ribeirão Preto, São Paulo, Brazil. email: bocolli@fmrp.usp.br.

© AANS, except where prohibited by US copyright law.

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Figures

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    Magnetic resonance T1-weighted images obtained preoperatively and immediately postoperatively in a patient with a chordoma. The patient underwent left maxillotomy with transoral and transsephenoidal approaches and total microsurgical resection of the tumor. Upper Left: Axial contrast-enhanced image revealing a large mass lesion with the epicenter in the clivus and extension into the posterior fossa. In addition to extending into the posterior retropharyngeal soft tissue, the lesion is producing lateral brainstem compression. The tumor moderately enhances with gadolinium. Upper Right: Postoperative axial image demonstrating an increased signal area in the retropharyngeal soft tissue and an approximately 1.5-cm-diameter enhancing lesion on the left side, lateral to the brainstem. These are residual lesions and demonstrated no progression from a previous examination. The area occupied by tumor is filled with fat. Lower Left: Coronal image revealing a large hypointense mass lesion with the epicenter in the clivus and extension to the right lateral mass of the atlas. The lesion is compressing the brainstem inferiorly. Lower Right: Postoperative coronal image demonstrating enhancement of contrast around the tumor bed (residual tumor or postoperative changes).

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    Graphs depicting RFS curves. Upper: Survival curves in all 63 patients, 53 with chordomas, and 10 with chondrosarcomas. There was no significant difference among the curves (p = 0.1141, df = 2, Kruskal—Wallis nonparametric ANOVA test). The Dunn multiple comparisons posttest showed a significant difference between curves for patients with chordomas and those with chondrosarcomas (p < 0.001). Lower: Curves for all patients when histological diagnosis was analyzed as a factor (41 patients with typical chordomas, 12 with chondroid chordoma, and 10 with chondrosarcomas). There was no significant difference among curves for patients with typical chordomas, those with chondroid chordomas, and those with chondrosarcomas. The Dunn multiple comparisons posttest showed a significant difference between patients with typical chordomas and those with chondrosarcomas (p < 0.01), as well as between patients with chondroid chordomas and those with chondrosarcomas (p < 0.05).

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    Graphs showing RFS curves for patients when extension of resection was analyzed as a factor. Upper: All patients (31 cases of radical resection, 18 cases of subtotal resection, and 14 cases of partial resection). There was a significant difference for patients who underwent radical, subtotal, and partial resection (p < 0.0001, df = 2, Kruskal—Wallis nonparametric ANOVA test). The Dunn multiple comparisons posttest showed no significant difference between radical and subtotal resections (p > 0.05), and a significant difference between radical and partial resection (p < 0.001), as well as between subtotal and partial resections (p < 0.001). Lower: Patients with chordomas (41 with typical chordomas and 12 with chondroid chordomas). There was a significant difference among patients who underwent radical, subtotal and partial resection (p < 0.0005, df = 2, Kruskal—Wallis nonparametric ANOVA test). The Dunn multiple comparisons posttest showed no significant difference between radical and subtotal resections (p > 0.05), but a significant difference between radical and partial resections (p < 0.001), as well as between subtotal and partial resections (p < 0.001).

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    Graph showing RFS curves for patients with chordomas who were treated or not treated with radiation therapy (25 patients who underwent proton-beam therapy, eight who underwent conventional radiation therapy, 17 who did not undergo radiation therapy, and three who underwent previous radiation therapy). No significant difference was found among the curves (p = 0.0537, four groups; Kruskal—Wallis nonparametric ANOVA test). The Dunn multiple comparisons posttest showed a significant difference between curves for patients who underwent proton-beam therapy and those who underwent conventional radiation therapy (p < 0.05), but no significant differences for all other combinations of groups (p > 0.05 for all combinations).

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    Graph showing RFS curves for patients with chordomas according to results of karyotype analysis (five patients with normal karyotypes and nine with abnormal karyotypes). There was no significant difference between the curves for either group (p = 0.1955, df = 1; log rank test).

References

  • 1.

    Al-Mefty OBorba LA: Skull base chordomas: a management challenge. J Neurosurg 86:1821891997Al-Mefty O Borba LA: Skull base chordomas: a management challenge. J Neurosurg 86:182–189 1997

    • Search Google Scholar
    • Export Citation
  • 2.

    Amendola BEAmendola MAOliver Eet al: Chordoma: role of radiation therapy. Radiology 158:8398431986Amendola BE Amendola MA Oliver E et al: Chordoma: role of radiation therapy. Radiology 158:839–843 1986

    • Search Google Scholar
    • Export Citation
  • 3.

    Austin JPUrie MMCardenosa Get al: Probable causes of recurrence in patients with chordoma and chondrosarcoma of the base of skull and cervical spine. Int J Radiat Oncol Biol Phys 25:4394441993Austin JP Urie MM Cardenosa G et al: Probable causes of recurrence in patients with chordoma and chondrosarcoma of the base of skull and cervical spine. Int J Radiat Oncol Biol Phys 25:439–444 1993

    • Search Google Scholar
    • Export Citation
  • 4.

    Austin-Seymour MMunzenrider JGoitein Met al: Fractionated proton radiation therapy of chordoma and low-grade chondrosarcoma of the base of the skull. J Neurosurg 70:13171989Austin-Seymour M Munzenrider J Goitein M et al: Fractionated proton radiation therapy of chordoma and low-grade chondrosarcoma of the base of the skull. J Neurosurg 70:13–17 1989

    • Search Google Scholar
    • Export Citation
  • 5.

    Benk VLiebsch NJMunzenrider JEet al: Base of skull and cervical spine chordomas in children treated by high-dose irradiation. Int J Radiat Oncol Biol Phys 31:5775811995Benk V Liebsch NJ Munzenrider JE et al: Base of skull and cervical spine chordomas in children treated by high-dose irradiation. Int J Radiat Oncol Biol Phys 31:577–581 1995

    • Search Google Scholar
    • Export Citation
  • 6.

    Berson AMCastro JRPetti Pet al: 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 15:5595651988Berson AM Castro JR Petti P et al: 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 1988

    • Search Google Scholar
    • Export Citation
  • 7.

    Borba LAAl-Mefty OMrak REet al: Cranial chordomas in children and adolescents. J Neurosurg 84:5845911996Borba LA Al-Mefty O Mrak RE et al: Cranial chordomas in children and adolescents. J Neurosurg 84:584–591 1996

    • Search Google Scholar
    • Export Citation
  • 8.

    Bourgouin PMTampieri DRobitaille Yet al: Low-grade myxoid chondrosarcoma of the base of the skull: CT, MR, and histopathology. J Comput Assist Tomogr 16:2682731992Bourgouin PM Tampieri D Robitaille Y et al: Low-grade myxoid chondrosarcoma of the base of the skull: CT MR and histopathology. J Comput Assist Tomogr 16:268–273 1992

    • Search Google Scholar
    • Export Citation
  • 9.

    Buonamici LRoncaroli FFioravanti Aet al: Cytogenetic investigation of chordomas of the skull. Cancer Genet Cytogenet 112:49521999Buonamici L Roncaroli F Fioravanti A et al: Cytogenetic investigation of chordomas of the skull. Cancer Genet Cytogenet 112:49–52 1999

    • Search Google Scholar
    • Export Citation
  • 10.

    Castro JRLinstadt DEBahary JPet al: Experience in charged particle irradiation of tumors of the skull base: 1977–1992. Int J Radiat Oncol Biol Phys 29:6476551994Castro JR Linstadt DE Bahary JP et al: Experience in charged particle irradiation of tumors of the skull base: 1977–1992. Int J Radiat Oncol Biol Phys 29:647–655 1994

    • Search Google Scholar
    • Export Citation
  • 11.

    Chetiyawardana AD: Chordoma: results of treatment. Clin Radiol 35:1591611984Chetiyawardana AD: Chordoma: results of treatment. Clin Radiol 35:159–161 1984

    • Search Google Scholar
    • Export Citation
  • 12.

    Chetty RLevin CVKalan MR: Chordoma: a 20-year clinicopathologic review of the experience at Groote Schuur Hospital, Cape Town. J Surg Oncol 46:2612641991Chetty R Levin CV Kalan MR: Chordoma: a 20-year clinicopathologic review of the experience at Groote Schuur Hospital Cape Town. J Surg Oncol 46:261–264 1991

    • Search Google Scholar
    • Export Citation
  • 13.

    Coffin CMSwanson PEWick MRet al: Chordoma in childhood and adolescence. A clinicopathologic analysis of 12 cases. Arch Pathol Lab Med 117:9279331993Coffin CM Swanson PE Wick MR et al: Chordoma in childhood and adolescence. A clinicopathologic analysis of 12 cases. Arch Pathol Lab Med 117:927–933 1993

    • Search Google Scholar
    • Export Citation
  • 14.

    Cummings BJHodson DIBush RS: Chordoma: the results of megavoltage radiation therapy. Int J Radiat Oncol Biol Phys 9:6336421983Cummings BJ Hodson DI Bush RS: Chordoma: the results of megavoltage radiation therapy. Int J Radiat Oncol Biol Phys 9:633–642 1983

    • Search Google Scholar
    • Export Citation
  • 15.

    Dahlin DCMacCarty CS: Chordoma: a study of fifty-nine cases. Cancer 5:117011781952Dahlin DC MacCarty CS: Chordoma: a study of fifty-nine cases. Cancer 5:1170–1178 1952

    • Search Google Scholar
    • Export Citation
  • 16.

    Dalpra LMalgara RMiozzo Met al: First cytogenetic study of a recurrent familial chordoma of the clivus. Int J Cancer 81:24301999Dalpra L Malgara R Miozzo M et al: First cytogenetic study of a recurrent familial chordoma of the clivus. Int J Cancer 81:24–30 1999

    • Search Google Scholar
    • Export Citation
  • 17.

    Debus JSchulz-Ertner DSchad Let al: Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int J Radiat Oncol Biol Phys 47:5915962000Debus J Schulz-Ertner D Schad L et al: Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int J Radiat Oncol Biol Phys 47:591–596 2000

    • Search Google Scholar
    • Export Citation
  • 18.

    Eisenberg MBWoloschak MSen Cet al: Loss of heterozygosity in the retinoblastoma tumor suppressor gene in skull base chordomas and chondrosarcomas. Surg Neurol 47:1561611997Eisenberg MB Woloschak M Sen C et al: Loss of heterozygosity in the retinoblastoma tumor suppressor gene in skull base chordomas and chondrosarcomas. Surg Neurol 47:156–161 1997

    • Search Google Scholar
    • Export Citation
  • 19.

    Eriksson BGunterberg BKindblom LG: Chordoma. A clinicopathologic and prognostic study of a Swedish national series. Acta Orthop Scand 52:49581981Eriksson B Gunterberg B Kindblom LG: Chordoma. A clinicopathologic and prognostic study of a Swedish national series. Acta Orthop Scand 52:49–58 1981

    • Search Google Scholar
    • Export Citation
  • 20.

    Evans HLAyala AGRomsdahl MM: Prognostic factors in chondrosarcoma of bone. A clinicopathologic analysis with emphasis on histologic grading. Cancer 40:8188311977Evans HL Ayala AG Romsdahl MM: Prognostic factors in chondrosarcoma of bone. A clinicopathologic analysis with emphasis on histologic grading. Cancer 40:818–831 1977

    • Search Google Scholar
    • Export Citation
  • 21.

    Forsyth PACascino TLShaw EGet al: Intracranial chordomas: a clinicopathological and prognostic study of 51 cases. J Neurosurg 78:7417471993Forsyth PA Cascino TL Shaw EG et al: Intracranial chordomas: a clinicopathological and prognostic study of 51 cases. J Neurosurg 78:741–747 1993

    • Search Google Scholar
    • Export Citation
  • 22.

    Gay ESekhar LNRubinstein Eet al: Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36:8878961995Gay E Sekhar LN Rubinstein E et al: Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36:887–896 1995

    • Search Google Scholar
    • Export Citation
  • 23.

    Heffelfinger MJDahlin DCMacCarty CSet al: Chordomas and cartilaginous tumors at the skull base. Cancer 32:4104201973Heffelfinger MJ Dahlin DC MacCarty CS et al: Chordomas and cartilaginous tumors at the skull base. Cancer 32:410–420 1973

    • Search Google Scholar
    • Export Citation
  • 24.

    Higinbotham NLPhillips RFFarr HWet al: Chordoma. Thirty-five-year study at Memorial Hospital. Cancer 20:184118501967Higinbotham NL Phillips RF Farr HW et al: Chordoma. Thirty-five-year study at Memorial Hospital. Cancer 20:1841–1850 1967

    • Search Google Scholar
    • Export Citation
  • 25.

    Hug EBLoredo LNSlater JDet al: Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 91:4324391999Hug EB Loredo LN Slater JD et al: Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 91:432–439 1999

    • Search Google Scholar
    • Export Citation
  • 26.

    Hug EBMunzenrider JE: Charged particle therapy for base of skull tumors: past accomplishments and future challenges. Int J Radiat Oncol Biol Phys 29:9119121994Hug EB Munzenrider JE: Charged particle therapy for base of skull tumors: past accomplishments and future challenges. Int J Radiat Oncol Biol Phys 29:911–912 1994

    • Search Google Scholar
    • Export Citation
  • 27.

    Kanrim RPPotanos JNPool JL: An evaluation of the diagnosis and treatment of chordoma. J Neurol Neurosurg Psychiatry 27:1571651964Kanrim RP Potanos JN Pool JL: An evaluation of the diagnosis and treatment of chordoma. J Neurol Neurosurg Psychiatry 27:157–165 1964

    • Search Google Scholar
    • Export Citation
  • 28.

    Keisch MEGarcia DMShibuya RB: Retrospective long-term follow-up analysis in 21 patients with chordomas of various sites treated at a single institution. J Neurosurg 75:3743771991Keisch ME Garcia DM Shibuya RB: Retrospective long-term follow-up analysis in 21 patients with chordomas of various sites treated at a single institution. J Neurosurg 75:374–377 1991

    • Search Google Scholar
    • Export Citation
  • 29.

    Kondziolka DLunsford LDFlickinger JC: The role of radiosurgery in the management of chordoma and chondrosarcoma of the cranial base. Neurosurgery 29:38451991Kondziolka D Lunsford LD Flickinger JC: The role of radiosurgery in the management of chordoma and chondrosarcoma of the cranial base. Neurosurgery 29:38–45 1991

    • Search Google Scholar
    • Export Citation
  • 30.

    Krayenbühl HYaşargil MG: Cranial chordomas. Prog Neurol Surg 6:3804341975Krayenbühl H Yaşargil MG: Cranial chordomas. Prog Neurol Surg 6:380–434 1975

    • Search Google Scholar
    • Export Citation
  • 31.

    Menezes AHGantz BJTraynelis VCet al: Cranial base chordomas. Clin Neurosurg 44:4915091997Menezes AH Gantz BJ Traynelis VC et al: Cranial base chordomas. Clin Neurosurg 44:491–509 1997

    • Search Google Scholar
    • Export Citation
  • 32.

    Menezes AHTraynelis VC: Tumors of the craniovertebral junction in Youmans JR (ed): Neurological Surgeryed 4. Philadelphia: WB Saunders1996 Vol 4 pp 30413072Menezes AH Traynelis VC: Tumors of the craniovertebral junction in Youmans JR (ed): Neurological Surgery ed 4. Philadelphia: WB Saunders 1996 Vol 4 pp 3041–3072

    • Search Google Scholar
    • Export Citation
  • 33.

    Mitchell AScheithauer BWUnni KKet al: Chordoma and chondroid neoplasms of the spheno-occiput. An immunohistochemical study of 41 cases with prognostic and nosologic implications. Cancer 72:294329491993Mitchell A Scheithauer BW Unni KK et al: Chordoma and chondroid neoplasms of the spheno-occiput. An immunohistochemical study of 41 cases with prognostic and nosologic implications. Cancer 72:2943–2949 1993

    • Search Google Scholar
    • Export Citation
  • 34.

    Muthukumar NKondziolka DLunsford LDet al: Stereotactic radiosurgery for chordoma and chondrosarcoma: further experiences. Int J Radiat Oncol Biol Phys 41:3873921998Muthukumar N Kondziolka D Lunsford LD et al: Stereotactic radiosurgery for chordoma and chondrosarcoma: further experiences. Int J Radiat Oncol Biol Phys 41:387–392 1998

    • Search Google Scholar
    • Export Citation
  • 35.

    Paavolainen PTeppo L: Chordoma in Finland. Acta Orthop Scand 47:46511976Paavolainen P Teppo L: Chordoma in Finland. Acta Orthop Scand 47:46–51 1976

    • Search Google Scholar
    • Export Citation
  • 36.

    Raffel CWright DCGutin PHet al: Cranial chordoma: clinical presentation and results of operative and radiation therapy in twenty-six patients. Neurosurgery 17:7037101985Raffel C Wright DC Gutin PH et al: Cranial chordoma: clinical presentation and results of operative and radiation therapy in twenty-six patients. Neurosurgery 17:703–710 1985

    • Search Google Scholar
    • Export Citation
  • 37.

    Reddy EKMansfield CMHartman GV: Chordoma. Int J Radiat Oncol Biol Phys 7:170917111981Reddy EK Mansfield CM Hartman GV: Chordoma. Int J Radiat Oncol Biol Phys 7:1709–1711 1981

    • Search Google Scholar
    • Export Citation
  • 38.

    Rich TASchiller ASuit HDet al: Clinical and pathologic review of 48 cases of chordoma. Cancer 56:1821871985Rich TA Schiller A Suit HD et al: Clinical and pathologic review of 48 cases of chordoma. Cancer 56:182–187 1985

    • Search Google Scholar
    • Export Citation
  • 39.

    Rosenberg AENielsen GPKeel SBet al: Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma. Am J Surg Pathol 23:137013781999Rosenberg AE Nielsen GP Keel SB et al: 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 1999

    • Search Google Scholar
    • Export Citation
  • 40.

    Sekhar LNGay EWrigth DC: Chordomas and chondrosarcomas of the cranial base in Wilkins RHRengachary SS (eds): Neurosurgeryed 2. New York: McGraw-Hill1996 Vol 2 pp 15291543Sekhar LN Gay E Wrigth DC: Chordomas and chondrosarcomas of the cranial base in Wilkins RH Rengachary SS (eds): Neurosurgery ed 2. New York: McGraw-Hill 1996 Vol 2 pp 1529–1543

    • Search Google Scholar
    • Export Citation
  • 41.

    Sekhar LNJanecka IPJones NF: Subtemporal-infratemporal and basal subfrontal approach to extensive cranial base tumors. Acta Neurochir 92:83921988Sekhar LN Janecka IP Jones NF: Subtemporal-infratemporal and basal subfrontal approach to extensive cranial base tumors. Acta Neurochir 92:83–92 1988

    • Search Google Scholar
    • Export Citation
  • 42.

    Sen CNSekhar LNSchramm VLet al: Chordoma and chondrosarcoma of the cranial base: an 8-year experience. Neurosurgery 25:9319411989Sen CN Sekhar LN Schramm VL et al: Chordoma and chondrosarcoma of the cranial base: an 8-year experience. Neurosurgery 25:931–941 1989

    • Search Google Scholar
    • Export Citation
  • 43.

    Suit HDGoitein MMunzenrider Jet al: Definitive radiation therapy for chordoma and chondrosarcoma of base of skull and cervical spine. J Neurosurg 56:3773851982Suit HD Goitein M Munzenrider J et al: Definitive radiation therapy for chordoma and chondrosarcoma of base of skull and cervical spine. J Neurosurg 56:377–385 1982

    • Search Google Scholar
    • Export Citation
  • 44.

    Tewfik HHMcGinnis WLNordstrom DGet al: Chordoma: evaluation of clinical behavior and treatment modalities. Int J Radiat Oncol Biol Phys 2:9599621977Tewfik HH McGinnis WL Nordstrom DG et al: Chordoma: evaluation of clinical behavior and treatment modalities. Int J Radiat Oncol Biol Phys 2:959–962 1977

    • Search Google Scholar
    • Export Citation
  • 45.

    Watkins LKhudados ESKaleoglu Met al: Skull base chordoma: a review of 38 patients, 1958–88. Br J Neurosurg 7:2412481993Watkins L Khudados ES Kaleoglu M et al: Skull base chordoma: a review of 38 patients 1958–88. Br J Neurosurg 7:241–248 1993

    • Search Google Scholar
    • Export Citation

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