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Long-term control of clival chordoma with initial aggressive surgical resection and gamma knife radiosurgery for recurrence

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Abstract

Purpose

Chordomas are locally destructive tumors with high rates of recurrence, and therapeutic strategies remain controversial. This study analyzed long-term outcomes for clival chordomas after initial aggressive surgical resection and gamma knife radiosurgery for recurrence and investigated clinical factors predicting recurrence.

Methods

Clinical records were reviewed for 19 consecutive patients (11 men, 8 women; mean age, 43.1 years) with clival chordoma who underwent initial surgical resection using skull base approaches (mean follow-up after surgical resection, 87.2 months). All tumors were aggressively removed, along with the surrounding bone. Four patients were treated with radiotherapy after surgical resection. Recurrent lesions were treated with gamma knife radiosurgery or reoperation. Factors predicting tumor recurrence were analyzed, including age, tumor extension, extent of resection and MIB-1 labeling index. Patient status was evaluated using the Karnofsky performance scale (KPS).

Results

Tumor resection was total, subtotal and partial in 14, 4 and 1 patients, respectively. Tumors recurred in 11 patients. Overall, 2- and 5-year progression-free survival rates were 77.9% and 47.9%, respectively. The MIB-1 labeling index was independently associated with recurrence. The optimum cutoff point for the MIB-1 labeling index was 3.44%. All recurrent tumors were totally resected or controlled by gamma knife (mean follow-up after recurrence, 71.2 months). All patients survived and were active (mean KPS at final follow-up, 89.5%).

Conclusion

Long-term control of clival chordomas was achieved. Recurrent tumors were controlled with gamma knife radiosurgery, since lesions were localized and small after initial aggressive resection. The MIB-1 labeling index can provide important information for predicting tumor recurrence.

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References

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

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  3. Ammirati M, Bernardo A (1999) Management of skull base chordoma. Crit Rev Neurosurg 9:63–69

    Article  PubMed  Google Scholar 

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

    Article  CAS  Google Scholar 

  5. Austin-Seymour M, Munzenrider J, Linggood R, Goitein M, Verhey L, Urie M, Gentry R, Birnbaum S, Ruotolo D, Crowell C et al (1990) Fractionated proton radiation therapy of cranial and intracranial tumors. Am J Clin Oncol 13:327–330

    Article  CAS  PubMed  Google Scholar 

  6. Austin JP, Urie MM, Cardenosa G, Munzenrider JE (1993) 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

    CAS  PubMed  Google Scholar 

  7. 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

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  9. 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

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  11. Colli BO, Al-Mefty O (2001) Chordomas of the skull base: follow-up review and prognostic factors. Neurosurg Focus 10:E1

    Article  CAS  PubMed  Google Scholar 

  12. 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

    Article  CAS  PubMed  Google Scholar 

  13. 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

    Article  CAS  PubMed  Google Scholar 

  14. 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

    CAS  PubMed  Google Scholar 

  15. 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 102(Suppl):165–170

    Article  PubMed  Google Scholar 

  16. 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

    Article  CAS  PubMed  Google Scholar 

  17. Fukuta K, Saito K, Takahashi M, Torii S (1997) Surgical approach to midline skull base tumors with olfactory preservation. Plast Reconstr Surg 100:318–325

    Article  CAS  PubMed  Google Scholar 

  18. 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 896–887

    Article  CAS  PubMed  Google Scholar 

  19. 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

    CAS  PubMed  Google Scholar 

  20. 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

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  22. Hasegawa T, Ishii D, Kida Y, Yoshimoto M, Koike J, Iizuka H (2007) Gamma knife surgery for skull base chordomas and chondrosarcomas. J Neurosurg 107:752–757

    Article  PubMed  Google Scholar 

  23. Holton JL, Steel T, Luxsuwong M, Crockard HA, Revesz T (2000) Skull base chordomas: correlation of tumour doubling time with age, mitosis and Ki67 proliferation index. Neuropathol Appl Neurobiol 26:497–503

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  25. 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

    Article  CAS  PubMed  Google Scholar 

  26. 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

    Article  PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  28. Martin JJ, Niranjan A, Kondziolka D, Flickinger JC, Lozanne KA, Lunsford LD (2007) Radiosurgery for chordomas and chondrosarcomas of the skull base. J Neurosurg 107:758–764

    Article  PubMed  Google Scholar 

  29. Matsuno A, Sasaki T, Nagashima T, Matsuura R, Tanaka H, Hirakawa M, Murakami M, Kirino T (1997) Immunohistochemical examination of proliferative potentials and the expression of cell cycle-related proteins of intracranial chordomas. Hum Pathol 28:714–719

    Article  CAS  PubMed  Google Scholar 

  30. 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

    Article  CAS  PubMed  Google Scholar 

  31. Miller RC, Foote RL, Coffey RJ, Gorman DA, Earle JD, Schomberg PJ, Kline RW (1997) The role of stereotactic radiosurgery in the treatment of malignant skull base tumors. Int J Radiat Oncol Biol Phys 39:977–981

    CAS  PubMed  Google Scholar 

  32. Monje ML, Palmer T (2003) Radiation injury and neurogenesis. Curr Opin Neurol 16:129–134

    Article  PubMed  Google Scholar 

  33. 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

    CAS  PubMed  Google Scholar 

  34. Nishigaya K, Kaneko M, Ohashi Y, Nukui H (1998) Intradural retroclival chordoma without bone involvement: no tumor regrowth 5 years after operation. Case report. J Neurosurg 88:764–768

    Article  CAS  PubMed  Google Scholar 

  35. O'Connell JX, Renard LG, Liebsch NJ, Efird JT, Munzenrider JE, Rosenberg AE (1994) Base of skull chordoma. A correlative study of histologic and clinical features of 62 cases. Cancer 74:2261–2267

    Article  PubMed  Google Scholar 

  36. Saito K, Takahashi M, Fukuta K, Tachibana E, Yoshida J (1999) Recovery of olfactory function after an anterior craniofacial approach. Skull Base Surg 9:201–206

    Article  CAS  PubMed  Google Scholar 

  37. 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

    CAS  PubMed  Google Scholar 

  38. Schamschula RG, Soo MY (1993) Clival chordomas. Australas Radiol 37:259–264

    Article  CAS  PubMed  Google Scholar 

  39. 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. J Neurosurg 76:198–206

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  41. Swearingen B, Joseph M, Cheney M, Ojemann RG (1995) A modified transfacial approach to the clivus. Neurosurgery 36:101–104 discussion 104–105

    Article  CAS  PubMed  Google Scholar 

  42. Vishteh AG, Crawford NR, Melton MS, Spetzler RF, Sonntag VK, Dickman CA (1999) Stability of the craniovertebral junction after unilateral occipital condyle resection: a biomechanical study. J Neurosurg 90:91–98

    CAS  PubMed  Google Scholar 

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Correspondence to Kiyoshi Saito.

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Comments

The authors describe their experience managing the 19 patients with chordomas using a combination of microsurgery and gamma knife radiosurgery (and in 4 cases radiotherapy). Radiosurgery was used for residual tumors as well as recurrences, and larger recurrent tumors were also considered for reoperation. Impressively, using this combination management, the tumors were either totally resected (that is at the time of review there was no visible recurrence yet) or controlled.

Management of chordomas is notoriously difficult, and so it is unclear which modality one should use and in which order. Admittedly, most centers that have access to high quality microsurgery and radiosurgery would choose to use a very similar, very sensible approach. Nevertheless, this publication is very useful, and it would help the general readership of this journal.

The reason for the relative lack of similar articles in the literature is that focal radiosurgical treatment was often dismissed as an option because of the size of the presenting or representing tumor. Fractionated techniques, interstitial radiotherapy, etc., have all been described. With the advances in safer microsurgery, a surgical reduction in size is extremely helpful before radiosurgery, and some turn out to be completely successful. This paper is tribute to the multidisciplinary combination.

Andras Kemeny

Sheffield, UK

The authors retrospectively report their management strategy in the treatment of 19 patients with clival chordoma over a 16-year period, which includes initial aggressive surgical removal, close follow-up and radiosurgical treatment for recurrence, sometimes repeated or combined with reoperation. The versatility of tailored skull base approaches described, without mortality and with a low rate of acceptable morbidity given their complexity, is to be commended.

An interesting aspect in the current series is the apparent lack of mid- and long-term morbidity from repeated radiosurgical treatments, some of them on top of conventional or stereotactic fractionated radiation.

As previously reported by other groups, RS seems to be a good option for complementary therapy in patients with a low residual (or recurrent) tumor volume achieved by aggressive surgery, given its availability and cost when compared with proton-beam therapy.

Manuel Cunha e Sá, MD

Bruno Santiago, MD

Almada, Portugal

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Ito, E., Saito, K., Okada, T. et al. Long-term control of clival chordoma with initial aggressive surgical resection and gamma knife radiosurgery for recurrence. Acta Neurochir 152, 57–67 (2010). https://doi.org/10.1007/s00701-009-0535-7

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