Abstract
Introduction
Primary chordomas and chondrosarcomas of the skull base are difficult tumours to treat successfully. Despite advances in surgical techniques, a gross total resection is often impossible to achieve. In addition, some patients may be deemed unsuitable or not wish to undergo extensive surgery for these conditions. This study examines the role of Gamma Knife Stereotactic Radiosurgery (GKRS) in the treatment of these difficult cases.
Methods
All patients harbouring either a chordoma or chondrosarcoma treated at the National Centre for Stereotactic Radiosurgery, Royal Hallamshire Hospital, Sheffield, UK, between 1985 and 2018, were reviewed with regard to their clinical presentations, pre- and post-treatment imaging, GKRS prescriptions and outcomes.
Results
In total, 24 patients with a mean tumour volume of 13 cm3 in the chordoma group (n=15) and 12 cm3 in the chondrosarcoma group (n=9) underwent GKRS. The 5- and 10-year overall survival rates for the chordoma group were 67% and 53% respectively, while for the chondrosarcoma group, they were 78% at both time points. The tumour control rates at 5 and 10 years in the chordoma group were 67% and 49% and for the chondrosarcoma group 78% at both time points. Patients with tumour volumes of less than 7 cm3 before GKRS treatment demonstrated a statistically significant longer overall survival rate (p=0.03).
Conclusions
GKRS offers a comparable option to proton beam therapy for the treatment of these tumours. Early intervention for tumour volumes of less than 7 cm3 gives the best long-term survival rates.
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References
Almefty K, Pravdenkova S, Colli BO, Al-Mefty O, Gokden M (2007) Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer 110(11):2467–2477
Amichetti M, Amelio D, Cianchetti M, Giacomelli I, Scartoni D (2017) The treatment of chordoma and chondrosarcoma of the skull base with particular attention to radiotherapy. Clin Oncol 2:7
Amichetti M, Amelio D, Minniti G (2012) Radiosurgery with photons or protons for benign and malignant tumours of the skull base: a review. Radiat Oncol 7(1):210
Amichetti M, Cianchetti M, Amelio D, Enrici RM, Minniti G (2009) Proton therapy in chordoma of the base of the skull: a systematic review. Neurosurg Rev 32(4):403–416
Boari N, Gagliardi F, Cavalli A, Gemma M, Ferrari L, Riva P, Mortini P (2016) Skull base chordomas: clinical outcome in a consecutive series of 45 patients with long-term follow-up and evaluation of clinical and biological prognostic factors. J Neurosurg 125(2):450–460
Brada M, Pijls-Johannesma M, De Ruysscher D (2007) Proton therapy in clinical practice: current clinical evidence. J Clin Oncol 25(8):965–970
Campbell VT, Nadesan P, Ali SA et al (2014) Hedgehog pathway inhibition in chondrosarcoma using the smoothened inhibitor IPI-926 directly inhibits sarcoma cell growth. Mol Cancer Ther 13(5):1259–1269
Campbell RG, Prevedello DM, Filho LD, Otto BA, Carrau RL (2015) Contemporary management of clival chordomas. Curr Opin Otolaryngol Head Neck Surg 23(2):153–161
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. Radiother Oncol 41(1):67–72
Cho YH, Kim JH, Khang SK, Lee J-K, Kim CJ (2007) Chordomas and chondrosarcomas of the skull base: comparative analysis of clinical results in 30 patients. Neurosurg Rev 31(1):35–43
Chow WA (2018) Chondrosarcoma: biology, genetics, and epigenetics. F1000Research 7:1826
Choy W, Terterov S, Ung N et al (2015) Adjuvant stereotactic radiosurgery and radiation therapy for the treatment of intracranial chordomas. J Neurol Surg Part B Skull Base 77(01):038–046
Colli BO, Al-Mefty O (2001) Chordomas of the skull base: follow-up review and prognostic factors. Neurosurg Focus 10(3):1–11
Connors SW, Aoun SG, Shi C, Peinado-Reyes V, Hall K, Bagley CA (2020) Recent advances in understanding and managing chordomas: an update. F1000Research 9:713
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(2):175–183
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 47(3):591–596
Di Maio S, Temkin N, Ramanathan D, Sekhar LN (2011) Current comprehensive management of cranial base chordomas: 10-year meta-analysis of observational studies. J Neurosurg 115(6):1094–1105
Förander P, Bartek J, Fagerlund M, Benmaklouf H, Dodoo E, Shamikh A, Stjärne P, Mathiesen T (2017) Multidisciplinary management of clival chordomas; long-term clinical outcome in a single-institution consecutive series. Acta Neurochir (Wien) 159(10):1857–1868
George B, Bresson D, Herman P, Froelich S (2015) Chordomas. Neurosurg Clin N Am 26(3):437–452
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(4):752–757
Hug EB, Devries A, Rosenberg AE (1999) Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 91:8
Ito E, Saito K, Okada T, Nagatani T, Nagasaka T (2010) Long-term control of clival chordoma with initial aggressive surgical resection and gamma knife radiosurgery for recurrence. Acta Neurochir (Wien) 152(1):57–67
Jägersberg M, El Rahal A, Dammann P, Merkler D, Weber DC, Schaller K (2017) Clival chordoma: a single-centre outcome analysis. Acta Neurochir (Wien) 159(10):1815–1823
Jahangiri A, Chin AT, Wagner JR et al (2015) Factors predicting recurrence after resection of clival chordoma using variable surgical approaches and radiation modalities. Neurosurgery 76(2):179–186
Jiang B, Veeravagu A, Lee M, Harsh GR, Lieberson RE, Bhatti I, Soltys SG, Gibbs IC, Adler JR, Chang SD (2012) Management of intracranial and extracranial chordomas with CyberKnife stereotactic radiosurgery. J Clin Neurosci 19(8):1101–1106
Kano H, Iqbal FO, Sheehan J et al (2011) Stereotactic radiosurgery for chordoma. Neurosurgery 68(2):379–389
Kano H, Lunsford LD (2013) Stereotactic radiosurgery of intracranial chordomas, chondrosarcomas, and glomus tumors. Neurosurg Clin N Am 24(4):553–560
Kim JH, Chang JW, Chang WS (2014) Gamma Knife surgery for intracranial chordoma and chondrosarcoma: radiosurgical perspectives and treatment outcomes. J Neurosurg 121:10
Koga T, Shin M, Saito N (2010) Treatment with high marginal dose is mandatory to achieve long-term control of skull base chordomas and chondrosarcomas by means of stereotactic radiosurgery. J Neurooncol 98(2):233–238
Kondziolka D, Lunsford LD, McLaughlin MR, Flickinger JC (1998) Long-term outcomes after radiosurgery for acoustic neuromas. N Engl J Med 339(20):1426–1433
Krishnan S, Foote RL, Brown PD, Pollock BE, Link MJ, Garces YI (2005) Radiosurgery for cranial base chordomas and chondrosarcomas. Neurosurgery 56(4):777–784
Labidi M, Watanabe K, Bouazza S, Bresson D, Bernat A-L, George B, Froelich S (2016) Clivus chordomas: a systematic review and meta-analysis of contemporary surgical management. J Neurosurg Sci 60(4):9
Lanzino G, Dumont AS, Lopes MBS, Laws ER (2001) Skull base chordomas: overview of disease, management options, and outcome. Neurosurg Focus 10(3):1–9
Leah P, Dower A, Vescovi C, Mulcahy M, Al Khawaja D (2018) Clinical experience of intracranial chordoma – a systematic review and meta-analysis of the literature. J Clin Neurosci 53:6–12
Macdonald DR, Cascino TL, Schold SCJ, Cairncross JG (1990) Response criteria for phase II studies of supratentorial malignant glioma. J Clin Oncol Off J Am Soc Clin Oncol 8(7):1277–1280
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(4):758–764
Nguyen Q-N, Chang EL (2008) Emerging role of proton beam radiation therapy for chordoma and chondrosarcoma of the skull base. Curr Oncol Rep 10(4):338–343
Noël G, Habrand J-L, Jauffret E et al (2003) Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine: prognostic factors and patterns of failure. Strahlenther Onkol 179(4):241–248
Rowe JG (2003) Gamma knife stereotactic radiosurgery for unilateral acoustic neuromas. J Neurol Neurosurg Psychiatry 74(11):1536–1542
Saberi H, Meybodi AT, Rezai AS (2006) Levine-Sekhar grading system for prediction of the extent of resection of cranial base meningiomas revisited: study of 124 cases. Neurosurg Rev 29(2):138–144
Sanusi O, Arnaout O, Rahme RJ, Horbinski C, Chandler JP (2018) Surgical resection and adjuvant radiation therapy in the treatment of skull base chordomas. World Neurosurg 115:e13–e21
Schulz-Ertner D, Karger CP, Feuerhake A, Nikoghosyan A, Combs SE, Jäkel O, Edler L, Scholz M, Debus J (2007) Effectiveness of carbon ion radiotherapy in the treatment of skull-base chordomas. Int J Radiat Oncol 68(2):449–457
Sen C, Triana AI, Berglind N, Godbold J, Shrivastava RK (2010) Clival chordomas: clinical management, results, and complications in 71 patients. J Neurosurg 113(5):1059–1071
Siu I-M, Ruzevick J, Zhao Q, Connis N, Jiao Y, Bettegowda C, Xia X, Burger PC, Hann CL, Gallia GL (2013) Erlotinib inhibits growth of a patient-derived chordoma xenograft. PLoS ONE 8(11):e78895
Stacchiotti S, Sommer J (2015) Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol 16(2):e71–e83
Tamura T, Sato T, Kishida Y, Ichikawa M, Oda K, Ito E, Watanabe T, Sakuma J, Saito K (2015) Outcome of clival chordomas after skull base surgeries with mean follow-up of 10 years. FUKUSHIMA J Med Sci 61(2):131–140
Tzortzidis F, Elahi F, Wright D, Natarajan SK, Sekhar LN (2006) Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas. Neurosurgery 59(2):230–237
Van Gompel JJ, Janus JR (2015) Chordoma and chondrosarcoma. Otolaryngol Clin North Am 48(3):501–514
Volpe NJ, Liebsch NJ, Munzenrider JE, Lesseil S (1993) Neuro-ophthalmologic findings in chordoma and chondrosarcoma of the skull base. Am J Ophthalmol 115(1):97–104
Walcott BP, Nahed BV, Mohyeldin A, Coumans J-V, Kahle KT, Ferreira MJ (2012) Chordoma: current concepts, management, and future directions. Lancet Oncol 13(2):e69–e76
Wang L, Tian K, Wang K, Ma J, Ru X, Du J, Jia G, Zhang L, Wu Z, Zhang J (2016) Factors for tumor progression in patients with skull base chordoma. Cancer Med 5(9):2368–2377
Welzel T, Meyerhof E, Uhl M, Huang K, von Deimling A, Herfarth K, Debus J (2018) Diagnostic accuracy of DW MR imaging in the differentiation of chordomas and chondrosarcomas of the skull base: a 3.0-T MRI study of 105 cases. Eur J Radiol 105:119–124
Yan X, Li Z, Li H, Liu P, Zhao Z, Cheng S, Wang Z, Zhang Q (2019) Inhibition of glycogen synthase kinase 3 beta suppresses the growth and survival of skull base chordoma cells by downregulating brachyury expression. OncoTargets Ther Volume 12:9783–9791
Yasuda M, Bresson D, Chibbaro S, Cornelius JF, Polivka M, Feuvret L, Takayasu M, George B (2012) Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients. Neurosurg Rev 35(2):171–183
Zhou Y, Hu B, Wu Z, Cheng H, Dai M, Zhang B (2018) Clival chordoma: long-term clinical outcome in a single center. Medicine (Baltimore) 97(36):e12207
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Cahill, J., Ibrahim, R., Mezey, G. et al. Gamma Knife Stereotactic Radiosurgery for the treatment of chordomas and chondrosarcomas. Acta Neurochir 163, 1003–1011 (2021). https://doi.org/10.1007/s00701-021-04768-5
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DOI: https://doi.org/10.1007/s00701-021-04768-5