The management of clival chordomas: an Italian multicentric study

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  • 1 Department of Neurosciences and Reproductive and Odontostomatological Sciences, Division of Neurosurgery, University of Napoli “Federico II”, Naples;
  • | 2 Department of Biomedical and Neuromotor Sciences, University of Bologna, IRCCS Institute of Neurological Sciences of Bologna, “Bellaria” Hospital, Bologna;
  • | 3 Division of Neurosurgery, IRCCS “Casa Sollievo della Sofferenza”, San Giovanni Rotondo;
  • | 4 Division of Neurosurgery, Università degli Studi di Brescia–Spedali Civili, Brescia;
  • | 5 Division of Neurosurgery, Università degli Studi dell’Insubria, Ospedale di Circolo e Fondazione Macchi, Varese;
  • | 6 Division of Neurosurgery, AOU Senese “Le Scotte”, Siena;
  • | 7 Division of Neurosurgery, Humanitas Research Hospital, Rozzano;
  • | 8 Neurosurgery Unit, Department of Neurosciences, Santa Maria della Misericordia University Hospital, Udine;
  • | 9 Department of Neuroscience, “Rita Levi Montalcini”, Neurosurgery Unit, University of Turin; and
  • | 10 Neurosurgery and Neurotraumatology, San Martino Policlinico Hospital, IRCCS for Oncology and Neurosciences, Genoa, Italy
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OBJECTIVE

In the last 2 decades, the endoscopic endonasal approach in the treatment of clival chordomas has evolved to be a viable strategy to achieve maximal safe resection of this tumor. Here, the authors present a multicentric national study, intending to analyze the evolution of this approach over a 20-year time frame and its contribution in the treatment of clival chordomas.

METHODS

Clival chordoma cases surgically treated between 1999 and 2018 at 10 Italian neurosurgical departments were included in this retrospective study. Clinical, radiological, and surgical findings, adjuvant therapy, and outcomes were evaluated and compared according to classification in the treatment eras from 1999 to 2008 and from 2009 to 2018.

RESULTS

One hundred eighty-two surgical procedures were reviewed, with an increase in case load since 2009. The endoscopic endonasal transclival approach (EETA) was performed in 151 of 182 cases (83.0%) and other approaches were performed in 31 cases (17%). There was an increment in the use of EETA, neuronavigation, and Doppler ultrasound after 2008. The overall postoperative complication rate was 14.3% (26 of 182 cases) consisting of 9 CSF leaks (4.9%), 7 intracranial hemorrhages (3.8%), 5 cases of meningitis (2.7%), and 5 cerebral ischemic injuries (2.7%). Gross-total resection (GTR) was achieved in 93 of 182 cases (51.1%). Extent of resection (EOR) improved in the second era of the study. Signs and/or symptoms at presentation worsened in 27 cases (14.8%), and the Katz Index worsened in 10 cases (5.5%). Previous treatment, dural involvement, EETA, and intraoperative Doppler ultrasound correlated with GTR. Patients received adjuvant proton beam radiation in 115 of 182 cases (63.2%), which was administered more in the latter era. Five-year progression-free survival (PFS) and overall survival (OS) were 62.3% and 73.5%, respectively. GTR, EETA, proton beam therapy, and the chondroid subtype correlated with a better survival rate. The mean follow-up was 62 months.

CONCLUSIONS

Through multicentric data collection, this study encompasses the largest series in the literature of clival chordomas surgically treated through an EETA. An increase in the use of this approach was found among Italian neurosurgical departments together with an improved extent of resection over time. The satisfactory rate of GTR was marked by low surgical morbidity and the preservation of patient quality of life. Surgical outcome was reinforced, in terms of PFS and OS, by the use of proton beam therapy, which was increasingly performed along the period of study.

ABBREVIATIONS

EEA = endoscopic endonasal approach; EETA = endoscopic endonasal transclival approach; GTR = gross-total resection; OS = overall survival; PFS = progression-free survival; PR = partial resection; STR = subtotal resection.
Illustrations from Marx and Schroeder (pp 318–326). Copyright Henry W. S. Schroeder. Published with permission.

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Contributor Notes

Correspondence Elena d’Avella: University of Napoli “Federico II”, Naples, Italy. elenadavella@gmail.com.

INCLUDE WHEN CITING Published online September 4, 2020; DOI: 10.3171/2020.5.JNS20925.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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