J Neurol Surg B Skull Base 2015; 76 - P014
DOI: 10.1055/s-0035-1546642

Atypical Pathologic Features in Clival Chordoma

Jeffrey T. Jacob 1, Aditya Raghunathan 1, Joshua D. Hughes 1, Jeffrey R. Janus 1, Caterina Giannini 1, Colin L. Driscoll 1, Eric J. Moore 1, Michael J. Link 1, Jamie J. Van Gompel 1
  • 1Mayo Clinic, United States

Background: Clival chordomas are rare tumors believed to originate from the remnants of the primitive notochord with a high risk of regional recurrence. Some chordomas have harbored atypical pathologic features; however, there is little data correlating specific pathologic features with disease-free survival.

Methods: Patients who underwent resection or biopsy with pathologically proven clival chordoma from 1985 to 2012 were reviewed. Patients with > 1 year of radiographic follow-up and available pathologic slides for review were included in the study. Correlation of pathological features was made with disease-free survival.

Results: A total of 20 patients met study criteria and were included. Overall, mean follow-up was 109.5 months. Overall, 17 patients (85%) underwent postoperative radiotherapy with proton beam (n = 6), gamma knife (n = 6), or external beam (n = 5). Overall, 10 patients (50%) had disease progression following initial treatment at a mean time to recurrence of 96.7 months. Of the 20 patients, 7 (35%) died from disease progression during the follow-up period. Mean age of those with disease progression was 49 versus 33.2 years in those without disease progression (OR, 1.08; 95% CI, 1.01–1.17; p = 0.02). Overall, 11 patients demonstrated conventional morphology, 7 patients had chondroid morphology, and 2 patients had a dedifferentiated/cellular morphology. Pathologic features including cellularity, nuclear grade, nuclear anaplasia, mitoses, degree of necrosis, vascular invasion, cellular morphology, and stromal features were analyzed. There was no statistically significant correlation between any of the pathologic features studied with disease-free survival.

Conclusion: Clival chordoma carries a significant rate of recurrence despite advancements in surgical treatment and radiation therapy. Optimal management should include maximal surgical resection followed by postoperative radiotherapy. Young age is associated with longer survival. Further studies are needed to show a correlation between pathologic variability and disease-free survival.