Skull base chordoma (SBC) is a rare cranial neoplasm and one of the most challenging
targets because of its deep ventral skull base locations, tenacious recurrence, and
resistance to radiation. Endoscopic transnasal surgery (ETS) is now considered as
a standard modality for SBCs, though its long-term outcomes remain to be clarified.
To examine the tumor control, functional outcomes, and survival as well as factors
associated with them, we conducted the present single-institution retrospective study.
Patients with SBC who were treated with ETS between 2009 and 2021 were included. The
rates of local control (LCR), and poor functionality (PFR: KPS of ≤ 40), and overall
survival (OS) were evaluated, and compared using the log-rank test. Factors associated
with the endpoints were examined using the Cox proportional hazard analysis.
Sixty-five patients with a mean follow-up period of 5 years were enrolled. The median
age and tumor volume were 52 years and 12.4 mL. Nineteen (29%) patients were recurrent
cases. All underwent ETS; the extent of resection (EOR) was total/near-total (≥99%)
in 36 (55%) and subtotal (90–98%)/partial (< 90%) in 29 (45%). Upfront adjuvant radiosurgery
was used in 20 (31%). Histologically 49 (75%) had conventional chordoma, and 16 (25%)
had chondroid subtype. The 1-, 5-, and 7-year LCRs were 78, 36, and 36%, respectively.
The LCRs were significantly better in primary cases (p < 0.01), those with tumor volume of ≤12 mL (p = 0.02), those in whom total/near-total removal was achieved (p < 0.01), those with Ki-67 index of ≤7% (p = 0.02). Multivariate Cox proportional hazard analysis demonstrated that recurrent
case (hazard ratio [HR]: 5.2, 95% confidence interval [CI]: 2.2–12.0) and tumor volume
of > 12 mL (HR 2.9, 95% CI 1.3–6.5) were significantly associated with a worse LCR.
The LCRs following subtotal/partial resection was significantly improved by adding
radiosurgery (45% at 4 year vs. 0% at 1 year; p < 0.01), reaching the similar level to LCR following total/near-total removal (figures).
KPS declined to ≤ 40 in 10 patients; 9 of them developed dissemination or metastatic
lesions during their courses. The 3-, 5-, and 10-year PFRs were 90%, 80%, and 80%,
respectively. The PFR was significantly better in primary cases (p < 0.01), those without intradural extension (p < 0.01), those in whom total/near-total removal was achieved (p < 0.01), those with KPS better than 80 (p = 0.02).
Five mortalities were observed; all of them developed dissemination or metastatic
lesions. The 3-, 5-, and 10-year OSs were 97, 89, and 89%, respectively. The OS was
significantly better in those without intradural extension (p = 0.04) and those in whom total/near-total removal was achieved (p = 0.04). However, no factors were significantly associated with the OS and PFR in
the multivariate analyses.
Skull base chordomas remain a challenging target, and multiple interventions are likely
to be needed. Recurrent case and large tumor are risk for a failed LCR. Dissemination
and metastatic lesions are the leading causes of functional deterioration and mortality.