Clival chordomas: considerations after 16 years of endoscopic endonasal surgery

Abstract

OBJECTIVE In the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach. METHODS All consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter. Postoperative adjuvant therapies were considered. RESULTS Sixty-five patients (male/female ratio 1:0.9) were included in this study. The median age was 48 years (range 9-80 years). Gross-total resection (GTR) was achieved in 47 cases (58.7%). On univariate analysis, primary procedures (p = 0.001), location in the superior or middle third of the clivus (p = 0.043), extradural location (p = 0.035), and histology of conventional chordomas (p = 0.013) were associated with a higher rate of GTR. The complication rate was 15.1%, and there were no perioperative deaths. Most complications did not result in permanent sequelae and included 2 CSF leaks (2.5%), 5 transient cranial nerve VI palsies (6.2%), and 2 internal carotid artery injuries (2.5%), which were treated with coil occlusion of the internal carotid artery without neurological deficits. Three patients (3.8%) presented with complications resulting in permanent neurological deficits due to a postoperative hematoma (1.2%) causing a hemiparesis, and 2 permanent ophthalmoplegias (2.5%). Seventeen patients (26.2%) have died of tumor progression over the course of follow-up (median 52 months, range 7-159 months). Based on Kaplan-Meier analysis, the survival rate was 77% at 5 years and 57% at 10 years. On multivariate analysis, the extent of tumor removal (p = 0.001) and the absence of previous treatments (p = 0.001) proved to be correlated with a longer survival rate. CONCLUSIONS The EEA was associated with a high rate of tumor removal and symptom control, with low morbidity and preservation of a good quality of life. These results allow for a satisfactory overall survival rate, particularly after GTR and for primary surgery. Considering these results, the authors believe that an EEA can be a helpful tool in chordoma surgery, achieving a good balance between as much tumor removal as possible and the preservation of an acceptable patient quality of life.

Peculiar pathological, radiological and clinical features of skull‐base de‐differentiated chordomas. Results from a referral centre case–series and literature review

“…In contrast with sacrococcygeal chordomas, skull‐base chordomas cannot be removed en bloc because of their invasive nature and critical anatomical location, especially proximity to vital neurovascular structures . It has been recently reported that chordoma de‐differentiation significantly reduced the rate of gross total resection, as their firm and taut‐elastic consistency made radical resection impossible to achieve by a surgical approach, as confirmed by our experience.…”

ICAR: endoscopic skull‐base surgery

“…GTR was noted in 58.7% of cases; the extent of tumor removal and having primary (rather than recurrent) lesions were both correlated with longer survival. Primary procedures, lesions located in the upper two‐thirds of the clivus, and extradural lesions were noted to have an association with a greater likelihood of GTR . In another series of 60 cranial base chordomas, the GTR rate was 66.7%, although this was heavily influenced by whether this was a primary surgery vs surgery in a previously treated patient (82.9% GTR vs 44% GTR, respectively) …”

“…What has been established is that chordomas generally grow slowly, and recurrence rates may not necessarily stabilize after 5 years. In 1 of the larger datasets, encompassing aggregate patient data from 80 endoscopic endonasal resections, Zoli et al . reported 24 patients (40%) presenting with concern for recurrence and/or residual tumor (several recurred multiple times), with a median time of recurrence at 20.5 months.…”

“…Interestingly, 22 patients experienced regrowth after a partial resection, and only 4 had recurrence as demonstrated on MRI after GTR. Ultimately, 26.2% of patients in Zoli et al . passed away from tumor progression.…”

“…In 1 of the larger detailed retrospective reviews reporting aggregate patient data, Zoli et al . explored outcomes of 65 patients who underwent a total of 80 EEAs for clival chordomas.…”