Mayo Clinic Clival Chordoma Case Series: Impact of Endoscopic Training on Clinical Care

Abstract

The management of clival chordoma in our group shifted around 2013 to mostly endoscopic, and proton beam was introduced for our multidisciplinary team. Consecutive patients who had surgical resection from 1987 to 2021 were reviewed. A total of 58 patients (39 patients after 2013) were analyzed. The mean tumor size was 3.7 cm, and the most common location was the upper clivus (43%). Compared to before 2013, after 2013, the endoscopic endonasal approach was more common (90%, p < 0.001), and more gross or near total resections (64%, p = 0.002) were attained. Ten cases (17%) were revision surgeries referred from elsewhere, and three cases (5%) underwent additional surgery elsewhere before adjuvant radiation. The postoperative cerebrospinal fluid leak occurred in 7%. Post-operative new cranial nerve deficits occurred in 32% before 2013, compared to 2.6% after 2013 (p = 0.004). For cases before 2013, 10 patients (53%) recurred during the median follow-up of 144 months (mean, 142 months), whereas for cases after 2013, seven patients (18%) recurred with a median follow-up of 35 months (mean, 42 months). 5-year progression-free survival was 58%, and 5-year overall survival was 87%. A specialized multidisciplinary team improved the resection rate compared to a historical cohort with an excellent morbidity profile.

Keywords: adjuvant radiation; chordoma; clivus; endoscopic; multidisciplinary; proton; skull base.

Figure 1

Chart bars comparing various factors before 2013 and after 2013. (a) Endoscopic surgeries; (b) Resection degree; (c) Adjuvant radiation status (d) Recurrences in the first six years.

Figure 2

Kaplan–Meier curves of overall survival and progression-free survival (PFS). (a) Overall survivals of “before 2013” and “after 2013”; (b) PFS curves of “before 2013” and “after 2013”; (c) PFS curves based on the resection degree; (d) PFS curves based on adjuvant radiation status.

Figure 3

Magnetic resonance imaging (MRI) of five illustrative clival chordoma cases which required a different surgical strategy. (a) The first case is a holoclival chordoma (maximal diameter, 5 cm), which was resected by endoscopic endonasal approach (EEA). Preoperative T1-weighted (T1) fluid attenuated inversion recovery (FLAIR) sagittal MRI shows a clival mass displacing the pituitary gland and the brainstem; (b) The near total resection was attained by EEA. Enhanced T1 3D fast spin echo fat suppressed sagittal MRI in 3 years shows no recurrent tumor; (c) The second case is a holoclival chordoma (maximal diameter, 5 cm), which was resected by EEA after an occipito-cervical fusion (two stages). Preoperative enhanced T1 sagittal MRI shows a clival mass; (d) It was eroding the left occipital condyle (not shown) and an occipito-cervical fusion was done before EEA. Enhanced T1 fat suppressed sagittal MRI in 2.5 years shows no signs of recurrence; (e) The third case is a holoclival chordoma (maximal diameter, 8.8 cm), which was resected by endoscopic transcervical approach. Preoperative enhanced T1 fat suppressed sagittal MRI shows a clival mass extending as low as periodontoid space; (f) This case underwent two EEA and occipito-cervical fusion in an outside hospital. Enhanced T1 sagittal MRI shows a residual mass in the periodontoid space; (g) The subtotal resection was attained. T1 fast spin echo sagittal MRI in 1.5 years shows a stable residual mass; (h) The fourth case is a lower clival chordoma (maximal diameter, 4.3 cm), which was resected by EEA and open craniotomy. Preoperative enhanced T1 fast spin echo sagittal MRI shows an enhancing lobulated mass compression the cervicomedullary region; (i) EEA showed that the tumor was firm and arachnoid scarring was appreciated. One month after EEA, open tumor resection was done by right far lateral supracondylar craniotomy and C1 laminectomy (near total resection). Enhanced T1 spin echo sagittal MRI in about 8 years shows no signs of recurrence; (j) The last case is a holoclival chordoma (maximal diameter, 4.4 cm), which was resected by upfront open surgery. Preoperative enhanced T1 fat suppressed sagittal MRI shows an enhancing clival lobulated mass displacing the brainstem; (k) An open resection by left presigmoid subtemporal craniotomy with posterior petrosectomy was done (subtotal resection). T1 FLAIR sagittal MRI in 2.5 years shows a stable residual disease.

Figure 3

Magnetic resonance imaging (MRI) of five illustrative clival chordoma cases which required a different surgical strategy. (a) The first case is a holoclival chordoma (maximal diameter, 5 cm), which was resected by endoscopic endonasal approach (EEA). Preoperative T1-weighted (T1) fluid attenuated inversion recovery (FLAIR) sagittal MRI shows a clival mass displacing the pituitary gland and the brainstem; (b) The near total resection was attained by EEA. Enhanced T1 3D fast spin echo fat suppressed sagittal MRI in 3 years shows no recurrent tumor; (c) The second case is a holoclival chordoma (maximal diameter, 5 cm), which was resected by EEA after an occipito-cervical fusion (two stages). Preoperative enhanced T1 sagittal MRI shows a clival mass; (d) It was eroding the left occipital condyle (not shown) and an occipito-cervical fusion was done before EEA. Enhanced T1 fat suppressed sagittal MRI in 2.5 years shows no signs of recurrence; (e) The third case is a holoclival chordoma (maximal diameter, 8.8 cm), which was resected by endoscopic transcervical approach. Preoperative enhanced T1 fat suppressed sagittal MRI shows a clival mass extending as low as periodontoid space; (f) This case underwent two EEA and occipito-cervical fusion in an outside hospital. Enhanced T1 sagittal MRI shows a residual mass in the periodontoid space; (g) The subtotal resection was attained. T1 fast spin echo sagittal MRI in 1.5 years shows a stable residual mass; (h) The fourth case is a lower clival chordoma (maximal diameter, 4.3 cm), which was resected by EEA and open craniotomy. Preoperative enhanced T1 fast spin echo sagittal MRI shows an enhancing lobulated mass compression the cervicomedullary region; (i) EEA showed that the tumor was firm and arachnoid scarring was appreciated. One month after EEA, open tumor resection was done by right far lateral supracondylar craniotomy and C1 laminectomy (near total resection). Enhanced T1 spin echo sagittal MRI in about 8 years shows no signs of recurrence; (j) The last case is a holoclival chordoma (maximal diameter, 4.4 cm), which was resected by upfront open surgery. Preoperative enhanced T1 fat suppressed sagittal MRI shows an enhancing clival lobulated mass displacing the brainstem; (k) An open resection by left presigmoid subtemporal craniotomy with posterior petrosectomy was done (subtotal resection). T1 FLAIR sagittal MRI in 2.5 years shows a stable residual disease.