Management strategies in clival and craniovertebral junction chordomas: a 29-year experience

J Neurosurg 138:1640–1652, 2023

Chordomas represent one of the most challenging subsets of skull base and craniovertebral junction (CVJ) tumors to treat. Despite extensive resection followed by proton-beam radiation therapy, the recurrence rate remains high, highlighting the importance of developing efficient treatment strategies. In this study, the authors present their experience in treating clival and CVJ chordomas over a 29-year period.

METHODS The authors conducted a retrospective study of clival and CVJ chordomas that were surgically treated at their institution from 1991 to 2020. This study focuses on three aspects of the management of these tumors: the factors influencing the extent of resection (EOR), the predictors of survival, and the outcomes of the endoscopic endonasal approaches (EEAs) compared with open approaches (OAs).

RESULTS A total of 265 surgical procedures were performed in 210 patients, including 123 OAs (46.4%) and 142 EEAs (53.6%). Tumors that had an intradural extension (p = 0.03), brainstem contact (p = 0.005), cavernous sinus extension (p = 0.004), major artery encasement (p = 0.01), petrous apex extension (p = 0.003), or high volume (p = 0.0003) were significantly associated with a lower EOR. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 52.1% and 75.1%, respectively. Gross-total resection and Ki-67 labeling index < 6% were considered to be independent prognostic factors of longer PFS (p = 0.0005 and p = 0.003, respectively) and OS (p = 0.02 and p = 0.03, respectively). Postoperative radiation therapy correlated independently with a longer PFS (p = 0.006). Previous surgical treatment was associated with a lower EOR (p = 0.01) and a higher rate of CSF leakage after EEAs (p = 0.02) but did not have significantly lower PFS and OS compared with primary surgery. Previously radiation therapy correlated with a worse outcome, with lower PFS and OS (p = 0.001 and p = 0.007, respectively). EEAs were more frequently used in patients with upper and middle clival tumors (p = 0.002 and p < 0.0001, respectively), had a better rate of EOR (p = 0.003), and had a lower risk of de novo neurological deficit (p < 0.0001) compared with OAs. The overall rate of postoperative CSF leakage after EEAs was 14.8%.

CONCLUSIONS This large study showed that gross-total resection should be attempted in a multidisciplinary skull base center before providing radiation therapy. EEAs should be considered as the gold-standard approach for upper/middle clival lesions based on the satisfactory surgical outcome, but OAs remain important tools for large complex chordomas.

Clival Chordomas in the Endoscopic Endonasal Era: Comparison With Management With Open Skull Base Approaches

Neurosurgery 92:756–761, 2023

The most significant paradigm shift in surgical management of skull base chordomas has been the adoption of the endoscopic endonasal approach, but the impact on patient outcomes compared with open skull base approaches remains unclear.

OBJECTIVE: To compare a large series of patients treated by a single surgeon using primarily endoscopic endonasal approaches with previously published outcomes by the same surgeon using open skull base approaches.

METHODS: Between 2006 and 2020, 68 patients with skull base chordoma underwent resection using primarily endoscopic endonasal approaches. Outcomes and complications were compared with previously published results of resection of chordomas from 1991 to 2005 using open skull base approaches.

RESULTS: Compared with the prior cohort, the current principally endoscopic cohort demonstrated similar rates of OS (P = .86) and progression-free survival (P = .56), but patients undergoing first-time resection had significantly higher rates of radical resection (82.9% compared with 64.3%, P = .05) and required fewer staged surgeries (9.8% compared with 33.3%, P = .01).

CONCLUSION: There was no difference in survival rates for patients treated in the current era, primarily using endoscopic endonasal techniques, compared with previously published results using open skull-base approaches by the same surgeon. Although use of endoscopic endonasal approach resulted in higher rates of radical resection, patients undergoing first-time resection and fewer staged surgeries were required.

Clival Chordomas in the Endoscopic Endonasal Era: Clinical Management, Outcomes, and Complications

Neurosurgery 92:876–883, 2023

Surgical management of skull base chordomas has changed significantly in the past 2 decades, most notably with use of the endoscopic endonasal approach (EEA), although high quality outcome data using these modern approaches remain scarce.

OBJECTIVE: To evaluate outcomes in a large series of patients treated by a single surgeon, using primarily the EEA.

METHODS: Between 2006 and 2020, 68 patients with skull base chordoma underwent resection using mostly the EEA. Complications, outcomes, and potential contributing factors were evaluated using Kaplan-Meier survival analysis and univariable and multivariable Cox proportional hazards models.

RESULTS: Overall 5-year survival was 76.3% (95%CI 61.5%-86.0%), and 5-year progressionfree survival was 55.9%(95% CI 40.0%-69.0%). Inmultivariable analysis, radical resection was associated with significant reduction in risk of death (hazard ratio [HR] 0.04, 95% CI 0.005- 0.33, P = .003) and disease progression (HR 0.05, 95% CI 0.01-0.18, P < .001). Better preoperative function status reduced risk of death (HR 0.42 per 10-point increase in Karnofsky Performance Scale, 95% CI 0.28-0.63, P < .001) and progression (HR 0.60 per 10-point increase in Karnofsky Performance Scale, 95% CI 0.45-0.78, P < .001). Localization at the clivus reduced risk of death (HR 0.02, 95% CI 0.002-0.15, P < .001) and progression (HR 0.24, 95% CI 0.09-0.68, P = .007) compared with tumors at the craniovertebral junction.

CONCLUSION: In multivariable analysis, overall survival and progression-free survival of chordoma resection was most positively affected by radical resection, better preoperative functional status, and tumor location at the clivus rather than craniovertebral junction.

Stereotactic radiosurgery for intracranial chordomas: an international multiinstitutional study

J Neurosurg 137:977–984, 2022

The object of this study was to evaluate the safety, efficacy, and long-term outcomes of stereotactic radiosurgery (SRS) in the management of intracranial chordomas.

METHODS This retrospective multicenter study involved consecutive patients managed with single-session SRS for an intracranial chordoma at 10 participating centers. Radiological and neurological outcomes were assessed after SRS, and predictive factors were evaluated via statistical methodology.

RESULTS A total of 93 patients (56 males [60.2%], mean age 44.8 years [SD 16.6]) underwent single-session SRS for intracranial chordoma. SRS was utilized as adjuvant treatment in 77 (82.8%) cases, at recurrence in 13 (14.0%) cases, and as primary treatment in 3 (3.2%) cases. The mean tumor volume was 8 cm 3 (SD 7.3), and the mean prescription volume was 9.1 cm 3 (SD 8.7). The mean margin and maximum radiosurgical doses utilized were 17 Gy (SD 3.6) and 34.2 Gy (SD 6.4), respectively. On multivariate analysis, treatment failure due to tumor progression (p = 0.001) was associated with an increased risk for post-SRS neurological deterioration, and a maximum dose > 29 Gy (p = 0.006) was associated with a decreased risk. A maximum dose > 29 Gy was also associated with improved local tumor control (p = 0.02), whereas the presence of neurological deficits prior to SRS (p = 0.04) and an age > 65 years at SRS (p = 0.03) were associated with worse local tumor control. The 5- and 10-year tumor progression-free survival rates were 54.7% and 34.7%, respectively. An age > 65 years at SRS (p = 0.01) was associated with decreased overall survival. The 5and 10-year overall survival rates were 83% and 70%, respectively.

CONCLUSIONS SRS appears to be a safe and relatively effective adjuvant management option for intracranial chordomas. The best outcomes were obtained in younger patients without significant neurological deficits. Further well-designed studies are necessary to define the best timing for the use of SRS in the multidisciplinary management of intracranial chordomas.

The mini-combined transpetrosal approach: an anatomical study and comparison with the combined transpetrosal approach

Acta Neurochirurgica (2022) 164:1079–1093

The combined transpetrosal approach (CTPA) is a versatile technique suitable for challenging skull base pathologies. Despite the advantages provided by a wide surgical exposure, the soft tissue trauma, complex and time-consuming bony work, and cosmetic issues make it far from patient expectations. In this study, the authors describe a less invasive modification of the CTPA, the mini-combined transpetrosal approach (mini-CTPA), and perform a quantitative comparison between these two approaches.

Methods Five human specimens were used for this study. CTPA was performed on one side and mini-CTPA on the opposite side. The surgical freedom, petroclival and brainstem area of exposure, and maneuverability for 6 anatomical targets, provided by the CTPA and mini-CTPA, were calculated and statistically compared. The bony volumes corresponding to each anterior petrosectomy were also measured and compared. Three clinical cases with an operative video are also reported to illustrate the effectiveness of the approach.

Results The question-mark skin incision done along the muscle attachments permits an optimal cosmetic result. Even though the limited incision, the smaller craniotomy, and the less extensive bone drilling of mini-CTPA provide a smaller area of surgical freedom, the areas of exposure of petroclival region and brainstem were not statistically different between the two approaches. The antero-posterior maneuverability for the oculomotor foramen (OF), Meckel’s cave (MC) and the REZ of trigeminal nerve, and the supero-inferior maneuverability for OF, MC, Dorello’s canal, and REZ of CN VII are significantly reduced by the smaller opening. The bony volume of anterior petrosectomy resulted similar among the approaches.

Conclusions The mini-CTPA is an interesting alternative to the CTPA, providing comparable surgical exposure both for petroclival region and for brainstem. Although the lesser soft tissue dissection and bony opening decrease the surgical maneuverability, the mini-CTPA may reduce surgical time, potential approach-related morbidities, and improve cosmetic and functional outcomes for the patients.

Tumor Growth Rate as a New Predictor of Progression-Free Survival After Chordoma Surgery

Neurosurgery 89:291–299, 2021

Currently, different postoperative predictors of chordoma recurrence have been identified. Tumor growth rate (TGR) is an image-based calculation that provides quantitative information of tumor’s volume changing over time and has been shown to predict progression-free survival (PFS) in other tumor types.

OBJECTIVE: To explore the usefulness of TGR as a new preoperative radiological marker for chordoma recurrence.

METHODS: A retrospective single-institution study was carried out including patients reflecting these criteria: confirmed diagnosis of chordoma on pathological analysis, no history of previous radiation, and at least 2 preoperative thin-slice magnetic resonance images available to measure TGR. TGR was calculated for all patients, showing the percentage change in tumor size over 1 mo.

RESULTS: A total of 32 patients were retained for analysis. Patients with a TGR ≥ 10.12%/m had a statistically significantly lower mean PFS (P<.0001). TGR≥10.12%/m (odds ratio=26, P=.001) was observed more frequently in recurrent chordoma. In a subgroup analysis, we found that the association of Ki-67 labeling index≥6% and TGR≥10.12%/m was correlated with recurrence (P = .0008).

CONCLUSION: TGR may be considered as a preoperative radiological indicator of tumor proliferation and seems to preoperatively identify more aggressive tumors with a higher tendency to recur. Our findings suggest that the therapeutic strategy and clinical radiological follow-up of patients with chordoma can be adapted also according to this new parameter.

Augmented reality–mediated stereotactic navigation for execution of en bloc lumbar spondylectomy osteotomies

J Neurosurg Spine 34:700–705, 2021

En bloc spinal tumor resections are technically demanding procedures with high morbidity because of the conventionally large exposure area and aggressive resection goals. Stereotactic surgical navigation presents an opportunity to perform the smallest possible resection plan while still achieving an en bloc resection.

Augmented reality (AR)–mediated spine surgery (ARMSS) via a mounted display with an integrated tracking camera is a novel FDA-approved technology for intraoperative “heads up” neuronavigation, with the proposed advantages of increased precision, workflow efficiency, and cost-effectiveness. As surgical experience and capability with this technology grow, the potential for more technically demanding surgical applications arises.

Here, the authors describe the use of ARMSS for guidance in a unique osteotomy execution to achieve an en bloc wide marginal resection of an L1 chordoma through a posterior-only approach while avoiding a tumor capsule breach. A technique is described to simultaneously visualize the navigational guidance provided by the contralateral surgeon’s tracked pointer and the progress of the BoneScalpel aligned in parallel with the tracked instrument, providing maximum precision and safety. The procedure was completed by reconstruction performed with a quad-rod and cabled fibular strut allograft construct, and the patient did well postoperatively.

Finally, the authors review the technical aspects of the approach, as well as the applications and limitations of this new technology.

Long-term outcomes of high-dose single-fraction radiosurgery for chordomas of the spine and sacrum

J Neurosurg Spine 32:79–88, 2020

The current treatment of chordomas is associated with significant morbidity, high rates of local recurrence, and the potential for metastases. Stereotactic radiosurgery (SRS) as a primary treatment could reduce the need for en bloc resection to achieve wide or marginal margins. Spinal SRS outcomes support the exploration of SRS’s role in the durable control of these conventionally radioresistant tumors. The goal of the study was to evaluate outcomes of patients with primary chordomas treated with spinal SRS alone or in combination with surgery.

METHODS Clinical records were reviewed for outcomes of patients with primary chordomas of the mobile spine and sacrum who underwent single-fraction SRS between 2006 and 2017. Radiographic local recurrence-free survival (LRFS), overall survival (OS), symptom response, and toxicity were assessed in relation to the extent of surgery.

RESULTS In total, 35 patients with de novo chordomas of the mobile spine (n = 17) and sacrum (n = 18) received SRS and had a median post-SRS follow-up duration of 38.8 months (range 2.0–122.9 months). The median planning target volume dose was a 24-Gy single fraction (range 18–24 Gy). Overall, 12 patients (34%) underwent definitive SRS and 23 patients (66%) underwent surgery and either neoadjuvant or postoperative adjuvant SRS. Definitive SRS was selectively used to treat both sacral (n = 7) and mobile spine (n = 5) chordomas. Surgical strategies for the mobile spine were either intralesional, gross-total resection (n = 5) or separation surgery (n = 7) and for the sacrum en bloc sacrectomy (n = 11). The 3- and 5-year LRFS rates were 86.2% and 80.5%, respectively. Among 32 patients (91%) receiving 24-Gy radiation doses, the 3- and 5-year LRFS rates were 96.3% and 89.9%, respectively. The 3- and 5-year OS rates were 90.0% and 84.3%, respectively. The symptom response rate to treatment was 88% for pain and radiculopathy. The extent or type of surgery was not associated with LRFS, OS, or symptom response rates (p > 0.05), but en bloc resection was associated with higher surgical toxicity, as measured using the Common Terminology Criteria for Adverse Events (version 5.0) classification tool, than epidural decompression and curettage/intralesional resection (p = 0.03). The long-term rate of toxicity ≥ grade 2 was 31%, including 20% grade 3 tissue necrosis, recurrent laryngeal nerve palsy, myelopathy, fracture, and secondary malignancy.

CONCLUSIONS High-dose spinal SRS offers the chance for durable radiological control and effective symptom relief with acceptable toxicity in patients with primary chordomas as either a definitive or adjuvant therapy.



Prospective validation of a molecular prognostication panel for clival chordoma

J Neurosurg 130:1528–1537, 2019

There are currently no reliable means to predict the wide variability in behavior of clival chordoma so as to guide clinical decision-making and patient education. Furthermore, there is no method of predicting a tumor’s response to radiation therapy.

METHODS A molecular prognostication panel, consisting of fluorescence in situ hybridization (FISH) of the chromosomal loci 1p36 and 9p21, as well as immunohistochemistry for Ki-67, was prospectively evaluated in 105 clival chordoma samples from November 2007 to April 2016. The results were correlated with overall progression-free survival after surgery (PFSS), as well as progression-free survival after radiotherapy (PFSR).

RESULTS Although Ki-67 and the percentages of tumor cells with 1q25 hyperploidy, 1p36 deletions, and homozygous 9p21 deletions were all found to be predictive of PFSS and PFSR in univariate analyses, only 1p36 deletions and homozygous 9p21 deletions were shown to be independently predictive in a multivariate analysis. Using a prognostication calculator formulated by a separate multivariate Cox model, two 1p36 deletion strata (0%–15% and > 15% deleted tumor cells) and three 9p21 homozygous deletion strata (0%–3%, 4%–24%, and ≥ 25% deleted tumor cells) accounted for a range of cumulative hazard ratios of 1 to 56.1 for PFSS and 1 to 75.6 for PFSR.

CONCLUSIONS Homozygous 9p21 deletions and 1p36 deletions are independent prognostic factors in clival chordoma and can account for a wide spectrum of overall PFSS and PFSR. This panel can be used to guide management after resection of clival chordomas.


Cranial Chordoma: A New Preoperative Grading System

Neurosurgery 83:403–415, 2018

Chordomas are rare but challenging neoplasms involving the skull base. A preoperative grading system will be useful to identify both areas for treatment and risk factors, and correlate to the degree of resection, complications, and recurrence.

OBJECTIVE: To propose a new grading system for cranial chordomas designed by the senior author. Its purpose is to enable comparison of different tumors with a similar pathology to clivus chordoma, and statistically correlate with postoperative outcomes.

METHODS: The numerical grading system included tumor size, site of the tumor, vascular encasement, intradural extension, brainstem invasion, and recurrence of the tumor either after surgery or radiotherapy with a range of 2 to 25 points; it was used in 42 patients with cranial chordoma. The grading system was correlated with number of operations for resection, degree of resection, number and type of complications, recurrence, and survival.

RESULTS: We found 3 groups: low-risk 0 to 7 points, intermediate-risk 8 to 12 points, and high-risk≥13 points in the grading system. The 3 groupswere correlated with the following: extent of resection (partial, subtotal, or complete; P < .002); number of operative stages to achieve removal (P < .014); tumor recurrence (P = .03); postoperative Karnofsky Performance Status (P < .001); and with successful outcome (P = .005). The grading system itself correlated with the outcome (P = .005).

CONCLUSION: The proposed chordoma grading system can help surgeons to predict the difficulty of the case and know which areas of the skull base will need attention to plan further therapy.

Contralateral transmaxillary corridor: an augmented endoscopic approach to the petrous apex

J Neurosurg 129:211–219, 2018

The endoscopic endonasal approach (EEA) has been shown to be an effective means of accessing lesions of the petrous apex. Lesions that are lateral to the paraclival segment of the internal carotid artery (ICA) require lateralization of the paraclival segment of the ICA or a transpterygoid infrapetrous approach. In this study the authors studied the feasibility of adding a contralateral transmaxillary (CTM) corridor to provide greater access to the petrous apex with decreased need for manipulation of the ICA.

METHODS Using image guidance, EEA and CTM extension were performed bilaterally on 5 cadavers. The anterior wall of the sphenoid sinus and rostrum were removed. The angle of the surgical approach from the axis of the petrous segment of the ICA was measured. Five illustrative clinical cases are presented.

RESULTS The CTM corridor required a partial medial maxillectomy. When measured from the axis of the petrous ICA, the CTM corridor decreased the angle from 44.8° ± 2.78° to 20.1° ± 4.31°, a decrease of 24.7° ± 2.58°. Drilling through the CTM corridor allowed the drill to reach lateral aspects of the petrous apex that would have required lateralization of the ICA or would not have been accessible via EEA. The CTM corridor allowed us to achieve gross-total resection of the petrous apex region in 5 clinical cases with significant paraclival extension.

CONCLUSIONS The CTM corridor is a feasible extension to the standard EEA to the petrous apex that offers a more lateral trajectory with improved access. This approach may reduce the risk and morbidity associated with manipulation of the paraclival ICA.



Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins’ Law

Neurosurgery 82:652–660, 2018

Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined.

OBJECTIVE: To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins’ law for embryonal tumors.

METHODS: Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263).

RESULTS: Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival.

CONCLUSION: Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins’ law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

Surgical resection of skull-base chordomas: experience in case selection for surgical approach according to anatomical compartments and review of the literature

Acta Neurochir (2017) 159:1835–1845

Chordoma is a rare bony malignancy known to have a high rate of local recurrence after surgery. The best treatment paradigm is still being evaluated. We report our experience and review the literature. We emphasize on the difference between endoscopic and open craniotomy in regard to the anatomical compartment harboring the tumor, the limitations of the approaches and the rate of surgical resection.

Method: We retrospectively collected all patients with skullbase chordomas operated on between 2004 and 2014. Detailed radiological description of the compartments being occupied by the tumor and the degree of surgical resection is discussed.

Results: Eighteen patients were operated on in our facility for skull-base chordoma. Seventeen endoscopic surgeries were done in 15 patients, and 7 craniotomies were done in 5 patients. The mean age was 48.9 years (±19.8 years). When reviewing the anatomical compartments, we found that the most common were the upper clivus (95.6%) and lower clivus (58.3%), left cavernous sinus (66.7%) and petrous apex (∼60%). Most of the patients had intradural tumor involvement (70.8%). In all craniotomy cases, there was residual tumor in multiple compartments. In the endoscopic cases, the most difficult compartments for total resection were the lower clivus, and lateral extensions to the petrous apex or cavernous sinus.

Conclusions: Our experience shows that the endoscopic approach is a good option for midline tumors without significant lateral extension. In cases with very lateral or lower extensions, additional approaches should be added trying to achieve complete resection.

High-dose proton-based radiation therapy in the management of spine chordomas


J Neurosurg Spine 23:788–797, 2015

Spinal chordomas can have high local recurrence rates after surgery with or without conventional dose radiation therapy (RT). Treatment outcomes and prognostic factors after high-dose proton-based RT with or without surgery were assessed.

Methods The authors conducted a retrospective review of 126 treated patients (127 lesions) categorized according to disease status (primary vs recurrent), resection (en bloc vs intralesional), margin status, and RT timing.

Results Seventy-one sacrococcygeal, 40 lumbar, and 16 thoracic chordomas were analyzed. Mean RT dose was 72.4 GyRBE (relative biological effectiveness). With median follow-up of 41 months, the 5-year overall survival (OS), local control (LC), locoregional control (LRC), and distant control (DC) for the entire cohort were 81%, 62%, 60%, and 77%, respectively. LC for primary chordoma was 68% versus 49% for recurrent lesions (p = 0.058). LC if treated with a component of preoperative RT was 72% versus 54% without this treatment (p = 0.113). Among primary tumors, LC and LRC were higher with preoperative RT, 85% (p = 0.019) and 79% (0.034), respectively, versus 56% and 56% if no preoperative RT was provided. Overall LC was significantly improved with en bloc versus intralesional resection (72% vs 55%, p = 0.016), as was LRC (70% vs 53%, p = 0.035). A trend was noted toward improved LC and LRC for R0/R1 margins and the absence of intralesional procedures.

Conclusions High-dose proton-based RT in the management of spinal chordomas can be effective treatment. In patients undergoing surgery, those with primary chordomas undergoing preoperative RT, en bloc resection, and postoperative RT boost have the highest rate of local tumor control; among 28 patients with primary chordomas who underwent preoperative RT and en bloc resection, no local recurrences were seen. Intralesional and incomplete resections are associated with higher local failure rates and are to be avoided.

Factors Predicting Recurrence After Resection of Clival Chordoma

Clivus chordomas

Neurosurgery 76:179–186, 2015

Clival chordomas frequently recur because of their location and invasiveness.

OBJECTIVE: To investigate clinical, operative, and anatomic factors associated with clival chordoma recurrence.

METHODS: Retrospective review of clival chordomas treated at our center from 1993 to 2013.

RESULTS: Fifty patients (56% male) with median age of 59 years (range, 8-76) were newly diagnosed with clival chordoma of mean diameter 3.3 cm (range, 1.5-6.7). Symptoms included headaches (38%), diplopia (36%), and dysphagia (14%). Procedures included transsphenoidal (n = 34), transoral (n = 4), craniotomy (n = 5), and staged approaches (n = 7). Gross total resection (GTR) rate was 52%, with 83%mean volumetric reduction, values that improved over time.While the lower third of the clivus was the least likely superoinferior zone to contain tumor (upper third = 72%/middle third = 82%/lower third = 42%), it most frequently contained residual tumor (upper third = 33%/middle third = 38%/lower third = 63%; P , .05). Symptom improvement rates were 61% (diplopia) and 53% (headache). Postoperative radiation included proton beam (n = 19), cyberknife (n = 7), intensity-modulated radiation therapy (n = 6), external beam (n = 10), and none (n = 4). At last follow-up of 47 patients, 23 (49%) remain disease-free or have stable residual tumor. Lower third of clivus progressed most after GTR (upper/mid/lower third = 32%/ 41%/75%). In a multivariate Cox proportional hazards model, male gender (hazard ratio [HR] = 1.2/P = .03), subtotal resection (HR = 5.0/P = .02), and the preoperative presence of tumor in the middle third (HR = 1.2/P = .02) and lower third (HR = 1.8/P = .02) of the clivus increased further growth or regrowth, while radiation modality did not.

CONCLUSION: Our findings underscore long-standing support for GTR as reducing chordoma recurrence. The lower third of the clivus frequently harbored residual or recurrent tumor, despite staged approaches providing mediolateral (transcranial 1 endonasal) or superoinferior (endonasal 1 transoral) breadth. There was no benefit of proton-based over photonbased radiation, contradicting conventional presumptions.

Full endoscopic endonasal expanded approach to the petroclival region: optimizing the carotid-clival window

Full endoscopic endonasal expanded approach to the petroclival region- optimizing the carotid-clival window

Acta Neurochir (2014) 156:1627–1629

The petroclival junction (PCJ) is a challenging skull base location from neurosurgical point of view, especially if the retrocarotid space has to be reached.

Method In response to this challenge, this report provides a detailed full description of the endoscopic endonasal expanded approach (EEA) to the petroclival region and retrocarotid space. We present the technique step by step, introducing a critical concept about the optimization of the petroclival drilling, generating the carotid-clival window (CCW). The CCW is delimited by the paraclival segment of the internal carotid artery ICA anterolaterally, the petrous bone posterolaterally, the clival dura medially, the synchondrosis inferiorly, and the cavernous sinus superiorly; therefore, this approach exposes an important nuance to augment the previous approaches for PCJ and retrocarotid space.

Conclusion This technique provides a good surgical window and carries minimal risk.

Endoscopic endonasal transcavernous posterior clinoidectomy

Interdural pituitary transposition

J Neurosurg 121:91–99, 2014

The object of this paper was to describe the surgical anatomy and technical nuances of the endonasal transcavernous posterior clinoidectomy approach with interdural pituitary transposition and to report the clinical outcome of this technical modification.

Methods. The surgical anatomy of the proposed approach was studied in 10 colored silicon-injected anatomical specimens. The medical records of 12 patients that underwent removal of the posterior clinoid(s) with this technique were reviewed.

Results. The natural anatomical corridor provided by the cavernous sinus is used to get access to the posterior clinoid by mobilizing the pituitary gland in an interdural fashion. The medial wall of the cavernous sinus is preserved intact and attached to the gland during its medial and superior mobilization. This provides protection to the gland, allowing for preservation of its venous drainage pathways. The inferior hypophyseal artery is transected to facilitate the manipulation of the medial wall of the cavernous sinus and pituitary gland. This approach was successfully performed in all patients, including 6 with chordomas, 5 with petroclival meningiomas, and 1 with an epidermoid tumor. No patient in this series had neurovascular injury related to the posterior clinoidectomy. There were no instances of permanent hypopituitarism or diabetes insipidus.

Conclusions. The authors introduce a surgical variant of the endoscopic endonasal posterior clinoidectomy approach that does not require intradural pituitary transposition and is more effective than the purely extradural approach. The endoscopic endonasal transcavernous approach facilitates the removal of prominent posterior clinoids increasing the working space at the lateral recess of the interpeduncular cistern, while preserving the pituitary function.

Endoscopic endonasal approach in the management of skull base chordomas

Endoscopic approach skull base chordomas

Neurosurg Rev (2014) 37:217–225

Skull base chordomas represent very interesting neoplasms, due to their rarity, biological behavior, and resistance to treatment. Their management is very challenging. Recently, the use of a natural corridor, through the nose and the sphenoid sinus, improved morbidity and mortality allowing also for excellent removal rates.

Prospective analysis of 54 patients harboring a skull base chordoma that were managed by extended endonasal endoscopic approach (EEA). Among the 54 patients treated (during a 72 months period), 21 were women and 33 men, undergoing 58 procedures. Twenty-two cases (40 %) were recurrent and 32 (60 %) newly diagnosed chordomas. Among the 32 newly diagnosed chordomas, a gross total resection was achieved in 28 cases (88%), a near total (>95%of tumor) in 2 cases (6%), a partial (>50 % of tumor) in 2 cases (6 %). Among the 22 recurrent chordomas, resection was complete in 7 cases (30 %), near total in 7 (30%), and partial in 8 (40 %). The global gross total resection rate was 65 % (35/54 cases). Four patients (11 %) recurred and 4 (11 %) progressed within a mean follow-up of 34 months (range 12–84 months). Four patients (11 %) were re-operated; one patient (1.8 %) died due to disease progression, one patient (1.8 %) died 2 weeks after surgery due to a massive bleeding from an ICA pseudo aneurysm. CSF leakage occurred in four patients (8 %), and meningitis in eight cases (14 %). No new permanent neurological deficit occurred.

The EEA management of skull base chordomas requires a long and gradual learning curve that once acquired offers the possibility of either similar or better resection rates as compared to traditional approaches while morbidity is improved.

The use of intraoperative navigation for complex upper cervical spine surgery

The use of intraoperative navigation for complex upper cervical spine surgery

Neurosurg Focus 36 (3):E5, 2014

Imaging guidance using intraoperative CT (O-arm surgical imaging system) combined with a navigation system has been shown to increase accuracy in the placement of spinal instrumentation. The authors describe 4 complex upper cervical spine cases in which the O-arm combined with the StealthStation surgical navigation system was used to accurately place occipital screws, C-1 screws anteriorly and posteriorly, C-2 lateral mass screws, and pedicle screws in C-6. This combination was also used to navigate through complex bony anatomy altered by tumor growth and bony overgrowth.

The 4 cases presented are: 1) a developmental deformity case in which the C-1 lateral mass was in the center of the cervical canal causing cord compression; 2) a case of odontoid compression of the spinal cord requiring an odontoidectomy in a patient with cerebral palsy; 3) a case of an en bloc resection of a C2–3 chordoma with instrumentation from the occiput to C-6 and placement of C-1 lateral mass screws anteriorly and posteriorly; and 4) a case of repeat surgery for a non-union at C1–2 with distortion of the anatomy and overgrowth of the bony structure at C-2.

Endoscopic Endonasal Transclival Approach to the Jugular Tubercle

Neurosurgery 71[ONS Suppl 1]:ons146–ons159, 2012

The jugular tubercle is a rounded bony prominence that arises from the inferolateral margin of the clivus. In a previous publication, we described the surgical anatomy of the expanded endonasal approach to the jugular tubercle.

OBJECTIVE: To illustrate the translation of laboratory work to the operating room describing the anatomic and technical nuances of the endonasal approach to the jugular tubercle.

METHODS: We review the relevant surgical anatomy needed to perform an endonasal approach to the jugular tubercle, and we select 4 different lesions to illustrate the application of our laboratory findings.

RESULTS: In the first case, exposure and partial drilling of the jugular tubercle was critical to gain an adequate corridor to the meningioma, particularly to its inferolateral margin. This allowed for early devascularization, safe extracapsular dissection, and preservation of surrounding neurovascular structures. In addition, the jugular tubercle was hyperostotic and its resection, along with generous dural removal, provided a grade I Simpson tumor resection. In the second (chondrosarcoma) and third (chordoma) cases, the jugular tubercle was infiltrated by tumor, and consequently its complete resection was essential to achieve total tumor removal. In the last case, an unusual adrenocorticotropic hormone-secreting adenoma recurrence at the jugular tubercle region, the technical modification of the transclival approach presented here was successfully applied to achieve complete resection and Cushing disease remission.

CONCLUSION: The transjugular tubercle variant of the expanded endonasal transclival approach allows for direct access to ventrolateral lesions in the inferior clival/petroclival region with no cerebral or cerebellar retraction, or cranial nerve manipulation during the approach.


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