Management Of Skull Base Chordomas Research Articles

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

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. To evaluate outcomes in a large series of patients treated by a single surgeon, using primarily the EEA. 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. Overall 5-year survival was 76.3% (95% CI 61.5%-86.0%), and 5-year progression-free survival was 55.9% (95% CI 40.0%-69.0%). In multivariable 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. 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.

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

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. 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. 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. 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). 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.

Gamma Knife Management of Skull Base Chordomas: Is it a Choice?

Background:Skull base chordomas are locally invasive tumors which able to extend in different directions with skull base invasion. Although they are histologically benign, they have invasive nature makes total resection virtually impossible to achieve in most cases and this lead to residual tumors after surgery. To decrease postoperative surgical resection morbidity of these tumors, gamma knife radiosurgery (GKRS) was performed as alternative management for these residual chordomas to evaluate its safety and efficacy.Materials and Methods:A retrospective study was made on eight residual skull base chordomas treated with GKRS between 2011 and 2015. The mean patient age was 49 years (range 30–73 years). Four patients harboring chordoma were male, and four patients were females with 1:1 ratio. All patients had undergone one prior surgery. Patients were treated with peripheral dose ranged between 12–15 gray (Gy) (mean 13.75 Gy) usually at 35% to 50% isodose curve (mean 38.8%). The maximum dose to the adjacent brain stem area ranged between 10 and 12 Gy. All patients were followed up from 8 to 39 months (mean 18 months).Results:The tumor control rate was 50% and 25% after 18 and 36 months, respectively, but we found that their wasdeclined in the tumor control rate with long follow-up time. Four tumors were stable in their size just for 18 months, and then there two of these tumors were progressed in their size, the other four patients showed progression in their tumors in their 1st year of treatment without sign of central tumor necrosis.Conclusion:Skull base chordoma patients complained from symptoms due to tumor mass effect which were not prospected to respond to GKRS alone as the aim of this type of treatment was the local tumor control, the tumor control rate declined with long follow-up time and this correlated with radioresistant nature of skull base chordoma. We advise a gross total resection to decrease the tumor volume, and this making gamma knife a reasonable treatment modality.

Clinical Application of Multimodal Neuronavigation System in Neuroendoscope-Assisted Skull Base Chordoma Resection.

Skull base chordoma is a rare tumor arising from embryonic remnants of the notochord with invasive potential. Due to the destruction of osseous landmarks and invasion of surrounding structures, surgical resection is challenging. The authors explored the clinical value of a multimodal neuronavigation system in skull base chordoma resection using a neuroendoscope. Between January 2012 and January 2016, the authors utilized neuroendoscopy to excise skull base chordoma in 93 patients. The authors performed 45 operations assisted by multimodal neuronavigation (neuronavigation group) and 48 without intraoperative imaging guidance (control group). In the control group, 35 patients (73%) underwent gross total resection. In the neuronavigation group, all patients underwent gross total resection without radiographically identified bleeding. Only 1 patient (2%) in the neuronavigation group showed a temporary reduction in vision, which improved after symptomatic treatment. In contrast, there were 4 patients (8%) with postoperative complication, including 2 patients with intracranial hematoma and 2 with neurological deficits. Complication rates were higher than the neuronavigation group. In the follow-up period, 2 patients in the control group with subtotal resection had recurrence within 24 months, but without extracranial metastases. The multimodal neuronavigation system could contribute intraoperative real-time guidance for spatial relationships between lesions and adjacent neurovascular structures, as well as eroded and distorted anatomical landmarks through multiple image fusion and 3-dimensional reconstruction. It significantly improves surgical outcome and provides a new insight into the management of skull base chordomas.

Initial Management of Skull Base Chordomas: Does Early Access to Multidisciplinary Care Affect Outcome?

Initial Management of Skull Base Chordomas: Does Early Access to Multidisciplinary Care Affect Outcome?

Three-dimensional endoscopy in transnasal transsphenoidal approach to clival chordomas.

The purpose of this prospective, observational study was to evaluate the management of skull base chordomas surgically resected via a 3D-endoscopic transnasal approach. Thirteen consecutive patients were observed and only 9 were surgically treated using a 3D-endoscopic transnasal approach assisted by a novel 3D visualization system. Nine consecutive male patients (mean age, 57.4 years) underwent exclusive 3D-endoscopic transnasal transsphenoidal resection of clival chordomas; gross total resection was achieved in 66.6% of cases (6 of 9 patients), near-total resection in 11.2% (1 of 9 patients), and partial resection in 22.2% (2 of 9 patients). The complications observed were 2 cases of postoperative cerebrospinal fluid (CSF) leaks and 1 case of temporary VI cranial nerve palsy. No discomfort was recorded; when a dura opening was required, 3D vision allowed an accurate intradural sharp dissection and a precise repair of the skull base. © 2015 Wiley Periodicals, Inc. Head Neck 38: E1814-E1819, 2016.

Impact of cytogenetic abnormalities on the management of skull base chordomas

Cytogenetic studies of chordomas are scarce and show multiple changes involving different chromosomes. These abnormalities are implicated in the pathogenesis of chordoma, but the clinical significance of these changes is yet to be determined. In this study, the authors discuss the cytogenetic changes in a large series of skull base chordomas with long-term follow-up and focus on the impact of these changes on the prognosis, progression, and management of the disease. The karyotypes of chordomas in 64 patients (36 men and 28 women) were studied in relation to survival and recurrence or progression over a mean follow-up period of 48 +/- 37.5 months. The standard G-banding technique was used for karyotype analysis. Statistical analysis was performed with the Fisher exact test and ORs, and Kaplan-Meier curves were generated for survival and recurrence/progression of disease. Seventy-four percent of de novo chordomas had normal karyotypes and a 3% recurrence rate; there was a 45% recurrence rate in de novo tumors with abnormal karyotypes (p < 0.01). Recurrent tumors were associated with a high incidence of abnormal karyotype (75%). The OR for recurrence in lesions with an abnormal versus a normal karyotype was 12. Aberrations in chromosomes 3, 4, 12, 13, and 14 were associated with frequent recurrence and decreased survival time. Ninety-five percent of cases with progression involved chromosome 3 and/or 13. The median survival time was 4 months when both of these chromosomes had aberrations (p = 0.02). Chordomas with normal karyotypes were associated with a low rate of recurrence and a long patient survival, and recurrent chordomas were associated with an abnormal karyotype, disease progression, and poor survival. De novo chordomas with normal karyotypes may be amenable to radical resection and adjunctive proton beam therapy. Recurrent and de novo chordomas with abnormal karyotypes were associated with complex cytogenetic abnormalities and a poor prognosis, particularly in the presence of aberrations underlying tumor progression in chromosomes 3, 4, 12, 13, and 14.

Chordomas of the skull base: surgical management and outcome

The goal of this study was to report on the surgical management of skull base chordomas and to evaluate both the short- and long-term treatment outcomes. The authors retrospectively studied data from 49 patients who had undergone consecutive surgeries at a single institution. They also analyzed patterns of chordoma extension. Complications and surgery-related morbidity were recorded. A Kaplan-Meier analysis was performed to determine survival rates in patients 5 and 10 years after their first surgery. Operative approaches were selected on the basis of the predominant tumor extension. The approach used most frequently was the transethmoidal in 36.3%, followed by the pterional in 23.4% and the retrosigmoid in 23.4%. The tumor was totally removed in 49.4% and subtotally in 50.6%. The rate of total removal was highest at initial surgery (78%) and progressively declined thereafter. In 11.8% of cases a new neurological deficit developed, while the preoperative deficit remained unchanged. In 20% of cases the preoperative deficits improved, but new deficits also appeared. The 5- and 10-year survival rates are 65 and 39%, respectively. With an individually tailored surgical approach, total tumor removal in 78% of the cases was achieved at the initial surgery. Radical surgery appears to increase slightly the surgical morbidity, but at the same time prolongs the recurrence-free interval. Chordomas cannot be regarded as surgically curable tumors given the 5- and 10-year survival rates in patients harboring such lesions.

Surgical Management of Petroclival Chordomas: Report of Eight Cases

In the management of skull base chordomas, surgical treatment is essential to achieve long-term control. A petroclival chordoma growing laterally in the skull base is one of the most challenging tumors for neurosurgeons. We have treated petroclival chordomas based on the principle of maximal surgical resection of the tumor with minimal morbidity. Lateral skull base approaches were used to approach petroclival chordomas in eight patients. The surgical procedure involved removal of soft tumor tissue and extensive drilling of adjacent bony structures. Gross total resection of the tumor was achieved in six patients. Subtotal resection in the remaining two patients was associated with acceptable morbidity. In cases of petroclival chordomas, lateral skull base approaches can be used as a primary procedure, although those approaches may be associated with high rates of morbidity and mortality.

A Prospective Study of the Management of Skull Base Chordomas and Chondrosarcomas

In the past, the similar presentation of these two distinct tumors has resulted in an emphasis on the complexities of their surgical removal. Little consideration has been given to their vastly different natural history.

Evaluation of a new concept for the management of skull base chordomas and chondrosarcomas

Chordomas and chondrosarcomas of the skull base are rare locally invasive tumors associated with high recurrence rates. The aim of this study was to evaluate the concept of microsurgical tumor volume reduction followed by early gamma knife surgery (GKS). Thirteen patients with 15 tumors were treated between October 2000 and June 2003. There were three patients (23.1%) with chordomas and 10 (76.9%) with chondrosarcomas. There were nine men and four women who ranged in age between 19 and 69 years. All patients first underwent maximal tumor resection. Within 2 to 10 months after surgery they were treated with GKS. The mean postoperative tumor volume treated with GKS was 9.7 cm3 (range 1.4-20.3 cm3). Follow-up computerized tomography and magnetic resonance imaging examinations with volumetric tumor analysis were performed every 6 months after GKS. The mean treatment dose was 17 Gy and the mean isodose was 52%. The mean follow-up duration was 17 months during which there was only one tumor recurrence at the margin of the radiation field. The mean volume reduction was 35.4%. Results of this treatment strategy are encouraging but the efficacy of this multimodal treatment combining surgery and early GKS requires a longer follow up.

Evaluation of a new concept for the management of skull base chordomas and chondrosarcomas

Object. Chordomas and chondrosarcomas of the skull base are rare locally invasive tumors associated with high recurrence rates. The aim of this study was to evaluate the concept of microsurgical tumor volume reduction followed by early gamma knife surgery (GKS). Methods. Thirteen patients with 15 tumors were treated between October 2000 and June 2003. There were three patients (23.1%) with chordomas and 10 (76.9%) with chondrosarcomas. There were nine men and four women who ranged in age between 19 and 69 years. All patients first underwent maximal tumor resection. Within 2 to 10 months after surgery they were treated with GKS. The mean postoperative tumor volume treated with GKS was 9.7 cm3 (range 1.4–20.3 cm3). Follow-up computerized tomography and magnetic resonance imaging examinations with volumetric tumor analysis were performed every 6 months after GKS. The mean treatment dose was 17 Gy and the mean isodose was 52%. The mean follow-up duration was 17 months during which there was only one tumor recurrence at the margin of the radiation field. The mean volume reduction was 35.4%. Conclusions. Results of this treatment strategy are encouraging but the efficacy of this multimodal treatment combining surgery and early GKS requires a longer follow up.