Supracerebellar Infratentorial Approach, Indications, and Technical Pitfalls.

Posterior tentorial incisura not infrequently requires to be exposed for tumors of pineal gland, pulvinar, midbrain and cerebellum, aneurysms, arteriovenous malformations. Residing almost at the center of the brain, this area is almost equal distance to any point on the calvarium behind coronal sutures enabling alternative routes to encounter. Compared to supratentorial routes either subtemporal or suboccipital approach, infratentorial supracerebellar route has several advantages as providing shortest, most direct approach to the lesions of this area without encountering any important arteries and veins. Since its initial description at the early twentieth century, a wide range of complications arising from cerebellar infarction, air embolism, and neural tissue damage have been encountered. Working in a deep, narrow corridor without enough illumination and visibility under very limited anesthesiology support hindered popularization of this approach. In the contemporary era of neurosurgery, advanced diagnostic tools and surgical microscopes with state-of-the-art microsurgery techniques coupled with modern anesthesiology have eliminated almost all drawbacks of infratentorial supracerebellar approach.

Prognostic value of Ki-67 index in primary intracranial tumors of infants.

OBJECTIVE: Primary intracranial tumors are rare tumors in infants. They differ from those found in other pediatric age groups in terms of clinical presentation, histopathological diagnosis, adjuvant therapies, and outcome. Ki-67 index has also shown promising results as a prognostic factor in different types of intracranial tumors in children and adults. However, the importance and the best cutoff point of Ki-67 index in primary intracranial tumors of infants remains unclear. We aimed to analyze prognostic value of Ki-67 index in primary intracranial tumors of infants. METHODS: This study retrospectively reviewed the records of 28 infants undergoing surgical resection for primary intracranial tumors between April 2016 and March 2021. We analyzed clinical characteristics, tumor location, extent of resection, histopathological diagnosis, Ki-67 index, and overall survival (OS). To define the most relevant cutoff value for Ki-67 index, "Cutoff Finder" was used. RESULTS: The median age at diagnosis was 188 days for all patients. Fifteen of the patients were boys and 13 were girls. Tumors were located supratentorial in 13 patients and infratentorial in 15 patients. Gross total resection was performed in 7 of 13 supratentorial tumors and 9 of 15 infratentorial tumors. The mean Ki-67 index of the supratentorial tumors was 49.6%, the median was 55%; for infratentorial tumors, the mean was 49.9%, and the median was 70%. Tumor grade (p = 0.019) and Ki-67 index (p = 0.003) were found as significant predictors of OS in log-rank testing for Kaplan-Meier survival analysis. Univariate cox regression analysis identified high Ki-67 index as prognostic factor for worse OS, with hazard ratio of 8.852 (95% CI 1.95-64.80; p = 0.0108). High Ki-67 index was found as independent prognostic factor for worse OS in multivariate cox regression analysis (HR 7.036; 95% CI 1.229-65.82; p = 0.0457). CONCLUSION: High-grade and high Ki-67 index were associated with worse outcome. Ki-67 index did show a distinct prognostic value for OS within our cohort at a cutoff value of 72.5%.

Does Gross Total Resection Improve Progression-Free and Overall Survival in Pediatric Intracranial Ependymomas? Single-Center Clinical Experience Of 61 Cases.

OBJECTIVE: To share our clinical experience of 25 years and identify prognostic factors for progression-free and overall survival in pediatric intracranial ependymomas. METHODS: In total, 61 children who were treated between 1995 and 2020 in a single institution were included in the study. Medical records of the patients were retrospectively reviewed to obtain and analyze the following data: patient age at first surgery, sex, presenting symptoms, hydrocephalus and any invasive treatment, anatomic site, extent of resection, pathologic grade, time to progression, and time to death. Progression-free and overall survival rates and affecting factors were analyzed by Kaplan-Meier method. RESULTS: Dysphagia, number of surgeries, and spinal seeding were associated with progression free and overall survival in univariate analysis. The extent of resection, World Health Organization grade, and visual problems were also associated with progression whereas sex was associated with overall survival. Cox regression identified the extent of resection and single surgery as an independent prognostic factor for progression-free survival. No independent factor was found for overall survival. CONCLUSIONS: This single center experience of 25 years confirms the beneficial effect of gross total resection on disease progression. Although spinal seeding seems to affect survival rates, greater number of cases are needed to reveal its full effect.

Preoperative and postoperative high angular resolution diffusion imaging tractography of cerebellar pathways in posterior fossa tumors.

This study aimed to utilize high angular resolution diffusion magnetic resonance imaging (HARDI) tractography in the mapping of the pathways of the cerebellum associated with posterior fossa tumors (infratentorial neoplasms) and to determine whether it is useful for preoperative and postoperative evaluation. Retrospective data from 30 patients (age 2-16 yr) with posterior fossa tumor (17 low grade, 13 high grade) and 30 age-sex-matched healthy controls were used. Structural and diffusion-weighted images were collected at a 3-tesla scanner. Tractography was performed using Diffusion Toolkit software, Q-ball model, FACT algorithm, and angle threshold of 45 degrees. Manually assessed regions of interest were placed to identify reconstructed fiber pathways passing through the superior, medial, and inferior cerebellar peduncles for the preoperative, postoperative, and healthy control groups. Fractional anisotropy (FA), apparent diffusion coefficient (ADC), and track volume measures were obtained and analyzed. Statistically significant differences were found between the preop/postop, preop/control, and postop/control comparisons for the volume of the tracts in both groups. Displacement and disruption of the pathways seemed to differ in relation to the severity of the tumor. The loss of pathways after the operation was associated with selective resection during surgery due to tumor infiltration. There were no FA differences but significantly higher ADC in low-grade tumors, and no difference in both FA and ADC in high-grade tumors. The effects of posterior fossa tumors on cerebellar peduncles and reconstructed pathways were successfully evaluated by HARDI tractography. The technique appears to be useful not only for preoperative but also for postoperative evaluation.

Pediatric brain tumors: a bibliometric analysis.

PURPOSE: The purpose of this study was to identify tendency and current issues in research on pediatric brain tumors over the past 20 years and to help researchers and investors explore new directions for future research in this subject. METHODS: Web of Science Core Collection was used for article selection and CiteSpace 5.8.R 1 was used for bibliometric analyses with these articles. RESULTS: The overall h-index was found to be 131 in the analysis made in a total of 4019 publications on the subject between the years 2000 and 2021. A total of 16,101 authors have published articles on pediatric brain tumors. The most active author in this field was Michael D. Taylor (h-index: 105). The publication which received the strongest citation burst among publications was published in 2016 by Louis et al. published in Acta Neuropathologica, and its content is the World Health Organization's classification of central nervous system tumors. Considering the country contribution, the USA is seen in the leading position. The most publications on the subject were followed by the Journal of Clinical Oncology. CONCLUSION: By examining the studies on childhood brain tumors carried out around the world, the subjects that can be determined as the focus were tried to be highlighted. And it has been seen that the scientific and industrial community should work together and the financial support for multidisciplinary studies should increase.

Lesional resective epilepsy surgery in childhood: Comparison of two decades and long-term seizure outcome from a single center.

OBJECTIVE: Epilepsy surgery has shown efficacy in children. We aimed to assess long-term seizure outcome in children who underwent epilepsy surgery and determine predictive factors for seizure freedom. METHODS: This is a retrospective study of 196 children who underwent epilepsy surgery between 1994 and 2015 and had a minimum postoperative follow-up of 5 years. RESULTS: The median age at the time of surgery was 9.5 (0.08-19.8) years; 110 (56.1%) had temporal, 62 (31.6%) had extratemporal resections, and 24 (12.2%) had hemispheric surgery. The duration of postsurgical follow-up was between 5 and 20 years (mean±SD: 7 ± 3.2). Overall, 129 of 196 (65.8%) patients had Engel class I outcome at final visit. Among patients who underwent temporal, extratemporal and hemispheric surgery; 84 of 110 (76.4%), 34 of 62 (54.8%), and 11 of 24 (45.8%) patients had complete seizure freedom, respectively (p: 0.016). Patients with tumors had the best outcome, with 83.1% seizure freedom. The number of preoperative antiseizure medications (OR 3.19, 95% CI 1.07-9.48), the absence of postoperative focal epileptiform discharges (OR 8.98, 95% CI 4.07-19.79) were independent predictors of seizure freedom. Across two decades, the age at surgery was decreased (p: 0.003), overall seizure freedom (61.8% vs 68%) did not differ. In the past decade, a higher proportion of malformations of cortical development was operated (14.7% vs 35.9%, p: 0.007). SIGNIFICANCE: Our findings showed favorable long-term seizure outcome in children who underwent epilepsy surgery. The results are encouraging for developing centers with limited resources to establish pediatric epilepsy programs.

The Endoscopic Trans-Sinusoidal Trans-Pterygopalatine Route to the Foramen Rotundum Approach in Trigeminal Neuralgia Treatment.

AIM: To describe a new, minimally invasive, and safe access to foramen rotundum via the endoscopic endonasal trans-sinusoidal route for trigeminal nerve blockage. MATERIAL AND METHODS: We studied 5 fresh cadavers bilaterally, and 10 pterygopalatine fossae and maxillary nerves using the endoscopic endonasal trans-sinusoidal trans-pterygopalatine approach. RESULTS: The proposed approach enabled the maxillary nerve to be visualized after revealing the foramen rotundum with the help of some craniometric measurements. Distance between sphenoid sinus lateral wall and maxillary sinus posterior wall was measured. This measurement was found to be an important triangulation point to determine the location of the entrance to the pterygopalatine fossa. The distance between the foramen rotundum and the sphenopalatine artery was found to be significant. The foramen rotundum diameter was calculated, and the maxillary nerve was found to be situated inferolateral to the foramen rotundum. Therefore, the safe entry zone was determined superomedially. No significant difference was found between male and female and the right and left nostrils. CONCLUSION: Various surgical procedures have been applied to treat drug-resistant trigeminal neuralgia. Nevertheless, their cure rates remain less than anticipated. Recurrences up to 30% have been reported in the literature. In this study, the endoscopic endonasal transmaxillary trans-pterygopalatine route offers a new perspective on the foramen rotundum approach and provides a panoramic and safe view in previously high-risk percutaneous interventions.

The effect of pentoxifylline on cerebral vasospasm following experimental subarachnoid hemorrhage.

OBJECTS: Cerebral vasospasm is an important event that occurs following subarachnoid hemorage which has significant mortality and morbidity. The goal in this study was to investigate the effect of pentoxifylline on vasospasm in an experimental subarachnoid hemorrhage model. METHODS: In this study, 20 male New Zeland White rabbits weighing 3000-3500 g were assigned randomly to four groups. Animals in group 1 served as controls. Animals in group two received only intravenous pentoxifylline injection 3 times in 12 h intervals. In group 3, SAH was induced and no injection was given. Animals in group 4 received intravenous pentoxifylline (6 mg/kg) injections 3 times at 12th, 24th and 36th hours after subarachnoid hemorrhage induction. All animals were sacrificed and basilar arteries were removed at 48th hour. Basilar artery vessel diameters, wall thicknesses and luminal section areas were measured with Spot for Windows version 4.1. Statistical analysis was performed using ANOVA and Kruskall-Wallis tests. RESULTS: Mean basilar artery luminal section areas and luminal diameters in group 4 were significantly higher compared to group 3 (p < 0.05). Basilar artery wall thicknesses and were found to be higher in group 3 than in other groups and this was also statistically significant (p < 0.05). CONCLUSION: Our study demonstrated that intravenous administration of pentoxifylline significantly decreases vasospasm after subarachnoid hemorrhage.

International consensus classification of hippocampal sclerosis and etiologic diversity in children with temporal lobectomy.

INTRODUCTION: The distribution of hippocampal sclerosis (HS) subtypes, according to the classification of the International League Against Epilepsy (ILAE), has been reported mainly in adult patients. We aimed to review the pathological findings in children who had anterior temporal lobectomy accompanied with amygdalohippocampectomy, in view of the current classification, and evaluate postsurgical outcome with respect to HS subtypes in childhood. METHODS: Seventy children who underwent temporal resections for treatment of medically refractory epilepsy, with a minimum follow-up of 2 years, were included; the surgical hippocampus specimens were re-evaluated under the HS ILAE classification. RESULTS: Neuropathological evaluations revealed HS type 1 in 38 patients (54.3%), HS type 2 in 2 (2.8%), HS type 3 in 21 patients (30%), and no HS in 9 patients (12.9%). Of 70 patients, 23 (32.9%) had dual pathology, and the most common pattern was HS type 3 with low-grade epilepsy-associated brain tumors (LEAT). The distribution of HS types with respect to age revealed that HS type 3 and no HS subgroups had significantly more patients younger than 12 years, compared with those of HS type 1 (90.5%, 77.8% vs 47.4%, respectively). History of febrile seizures was higher in HS type 1. Prolonged/recurrent febrile seizures were most common in patients 12 years and older, whereas LEAT was the most common etiology in patients under 12 years of age (p < 0.001). Patients with HS type 1 had longer duration of epilepsy and an older age at the time of surgery compared with patients with HS type 3 and no HS (p: 0.031, p: 0.007). At final visit, 74.3% of the patients were seizure-free. Seizure outcome showed no significant difference between pathological subtypes. CONCLUSIONS: Our study presents the distribution of HS ILAE subtypes in an exclusively pediatric series along with long-term seizure outcome. The study reveals that the leading pathological HS subgroup in children is HS type 1, similar with adult series. Hippocampal sclerosis type 2 is significantly less in children compared with adults; however, HS type 3 emerges as the second most predominant group because of dual pathology, particularly LEAT. Further studies are required regarding clinicopathological features of isolated HS in pediatric cohort. Seizure-free outcome was favorable and similar in all HS types in children. The proportion of HS types may be better defined in pediatric patients with temporal resections, as the current HS ILAE classification becomes more widely used, and may help reveal the surgical and cognitive outcome with respect to HS types.

Long-term effects of vagus nerve stimulation in refractory pediatric epilepsy: A single-center experience.

INTRODUCTION: Vagus nerve stimulation (VNS) has been used as an adjunctive therapy for both children and adults with refractory epilepsy, over the last two decades. In this study, we aimed to evaluate the long-term effects and tolerability of VNS in the pediatric drug-resistant epilepsy (DRE) and to identify the predictive factors for responsiveness to VNS. METHODS: We retrospectively reviewed the medical records of pediatric patients who underwent VNS implantation between 1997 and 2018. Patients with ≥50% reduction of seizure frequency compared with the baseline were defined as "responders". The clinical characteristics of responders and nonresponders were compared. RESULTS: A total of 58 children (male/female: 40/18) with a mean follow-up duration of 5.7 years (3 months to 20 years) were included. The mean age at implantation was 12.4 years (4.5 to 18.5 years). Approximately half (45%) of our patients were responders, including 3 patients (5.8%) who achieved seizure freedom during follow-up. The age of seizure-onset, duration of epilepsy, age at implantation, and etiologies of epilepsy showed no significant difference between responders and nonresponders. Responders were more likely to have focal or multifocal epileptiform discharges (63%) on interictal electroencephalogram (EEG), when compared to nonresponders (36%) (p = .07). Vocal disturbances and paresthesias were the most common side effects, and in two patients, VNS was removed because of local reaction. CONCLUSION: Our series had a diverse etiological profile and patients with transition to adult care. Long-term follow-up showed that VNS is an effective and well-tolerated treatment modality for refractory childhood onset epilepsy. Age at implantation, duration of epilepsy and underlying etiology are not found to be predictors of responsiveness to VNS. Higher response rates were observed for a subset of patients with focal epileptiform discharges.

Cranial and ventricular size following shunting or endoscopic third ventriculostomy (ETV) in infants with aqueductal stenosis: further insights from the International Infant Hydrocephalus Study (IIHS).

PURPOSE: The craniometrics of head circumference (HC) and ventricular size are part of the clinical assessment of infants with hydrocephalus and are often utilized in conjunction with other clinical and radiological parameters to determine the success of treatment. We aimed to assess the effect of endoscopic third ventriculostomy (ETV) and shunting on craniometric measurements during the follow-up of a cohort of infants with symptomatic triventricular hydrocephalus secondary to aqueductal stenosis. METHODS: We performed a post hoc analysis of data from the International Infant Hydrocephalus Study (IIHS)-a prospective, multicenter study of infants (< 24 months old) with hydrocephalus from aqueductal stenosis who were treated with either an ETV or shunt. During various stages of a 5-year follow-up period, the following craniometrics were measured: HC, HC centile, HC z-score, and frontal-occipital horn ratio (FOR). Data were compared in an analysis of covariance, adjusting for baseline variables including age at surgery and sex. RESULTS: Of 158 enrolled patients, 115 underwent an ETV, while 43 received a shunt. Both procedures led to improvements in the mean HC centile position and z-score, a trend which continued until the 5-year assessment point. A similar trend was noted for FOR which was measured at 12 months and 3 years following initial treatment. Although the values were consistently higher for ETV compared with shunt, the differences in HC value, centile, and z-score were not significant. ETV was associated with a significantly higher FOR compared with shunting at 12 months (0.52 vs 0.44; p = 0.002) and 3 years (0.46 vs 0.38; p = 0.03) of follow-up. CONCLUSION: ETV and shunting led to improvements in HC centile, z-score, and FOR measurements during long-term follow-up of infants with hydrocephalus secondary to aqueductal stenosis. Head size did not significantly differ between the treatment groups during follow-up, however ventricle size was greater in those undergoing ETV when measured at 1 and 3 years following treatment.

Is the Knowledge Pertaining to Adult Glioblastomas Enough for Pediatric Cases? Prognostic Factors in Childhood.

AIM: Pediatric glioblastoma (GBM) is still a topic obscurity. The aim of this study was to explore clinical, radiological and pathological features, and prognostic factors affecting the outcomes. MATERIAL AND METHODS: We retrospectively reviewed our database for prognostic factors for 42 consecutive pediatric patients with histologically proven GBM treated in our hospital. RESULTS: The study reached at 20 boys and 22 girls, with a mean age of 10.2 years. Almost all patients (97.6%) had supratentorial tumors; lobar/hemispheric (68.3%), thalamic (26.8%) and suprasellar-hypothalamic region (4.8%). Total of 11/42 children had seeding metastases (mean 11.5 months) either preoperatively or postoperatively. Gross total resection (GTR) was achieved in 13 patients (30.9%) in the first surgery. Perioperative mortality and morbidity rates were 4.7% and 19%, respectively. Patients were followed for an average of 18.1 months. The median progression-free and overall survivals were 7.0 (95% CI: 5.9-8.0) and 11.0 (95% CI: 8.9-13.1) months, respectively. 1-year, 2-year and 5-year progression-free survival and overall survivals were 30.9% vs. 50.0%, 11.9% vs. 19.0%, 4.8% vs. 9.5%; respectively. CONCLUSION: Gross total resection should be safely attempted in pediatric GBM. In addition, a thorough and frequent radiological evaluation of the entire neuraxis for seeding metastases is recommended both at diagnosis and follow-ups.

The time course changes in expression of aquaporin 4 and aquaporin 1 following global cerebral ischemic edema in rat.

BACKGROUND: The aim of this global cerebral ischemia study was to study the changes in expression levels of aquaporin 4 (AQP4) and AQP1 over time. METHODS: Sprague-Dawley type male rats were divided into six groups. Sham group and ischemia/reperfusion were performed on five other groups using the four-vessel occlusion model. Reperfusion was done 30 min after the occlusion, and each group was tested at 1, 6, 12, 24, and 48 h for brain wet-dry weight ratio and AQP4 and AQP1 expression levels using immunohistochemistry. To prove ischemia development exists in both hippocampal neurons and epithelia of choroid plexus, hematoxylin, and eosin and  neuronal marker (NeuN) immune-staining have been applied to the sham experimental group at 48 h. AQP4 expression levels are also measured with western blotting. RESULTS: After ischemia/reperfusion it is observed that the decrease in brain water content between 12 and 24 h was statistically significant (P < 0.01). In parallel and based on immunohistochemical staining, AQP4 expression levels did not exhibit any statistically significant change. AQP4 levels did not show any statistically significant change in western blotting. AQP1 expression in choroid plexus epithelial cells decreased at the 12 and 24 h but increased in 48 h (P < 0.05). CONCLUSIONS: Lack of change in AQP4 expression levels is thought as its dual role in formation and elimination of ischemic brain edema. Decrease in AQP1 expression levels in 24 h can be explained with necrosis in choroid plexus after ischemia and the increase in 48 h mark can be related to recovery in choroid plexus.

High-grade glioma in children and adolescents: a single-center experience.

PURPOSE: The aim of this study was to report the outcome in children with high-grade astrocytoma outside the brain stem and spinal cord that were treated at a single center. MATERIALS AND METHODS: The study included 26 patients with anaplastic astrocytoma and 37 patients with glioblastoma; all patients were aged ≤18 years. At initial diagnosis, 18 of the patients with glioblastoma received only temozolomide (TMZ), 14 received other chemotherapies, and 5 did not receive any chemotherapy. Among the patients with anaplastic astrocytoma, 9 received TMZ, 9 received other chemotherapy regimens, and 8 patients did not receive any chemotherapy. The median radiotherapy dose in all patients was 60 Gy. RESULTS: Median age of the patients was 12.5 years. Median overall survival was 20 months and mean progression-free survival was 4.7-11.3 months (median: 8 months) in all patients. Patients with a Karnofsky performance score (KPS) ≥70 had median overall survival of 32 months, versus 7 months in those with a KPS < 70. Patients aged <15 years had median survival of 38 months, versus 16 months in those aged 15-18 years. Patients with anaplastic astrocytoma that received TMZ, other chemotherapy regimens, and no chemotherapy had median survival of 21 months, 132 months, and 11 months, respectively. Patients with glioblastoma that received TMZ, other chemotherapy regimens, and no chemotherapy had median survival of 32 months, 12 months, and 8 months, respectively. CONCLUSION: In the present study, patients with anaplastic astrocytoma treated with chemotherapy protocols other than TMZ had the longest OS; however, in the glioblastoma group, OS was 32 months in those treated with standard TMZ and 12 months in those treated with other protocols (P = 0.493). Although TMZ is less toxic than PCV, it was not shown to be superior.

The seizure semiology consistent with frontal lobe symptomatogenic zone in children.

The aim of this study is to analyze the seizure semiology consistent with frontal lobe symptomatogenic zone in childhood. We analyzed 549 videotaped seizures from 79 patients (mean age 9.9 ± 3.8 years). Magnetic resonance imaging was normal in 30 patients. The seizures in the time interval of 10 p.m. to 6 a.m. were considered as nocturnal. The mean number of seizures per patient was 6.8 ± 7.3. The mean seizure duration was 25.7 ± 26.9 sec; postictal confusion was 27 ± 16.1 sec (7-92 seconds). The seizures were observed in sleep with a rate of 56.8%; 43.1% of them were during wakefulness. Overall 50.4% of the seizures occured during night-time sleep. Tonic seizure (77.2%) was the most frequent simple motor seizure. Versive seizures were the second most frequent type of simple motor seizure (26.7%). Clonic seizures were 17.7%, complex motor seizures were 20.5%, and dialeptic seizures were 3% of all the seizures. Epileptic spasm, myoclonic seizures, aphasia, and akinetic semiologies were not observed. Vocalization was observed in 16% of the seizures. Frontal lobe seizures in childhood have a short duration, occur frequently, especially during night time sleep, and have a brief postictal period. Tonic semiology, versive semiology are the most frequent seizure semiologies; hypermotor and secondary generalized tonic clonic seizures and vocalizations are observed less in children compared to adults.

Enigmatic entity in childhood: clival chordoma from a tertiary center's perspective.

PURPOSE: Chordoma is a rare neoplasm that arises from embryonic notochordal remnants along the axial skeleton (i.e., clivus, sacrum) and the vertebral bodies. They comprise less than 1 % of CNS tumors and 1-4 % of all bone malignancies. It rarely affects children and adolescents (<5 %). Chordomas are locally aggressive and highly recurrent. Their management is challenging for clinicians. METHODS: This retrospective study includes six pediatric patients with pathological evidence of clival chordoma. These cases were identified over a period of 15 years in a tertiary care institute. RESULTS: There were two boy and four girls with a mean age of 10.6 years (range, 4-16 years). The chief complaint was due to cranial nerve palsy (or dysfunction), mostly affecting lower cranial nerves (66.6 %), followed by diplopia and headache. One patient had obstructive sleep apnea. All patients were operated and a total of 15 surgeries were performed (mean, 2.5). Tumor recurrence was observed in four patients (67 %). Two-year and 5-year progression-free survivals (PFS) were 67 and 33 %, respectively. None of the patients were lost either during the surgery or the follow-up period (6.9 years: 1-14 years). CONCLUSIONS: Clival chordomas are challenging tumors in neurosurgical practice. A multidisciplinary approach is warranted in each patient. Today, the best management strategy seems to be surgical resection followed by radiotherapy. Chemotherapy should be considered in selective and preference basis. Sharing institutional experiences will provide future insights in prognosis of these rare tumors. Implementing newer surgical instruments, endoscope in particular, is encouraged in management of the clival chordomas.