Faculty Bibliography

BACKGROUND: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 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. CONCLUSION/CONCLUSIONS: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.

BACKGROUND: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/CONCLUSIONS: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.

Sphenoorbital meningiomas are a challenge to access and reconstruct. Although there is much neurosurgical literature on resection of such tumors, there is little discussion on the best methods for the reconstruction of consequent defects, which are often extensive due to large areas of hyperostosis requiring resection. We performed a retrospective analysis of patients who underwent resection and reconstruction of a sphenoorbital meningioma by the senior authors (C.S. and D.A.S.) between 2010 and 2020. Surgical access in all cases included an orbitozygomatic osteotomy. The study cohort consisted of 23 patients (20 female, 3 male) with an average age of 50 (range: 37-72) years at the time of surgery. Most patients had progressive proptosis before the ablative operation. Orbital reconstruction was with a combined titanium-Medpor implant in 18 patients, split calvarial bone graft in 3 patients, and a Medpor implant in 2 patients. Calvarial reconstruction was performed with titanium mesh in 21 patients, split calvarial bone graft and titanium mesh in 1 patient, and craniotomy bone and titanium plate in 1 patient. Reoperation was required in 7 patients due to hypoglobus or enophthalmos (N=2), orbital implant malposition (N=1), abscess (N=1), pain (N=1), intracranial fat graft modification (N=1), and soft tissue deformities (N=2). Our experience demonstrates that sphenoorbital meningiomas can require broad areas of resection of the skull base and calvarium and necessitate comprehensive reconstruction of the anterior cranial fossa, orbital walls, and cranium. Collaboration between craniofacial surgeons and neurosurgeons can achieve optimal results.

BACKGROUND/UNASSIGNED:Central nervous system (CNS) cancer is the 10th leading cause of cancer-associated deaths for adults, but the leading cause in pediatric patients and young adults. The variety and complexity of histologic subtypes can lead to diagnostic errors. DNA methylation is an epigenetic modification that provides a tumor type-specific signature that can be used for diagnosis. METHODS/UNASSIGNED:We performed a prospective study using DNA methylation analysis as a primary diagnostic method for 1921 brain tumors. All tumors received a pathology diagnosis and profiling by whole genome DNA methylation, followed by next-generation DNA and RNA sequencing. Results were stratified by concordance between DNA methylation and histopathology, establishing diagnostic utility. RESULTS/UNASSIGNED:Of the 1602 cases with a World Health Organization histologic diagnosis, DNA methylation identified a diagnostic mismatch in 225 cases (14%), 78 cases (5%) did not classify with any class, and in an additional 110 (7%) cases DNA methylation confirmed the diagnosis and provided prognostic information. Of 319 cases carrying 195 different descriptive histologic diagnoses, DNA methylation provided a definitive diagnosis in 273 (86%) cases, separated them into 55 methylation classes, and changed the grading in 58 (18%) cases. CONCLUSIONS/UNASSIGNED:DNA methylation analysis is a robust method to diagnose primary CNS tumors, improving diagnostic accuracy, decreasing diagnostic errors and inconclusive diagnoses, and providing prognostic subclassification. This study provides a framework for inclusion of DNA methylation profiling as a primary molecular diagnostic test into professional guidelines for CNS tumors. The benefits include increased diagnostic accuracy, improved patient management, and refinements in clinical trial design.

OBJECTIVE:A) To describe an improved methodology for continuously monitoring the functional integrity of facial nerve by eliciting facial corticobulbar motor-evoked potentials (FCoMEP) and B) To establish the prognosis of facial nerve function based on changes in FCoMEP during skull base surgery. METHODS:Intraoperative monitoring of FCoMEP performed in 100 patients. Previously published methodology has been improved upon by a) doing preoperative mapping of the facial nerve, b) facilitating the corticobulbar tract (CBT) by continuous transcranial electrical stimulation (TES) at 2 Hz repetition rate, c) recording from multiple facial nerve innervated muscles, and d) eliciting blink reflex (BR). We analyzed changes in FCoMEP, comparing them with the clinical facial nerve outcome scored with the House-Brackman (HB) scale. RESULTS:The monitorability rate was 100%. Out of 100 patients, nine presented a new facial deficit after surgery. Eight of these showed significant changes in FCoMEP. In four patients FCoMEPs were lost; they presented a complete facial paralysis from which they did not recover. To discriminate the prognosis of patients, ROC analysis identified a cut-off at 65% for FCoMEPs amplitude decrease with a sensitivity of 89% and specificity of 99%. In four patients FCoMEP showed a decrease in amplitude greater than 65%, and they presented mild/moderate facial paresis that was transient. One patient did not present changes in FCoMEP but had a mild facial paresis from which the patient recovered. CONCLUSIONS:The improved methodology allows the maximum rate of monitorability and minimizes false positive and false negative results. This study shows that prognosis of facial nerve may be reliably established based on FCoMEP parameters. SIGNIFICANCE/CONCLUSIONS:We improved the previously described methodology for continuously monitoring the functional integrity of the facial nerve by increasing the monitorability rate, and we describe the impact of FCoMEP intraoperative management of facial nerve. This method may permit establishing the short-term and long-term prognosis of facial nerve function in skull base surgery.

BACKGROUND AND PURPOSE/OBJECTIVE:Skull base tumors are commonly supplied by dural branches of the meningohypophyseal and inferolateral trunks. Embolization through these arteries is often avoided due to technical challenges and inherent risks; however, successful embolization can be a valuable surgical adjunct. We aimed to review the success and complications in our series of tumor embolizations through the meningohypophyseal and inferolateral trunks. MATERIALS AND METHODS/METHODS:We performed a retrospective review of patients with tumor treated with preoperative embolization at our institution between 2010 and 2020. We reviewed the following data: patients' demographics, tumor characteristics, endovascular embolization variables, and surgical results including estimated blood loss, the need for transfusion, and operative time. RESULTS:= 4) trunk. In this group of patients, on average, 79% of tumors were embolized. No mortality or morbidity from the embolization procedure was observed in this subgroup of patients. The average estimated blood loss in the operation was 395 mL (range, 200-750 mL). None of the patients required a transfusion, and the average operative time was 7.3 hours. CONCLUSIONS:Some skull base tumors necessitate embolization through ICA branches such as the meningohypophyseal and inferolateral trunks. Our series demonstrates that an effective and safe embolization may be performed through these routes.

Soon after the World Health Organization declared the severe acute respiratory syndrome coronavirus 2 a global health emergency on January 30, 2020, New York City was plagued by the virus and its health system and economy pushed to their limits. The majority of the limited neurosurgical data in relation to COVID-19 is anecdotal and the higher theoretical risk of transmission of the virus among skull base aerosol generating (SBAG) cases has not been investigated or discussed in a neurosurgical population. We discuss a series of 13 patients who underwent 15 SBAG surgical procedures during the peak of COVID-19 in our hospital system and the protocols use perioperatively for their procedures. Our data support that with proper preoperative testing, a well-delineated surgical algorithm, and appropriate personal protective equipment, emergent/urgent cases can be done safely in hospitals that are currently experiencing high volumes of COVID-19 cases as we did in March to May of 2020.

The prognostic value of mutations in G-protein genes GNAQ and GNA11 in patients with intracranial and orbital melanocytomas is unknown. The authors present a case of GNA11 mutation (GNA11Q209L) in a 32-year-old male suffering from a meningeal melanocytoma with orbital involvement and ipsilateral Nevus of Ota. The patient underwent gamma knife stereotactic radiosurgery without biopsy and later partial transcranial resection of the melanocytic tumor that was subject to immunohistochemical and molecular analysis. A 50-gene next-generation sequencing panel revealed a 626A>T mutation in the GNA11 gene. One year later, intracranial extension of the melanocytoma necessitated a ventriculoperitoneal shunt and immunotherapy. Future work is needed to determine how GNA11 mutations in melanocytomas influence prognosis and monitoring strategies.