Treatment outcomes of single-fraction stereotactic radiosurgery for adenoid cystic carcinoma: a case series of 55 patients.

PURPOSE: This study aimed to analyze the treatment outcomes of single-fraction stereotactic radiosurgery (SRS) for adenoid cystic carcinoma patients. METHODS: Retrospective analysis was conducted for 55 patients with 66 lesions. SRS intentions were categorized as definitive, adjuvant, salvage, and palliative. Tumor control was defined as local (within 50% isodose line), marginal (outside 50% isodose line), and distant (metastasis outside head/neck). RESULTS: The median age was 60 years (range 21-85), with 53% males. Tumor origin was head/neck for 88% and trachea/lung for 12%. 61% were recurrent lesions. Median interval from diagnosis to SRS was 14 months. Preceding surgery was performed in 30%. SRS was administered as definitive (30 lesions), adjuvant (13), salvage (19), and palliative (4). SRS was used as a boost to external beam radiation therapy (EBRT) in 39%. Concurrent chemotherapy was administered in 26%. 5-, 10-, and 15-year local control rates were 60%, 33%, and 27%, respectively; local/marginal control rates were 29%, 13%, and 10%. For recurrent lesions treated with SRS without EBRT, 5-year local control rate was 14%, and local/marginal control rate was 5%. For recurrent lesions treated with SRS and EBRT, 5-year local control rate was 100%, and local/marginal control rate was 40%. The rate of distant failure after SRS was 40%. Older age and distant metastasis before SRS were negative factors for overall survival. CONCLUSION: SRS provided a high rate of local tumor control, but marginal failure was frequent. Integrating SRS with added EBRT exhibits potential for enhancing local and local/marginal tumor control, particularly in recurrent cases.

3D Models as a Source for Neuroanatomy Education: A Stepwise White Matter Dissection Using 3D Images and Photogrammetry Scans.

White matter dissection (WMD) involves isolating bundles of myelinated axons in the brain and serves to gain insights into brain function and neural mechanisms underlying neurological disorders. While effective, cadaveric brain dissections pose certain challenges mainly due to availability of resources. Technological advancements, such as photogrammetry, have the potential to overcome these limitations by creating detailed three-dimensional (3D) models for immersive learning experiences in neuroanatomy. This study aimed to provide a detailed step-by-step WMD captured using two-dimensional (2D) images and 3D models (via photogrammetry) to serve as a comprehensive guide for studying white matter tracts of the brain. One formalin-fixed brain specimen was utilized to perform the WMD. The brain was divided in a sagittal plane and both cerebral hemispheres were stored in a freezer at -20 °C for 10 days, then thawed under running water at room temperature. Micro-instruments under an operating microscope were used to perform a systematic lateral-to-medial and medial-to-lateral dissection, while 2D images were captured and 3D models were created through photogrammetry during each stage of the dissection. Dissection was performed with comprehensive examination of the location, main landmarks, connections, and functions of the white matter tracts of the brain. Furthermore, high-quality 3D models of the dissections were created and housed on SketchFab®, allowing for accessible and free of charge viewing for educational and research purposes. Our comprehensive dissection and 3D models have the potential to increase understanding of the intricate white matter anatomy and could provide an accessible platform for the teaching of neuroanatomy.

Long-term outcome of primary clival chordomas: a single-center retrospective study with an emphasis on the timing of recurrences based on the primary treatment.

OBJECTIVE: This study aimed to provide data on extended outcomes in primary clival chordomas, focusing on progression-free survival (PFS) and overall survival (OS). METHODS: A retrospective single-center analysis was conducted on patients with clival chordoma treated between 1987 and 2022 using surgery, stereotactic radiosurgery, or proton radiation therapy (PRT). RESULTS: The study included 100 patients (median age 44 years, 51% male). Surgery was performed using the endoscopic endonasal approach in 71 patients (71%). Gross-total resection (GTR) or near-total resection (NTR) was attained in 39 patients (39%). Postoperatively, new cranial nerve deficits occurred in 7%, CSF leak in 4%, and meningitis in none of the patients. Radiation therapy was performed in 79 patients (79%), with PRT in 50 patients (50%) as the primary treatment. During the median follow-up period of 73 (interquartile range [IQR] 38-132) months, 41 recurrences (41%) and 31 deaths (31%) were confirmed. Patients with GTR/NTR had a median PFS of 41 (IQR 24-70) months. Patients with subtotal resection or biopsy had a median PFS of 38 (IQR 16-97) months. The median PFS of patients who received radiation therapy was 43 (IQR 26-86) months, while that of patients who did not receive radiation therapy was 18 (IQR 5-62) months. The Kaplan-Meier method showed that patients with GTR/NTR (p = 0.007) and those who received radiation therapy (p < 0.001) had longer PFS than their counterparts. The PFS rates following primary treatment at 5, 10, 15, and 20 years were 51%, 25%, 17%, and 7%, respectively. The OS rates at the same intervals were 84%, 60%, 42%, and 34%, respectively. Multivariate Cox regression analysis showed that age < 44 years (p = 0.02), greater extent of resection (EOR; p = 0.03), and radiation therapy (p < 0.001) were associated with lower recurrence rates. Another multivariate analysis showed that age < 44 years (p = 0.01), greater EOR (p = 0.04), and freedom from recurrence (p = 0.02) were associated with lower mortality rates. Regarding pathology data, brachyury was positive in 98%, pan-cytokeratin in 93%, epithelial membrane antigen in 85%, and S100 in 74%. No immunohistochemical markers were associated with recurrence. CONCLUSIONS: In this study, younger age, maximal safe resection, and radiation therapy were important factors for longer PFS in patients with primary clival chordomas. Preventing recurrences played a crucial role in achieving longer OS.

Treatment modalities and outcomes of granular cell tumors and spindle cell oncocytomas of the pituitary gland: an analysis of two national cancer databases.

BACKGROUND: Spindle cell oncocytomas (SCO) and granular cell tumors (GCT) are rare primary pituitary neoplasms; the optimal treatment paradigms for these lesions are unknown and largely unexplored. Thus, using national registries, we analyze the epidemiology, management patterns, and surgical outcomes of SCOs and GCTs. METHODS: The National Cancer Database (NCDB; years 2003-2017) and the Surveillance, Epidemiology, and End Results Program (SEER; years 2004-2018) were queried for patients with pituitary SCOs or GCTs. Incidence, extent of surgical resection, and rate of postoperative radiation use for subtotally resected lesions comprised the primary outcomes of interest. All-cause mortality was also analyzed via time-to-event Kaplan-Meier curves. RESULTS: SCOs and GCTs have an annual incidence of 0.017 and 0.023 per 1,000,000, respectively. They comprise 0.1% of the benign pituitary tumors registered in NCDB. A total of 112,241 benign pituitary tumors were identified in NCDB during the study period, of which 83 (0.07%) were SCOs and 59 (0.05%) were GCTs. Median age at diagnosis was 55 years, 44% were females, and median maximal tumor diameter at presentation was 2.1 cm. Gross total resection was achieved in 54% patients. Ten patients (7%) had postoperative radiation. Comparing patients with GCTs versus SCOs, the former were more likely to be younger at diagnosis (48.0 vs. 59.0, respectively; p < 0.01) and female (59% vs. 34%, p = 0.01). GCTs and SCOs did not differ in terms of size at diagnoses (median maximal diameter: 1.9 cm vs. 2.2 cm, respectively; p = 0.59) or gross total resection rates (62% vs. 49%, p = 0.32). After matching SCOs and GCTs with pituitary adenomas on age, sex, and tumor size, the former were less likely to undergo gross total resection (53% vs. 72%; p = 0.03). Patients with SCOs and GCTs had a shorter overall survival when compared to patients with pituitary adenomas (p < 0.01) and a higher rate of thirty-day mortality (3.1% vs 0.0%; p = 0.013). CONCLUSION: SCOs and GCTs are rare pituitary tumors, and their management entails particular challenges. Gross total resection is often not possible, and adjuvant radiation might be employed following subtotal resection.

That which is unseen: 3D printing for pediatric cerebrovascular education.

INTRODUCTION: Pediatric cerebrovascular lesions are very rare and include aneurysms, arteriovenous malformations (AVM), and vein of Galen malformations (VOGM). OBJECTIVE: To describe and disseminate a validated, reproducible set of 3D models for optimization of neurosurgical training with respect to pediatric cerebrovascular diseases METHODS: All pediatric cerebrovascular lesions treated at our institution with adequate imaging studies during the study period 2015-2020 were reviewed by the study team. Three major diagnostic groups were identified: aneurysm, AVM, and VOGM. For each group, a case deemed highly illustrative of the core diagnostic and therapeutic principles was selected by the lead and senior investigators for printing (CSG/JM). Files for model reproduction and free distribution were prepared for inclusion as Supplemental Materials. RESULTS: Representative cases included a 7-month-old female with a giant left MCA aneurysm; a 3-day-old male with a large, complex, high-flow, choroidal-type VOGM, supplied from bilateral thalamic, choroidal, and pericallosal perforators, with drainage into a large prosencephalic vein; and a 7-year-old male with a left frontal AVM with one feeding arterial vessel from the anterior cerebral artery and one single draining vein into the superior sagittal sinus CONCLUSION: Pediatric cerebrovascular lesions are representative of rare but important neurosurgical diseases that require creative approaches for training optimization. As these lesions are quite rare, 3D-printed models and open source educational materials may provide a meaningful avenue for impactful clinical teaching with respect to a wide swath of uncommon or unusual neurosurgical diseases.

Immunohistochemical markers predicting long-term recurrence following clival and spinal chordoma resection: a multicenter study.

OBJECTIVE: Chordomas are rare tumors from notochordal remnants and account for 1%-4% of all primary bone malignancies, often arising from the clivus and sacrum. Despite margin-negative resection and postoperative radiotherapy, chordomas often recur. Further, immunohistochemical (IHC) markers have not been assessed as predictive of chordoma recurrence. The authors aimed to identify the IHC markers that are predictive of postoperative long-term (≥ 1 year) chordoma recurrence by using trained multiple tree-based machine learning (ML) algorithms. METHODS: The authors reviewed the records of patients who had undergone treatment for clival and spinal chordomas between January 2017 and June 2021 across the Mayo Clinic enterprise (Minnesota, Florida, and Arizona). Demographics, type of treatment, histopathology, and other relevant clinical factors were abstracted from each patient record. Decision tree and random forest classifiers were trained and tested to predict long-term recurrence based on unseen data using an 80/20 split. RESULTS: One hundred fifty-one patients diagnosed and treated for chordomas were identified: 58 chordomas of the clivus, 48 chordomas of the mobile spine, and 45 chordomas sacrococcygeal in origin. Patients diagnosed with cervical chordomas were the oldest among all groups (58 ± 14 years, p = 0.009). Most patients were male (n = 91, 60.3%) and White (n = 139, 92.1%). Most patients underwent resection with or without radiation therapy (n = 129, 85.4%). Subtotal resection followed by radiation therapy (n = 51, 33.8%) was the most common treatment modality, followed by gross-total resection then radiation therapy (n = 43, 28.5%). Multivariate analysis showed that S100 and pan-cytokeratin are more likely to predict the increase in the risk of postoperative recurrence (OR 3.67, 95% CI 1.09-12.42, p= 0.03; and OR 3.74, 95% CI 0.05-2.21, p = 0.02, respectively). In the decision tree analysis, a clinical follow-up > 1897 days was found in 37% of encounters and a 90% chance of being classified for recurrence (accuracy = 77%). Random forest analysis (n = 500 trees) showed that patient age, type of surgical treatment, location of tumor, S100, pan-cytokeratin, and EMA are the factors predicting long-term recurrence. CONCLUSIONS: The IHC and clinicopathological variables combined with tree-based ML tools successfully demonstrated a high capacity to identify recurrence patterns with an accuracy of 77%. S100, pan-cytokeratin, and EMA were the IHC drivers of recurrence. This shows the power of ML algorithms in analyzing and predicting outcomes of rare conditions of a small sample size.

The "candy wrapper" of the pituitary gland: a road map to the parasellar ligaments and the medial wall of the cavernous sinus.

PURPOSE: The anatomy of the medial wall of the cavernous sinus (MWCS) and parasellar ligaments (PLs) has acquired increasing importance in endoscopic endonasal (EE) surgery of the cavernous sinus (CS), including resection of the MWCS in functioning pituitary adenomas (FPAs). Although anatomical studies have been published, it represents a debated topic due to their complex morphology. The aim is to offer a description of the PLs that originate from the MWCS and reach the lateral wall of the cavernous sinus (LWCS), proposing the "candy wrapper" model. The relationships between the neurovascular structures and histomorphological aspects were investigated. METHODS: Forty-two CSs from twenty-one human heads were studied. Eleven specimens were used for EE dissection; five underwent a microscopic dissection. Five specimens were used for histomorphological analysis. RESULTS: Two groups of PLs with a fan-shaped appearance were encountered. The anterior group included the periosteal ligament (55% sides) and the carotico-clinoid complex (100% sides), formed by the anterior horizontal and the carotico-clinoid ligaments. The posterior group was formed by the posterior horizontal (78% sides), and the inferior hypophyseal ligament (34% sides). The periosteal ligament originated inferiorly from the MWCS, reaching the periosteal dura. The anterior horizontal ligament was divided in a superior and inferior branch. The superior one continued as the carotid-oculomotor membrane, and the inferior branch reached the CN VI. The carotico-clinoid ligament between the middle and anterior clinoid was ossified in 3 sides. The posterior horizontal ligament was related to the posterior genu and ended at the LWCS. The inferior hypophyseal ligament followed the homonym artery. The ligaments related to the ICA form part of the adventitia. CONCLUSION: The "candy wrapper" model adds further details to the previous descriptions of the PLs. Understanding this complex anatomy is essential for safe CS surgery, including MWCS resection for FPAs.

Anatomical step-by-step dissection of common approaches to the third ventricle for trainees: surgical anatomy of the anterior transcortical and interhemispheric transcallosal approaches, surgical principles, and illustrative pediatric cases.

PURPOSE: To create a high-quality, cadaver-based, operatively oriented resource documenting the anterior transcortical and interhemispheric transcallosal approaches as corridors to the third ventricle targeted towards neurosurgical trainees at all levels. METHODS: Two formalin-fixed, latex-injected specimens were dissected under microscopic magnification and endoscopic-assisted visualization. Dissections of the transcortical and transcallosal craniotomies with transforaminal, transchoroidal, and interforniceal transventricular approaches were performed. The dissections were documented in a stepwise fashion using three-dimensional photographic image acquisition techniques and supplemented with representative cases to highlight pertinent surgical principles. RESULTS: The anterior transcortical and interhemispheric corridors afford excellent access to the anterior two-thirds of the third ventricle with varying risks associated with frontal lobe versus corpus callosum disruption, respectively. The transcortical approach offers a more direct, oblique view of the ipsilateral lateral ventricle, whereas the transcallosal approach readily establishes biventricular access through a paramedian corridor. Once inside the lateral ventricle, intraventricular angled endoscopy further enhances access to the extreme poles of the third ventricle from either open transcranial approach. Subsequent selection of either the transforaminal, transchoroidal, or interforniceal routes can be performed through either craniotomy and is ultimately dependent on individual deep venous anatomy, the epicenter of ventricular pathology, and the concomitant presence of hydrocephalus or embryologic cava. Key steps described include positioning and skin incision; scalp dissection; craniotomy flap elevation; durotomy; transcortical versus interhemispheric dissection with callosotomy; the aforementioned transventricular routes; and their relevant intraventricular landmarks. CONCLUSIONS: Approaches to the ventricular system for maximal safe resection of pediatric brain tumors are challenging to master yet represent foundational cranial surgical techniques. We present a comprehensive operatively oriented guide for neurosurgery residents that combines stepwise open and endoscopic cadaveric dissections with representative case studies to optimize familiarity with third ventricle approaches, mastery of relevant microsurgical anatomy, and preparation for operating room participation.

The role of bevacizumab for treatment-refractory intracranial meningiomas: a single institution's experience and a systematic review of the literature.

INTRODUCTION: Meningiomas account for over 30% of all primary brain tumors. While surgery can be curative for these tumors, several factors may lead to a higher likelihood of recurrence. For recurrent meningiomas, bevacizumab may be considered as a therapeutic agent, but literature regarding its efficacy is sparse. Thus, we present a systematic review of the literature and case series of patients from our institution with treatment-refractory meningiomas who received bevacizumab. METHODS: Patients at our institution who were diagnosed with recurrent meningioma between January 2000 and September 2020 and received bevacizumab monotherapy were included in this study. Bevacizumab duration and dosages were noted, as well as progression-free survival (PFS) after the first bevacizumab injection. A systematic review of the literature was also performed. RESULTS: Twenty-three patients at our institution with a median age of 55 years at initial diagnosis qualified for this study. When bevacizumab was administered, 2 patients had WHO grade I meningiomas, 10 patients had WHO grade II meningiomas, and 11 patients had WHO grade III meningiomas. Median PFS after the first bevacizumab injection was 7 months. Progression-free survival rate at 6 months was 57%. Two patients stopped bevacizumab due to hypertension and aphasia. Systematic review of the literature showed limited ability for bevacizumab to control tumor growth. CONCLUSION: Bevacizumab is administered to patients with treatment-refractory meningiomas and, though its effectiveness is limited, outperforms other systemic therapies reported in the literature. Further studies are required to identify a successful patient profile for utilization of bevacizumab.

Jugular foramen capillary hemangioma masquerading as a paraganglioma.

Capillary hemangiomas are benign vascular lesions that are common in head and neck, but hemangiomas of jugular foramen and temporal bone are rare with only a few cases reported in the literature. We present a case report of this rare disease entity highlighting the subtle radiographic nuances that can benefit clinicians when encountered with similar unusual clinical scenario. Although radiographic features of capillary hemangioma can be distinctive, they are not specific. In this case, the lack of significant involvement of the jugular bulb and the absence of the typical pattern of osseous erosion of the jugular carotid spine led to the alternative diagnosis of a rare capillary hemangioma of the jugular foramen.

The role of single-fraction stereotactic radiosurgery for atypical meningiomas (WHO grade II): treatment results based on a 25-year experience.

PURPOSE: To clarify the role of stereotactic radiosurgery (SRS) for atypical meningiomas (AM). METHODS: A retrospective analysis of 68 patients with AM having SRS from 1995 until 2019. RESULTS: Nineteen patients (28%) had undergone prior external beam radiation therapy (EBRT) (median dose, 54 Gy). The median follow-up period was 52 months. Eighteen (26%), 17 (25%), and 33 (49%) patients received SRS as an upfront adjuvant (≤ 6 months), early salvage (7-18 months), or late salvage treatment (> 18 months), respectively. The 3-, 5-, and 10-year progression-free survivals (PFSs) were 52%, 35%, and 25%, respectively. The 3-, 5-, and 10-year disease-specific survivals were 85%, 78%, and 61%, respectively. Adverse radiation events (AREs) were observed in 12 patients (18%), with increased or new seizures being the most frequent complication (n = 7). Prior EBRT was associated with reduced PFS (HR 5.92, P < 0.01), reduced DSS (HR 5.84, P < 0.01), and an increased risk of ARE (HR 3.31, P = 0.04). Timing of SRS was correlated with reduced PFS for patients having early salvage treatment compared to upfront adjuvant (HR 3.17, P = 0.01) or late salvage treatment (HR 4.39, P < 0.01). CONCLUSION: PFS for patients with residual/recurrent AM remains poor despite SRS. Prior EBRT was associated with worse tumor control, higher tumor-related mortality, and an increased risk of ARE. Further study on the timing of SRS is needed to determine if upfront adjunctive SRS improves tumor control compared to salvage SRS.

Long-term outcomes of grade I/II skull base chondrosarcoma: an insight into the role of surgery and upfront radiotherapy.

PURPOSE: To clarify the need for post-operative radiation treatment in skull base chondrosarcomas (SBCs). METHODS: A retrospective analysis of patients with grade I or II SBC. Patients were divided according to post-surgical treatment strategies: (A) planned upfront radiotherapy and (B) watchful waiting. Tumor control and survival were compared between the treatment groups. The median follow-up after resection was 105 months (range, 9-376). RESULTS: Thirty-two patients (Grade 1, n = 16; Grade 2, n = 16) were included. The most frequent location was petroclival (21, 64%). A gross total resection (GTR) was achieved in 11 patients (34%). Fourteen (44%) underwent upfront radiotherapy (group A) whereas 18 (56%) were followed with serial MRI alone (group B). The tumor control rate for the entire group was 77% and 69% at 10- and 15-year, respectively. Upfront radiotherapy (P = 0.25), extent of resection (P = 0.11) or tumor grade (P = 0.83) did not affect tumor control. The majority of Group B patients with recurrent tumors (5/7) obtained tumor control with repeat resection (n = 2), salvage radiotherapy (n = 2), or a combination of both (n = 1). The 10-year disease-specific survival was 95% with no difference between the group A and B (P = 0.50). CONCLUSION: For patients with grade I/II SBC, a reasonable strategy is deferral of radiotherapy after maximum safe resection until tumor progression or recurrence. At that time, most patients can be successfully managed with salvage radiotherapy or surgery. Late recurrences may occur, and life-long follow-up is advisable.

Salvage Radiosurgery for Recurrent Supratentorial Primitive Neuroectodermal Tumors: A Single Institutional Series and Review of the Literature.

INTRODUCTION: Supratentorial primitive neuroectodermal tumor is a rare, aggressive intrinsic brain tumor with limited treatment options for recurrent disease. SRS as a treatment modality in the recurrent setting was investigated. METHODS: A retrospective review of 8 patients treated with SRS for local or distant recurrence of supratentorial PNET from 1999 to 2014 was conducted. RESULTS: Thirty-six tumors were treated in 15 sessions in 8 patients. The median patient age was 22.5 (interquartile range [IQR], 14.75-43.5 years) with a median 21-month period from diagnosis until SRS (IQR, 16-23.75 months). The median prescription isodose volume was 1.85 cm3 (IQR, 1.85-7.02 cm3); median tumor margin dose was 18 Gy (IQR 14-20 Gy); and median isocenters was 2 (range 1-13). No patients experienced adverse radiation effects. All but 1 patient died, and the median overall survival was 32 months (IQR, 26.75-53.5 months) with median overall survival following SRS of 9.5 months (IQR, 5.25-30 months). Univariate analysis failed to demonstrate a statistically significant association between age, number of gamma knife treatments, interval to gamma knife, and margin radiation dose with overall survival. DISCUSSION/CONCLUSION: This series supports the use of SRS in patients with recurrent supratentorial PNET following multimodal therapy.

Large and small vestibular schwannomas: same, yet different tumors.

BACKGROUND: Vestibular schwannomas (VS) present at variable size with heterogeneous symptomatology. Modern treatment paradigms for large VS include gross total resection, subtotal resection (STR) in combination with observation, and/or radiation to achieve optimal function preservation, whereas treatment is felt to be both easier and safer for small VS. The objective is to better characterize the presentation and surgical outcomes of large and small VS. METHODS: We collected data of patients who had surgically treated VS with a posterior fossa diameter of 4.0 cm or larger (large tumor group, LTG) and smaller than 1.0 cm in cisternal diameter (small tumor group, STG). Statistical significance was defined as p < 0.05. RESULTS: LTG included 48 patients (average tumor size: 44.9 mm) and STG 38 (7.9 mm). Patients in STG presented more frequently with tinnitus and sudden hearing loss. Patients in LTG underwent more STR than STG (50.0% vs. 2.6%, p < 0.0001). LTG had more complications (31.3% vs. 13.2%, p = 0.049). Postoperative facial nerve function in STG was significantly better than LTG. STG had better hearing preoperatively (p < 0.0001) and postoperatively than LTG (p = 0.0002). Postoperative headache was more common in STG (13.2% vs. 2.1%, p = 0.045). The rate of recurrence/progression needing treatment was not statistically different between the groups (12.5% in LTG vs. 7.9% in STG, p = 0.49). Those patients who required periprocedural cerebrospinal fluid diversion had higher risk of infection (20.8% vs 4.8%, p = 0.022). CONCLUSION: Large and small VS present differently. LTG showed more unsatisfactory outcomes in facial nerve function and postoperative hearing despite maximal efforts undertaken toward function-preservation strategy; however, similar tumor control was achieved.

Degree of preoperative hearing loss predicts time to early mobilization following vestibular schwannoma microsurgery.

OBJECTIVE: To test the hypothesis that severe to profound preoperative hearing loss predicts less acute postoperative vestibulopathy following microsurgical removal of vestibular schwannoma (VS) allowing for earlier postoperative mobilization and hospital discharge. METHODS: Patients with VS who underwent microsurgery and were found to have preoperative severe to profound hearing loss (pure tone average [PTA] > 70 dB HL) were matched 1:1 by age and tumor size to a group of randomly selected controls with preoperative serviceable hearing. RESULTS: A total of 57 patients met inclusion criteria and were matched to controls. Median age at the time of microsurgery was 56 years. The median PTA and WRS for cases were 91 dB HL (interquartile range [IQR] 78-120) and 0% (IQR 0-0), respectively. Median tumor size was 14.2 mm (IQR 10.9-20.9). A total of 35 (61%) patients exhibited nystagmus after surgery associated with acute vestibular deafferentation. Median time to ambulation in the hallway was 2 days. Controls exhibited similar tumor size (12.7 mm, p = 0.11) and age (57 years, p = 0.52). Preoperative hearing loss did not predict severity or duration of postoperative nystagmus or days to discharge; however, those with Class D hearing exhibited a shorter time to ambulation (p = 0.04). CONCLUSION: Following microsurgical removal of VS, preoperative profound hearing loss was associated with a shorter time to postoperative mobilization; however, there were no observed associations with duration or severity of nystagmus and time to hospital discharge. Although not a predictor of nystagmus, preoperative profound hearing loss may portend quicker recovery from clinically significant postoperative vestibulopathy.

Early Recurrence of an Infantile Endodermal Oculomotor Nerve Cyst following Surgical Fenestration: A Case Report.

INTRODUCTION: Infantile endodermal oculomotor nerve cyst (EONC) is an extremely rare entity. There are very few pediatric cases reported in the literature, and as expected, oculomotor palsy is the most common presenting symptom. To date however, the risk of recurrence of these lesions following surgical intervention is unclear due to a lack of long-term radiological follow-up. CASE PRESENTATION: We present a case of a 13-month-old male patient with an EONC and detail his surgical fenestration and postoperative course. Somewhat surprisingly, re-expansion occurred within 6 months and remained stable 2 years later. DISCUSSION: A surgical approach to fenestration of an EONC in an infant is possible and should be performed by an expert neurosurgeon. Early recurrence is underreported in the current literature, and we encourage longer term radiological surveillance of these lesions after surgery to optimize primary and recurrent management in the future.

Detection of Asymptomatic Radiation Induced Optic Neuropathy with Optical Coherence Tomography.

Radiation-induced optic neuropathy is a rare complication of radiation therapy that often results in profound, irreversible vision loss. We present a unique case of a patient in whom optical coherence tomography detected an early underlying optic neuropathy despite being visually asymptomatic in the affected eye.

Anterior interhemispheric approach for clipping of subcallosal distal anterior cerebral artery aneurysms: case series and technical notes.

Distal anterior cerebral artery (DACA) aneurysms are rare, accounting for 1-9% of all intracranial aneurysms. Previous systematic reviews have highlighted that given the markedly increased incidence of major complications after endovascular treatment, microsurgical clipping is the more attractive treatment option with generally excellent clinical outcomes. Subcallosal DACA aneurysms constitute a rare subset of these aneurysms, requiring special anatomic considerations-particularly with regard to the approach. The aim of this study is to review the technical nuances of microsurgical treatment of subcallosal DACA aneurysms, including review of contemporary techniques through presentation of a microneurosurgical operative video. This is a retrospective case series and intraoperative microsurgical videos review. Three subcallosal DACA aneurysms were identified via retrospective query of our institutional neurosurgical database from December 2017 to May 2018. Two were female; median age was 74 years (range 70-83); all 3 underwent bifrontal craniotomy via bicoronal skin incision for aneurysm clipping. Aneurysms were located in left pericallosal-callosomarginal artery junction, bifurcation of azygos A2, and pericallosal artery related with azygos A2, and the anterior interhemispheric approach was used in all 3 operations. No acute stroke, hemorrhage, or major complications occurred, and all patients remained neurologically intact at the time of last follow-up (median 3 months, range 1-6). Although DACA aneurysms are rare, they represent an important variant for cerebrovascular neurosurgeons where microsurgical clipping can have better angiographic outcomes than endovascular treatment. Detail-oriented anterior interhemispheric arachnoid dissection through bifrontal craniotomy with its lower margin sitting at the superior orbital rim maximizes safe and effective clipping of subcallosal DACA aneurysms.

Surgical management for large vestibular schwannomas: a systematic review, meta-analysis, and consensus statement on behalf of the EANS skull base section.

BACKGROUND AND OBJECTIVE: The optimal management of large vestibular schwannomas continues to be debated. We constituted a task force comprising the members of the EANS skull base committee along with international experts to derive recommendations for the management of this problem from a European perspective. MATERIAL AND METHODS: A systematic review of MEDLINE database, in compliance with the PRISMA guidelines, was performed. A subgroup analysis screening all surgical series published within the last 20 years (January 2000 to March 2020) was performed. Weighted summary rates for tumor resection, oncological control, and facial nerve preservation were determined using meta-analysis models. This data along with contemporary practice patterns were discussed within the task force to generate consensual recommendations regarding preoperative evaluations, optimal surgical strategy, and follow-up management. RESULTS: Tumor classification grades should be systematically used in the perioperative management of patients, with large vestibular schwannomas (VS) defined as > 30 mm in the largest extrameatal diameter. Grading scales for pre- and postoperative hearing (AAO-HNS or GR) and facial nerve function (HB) are to be used for reporting functional outcome. There is a lack of consensus to support the superiority of any surgical strategy with respect to extent of resection and use of adjuvant radiosurgery. Intraoperative neuromonitoring needs to be routinely used to preserve neural function. Recommendations for postoperative clinico-radiological evaluations have been elucidated based on the surgical strategy employed. CONCLUSION: The main goal of management of large vestibular schwannomas should focus on maintaining/improving quality of life (QoL), making every attempt at facial/cochlear nerve functional preservation while ensuring optimal oncological control, thereby allowing to meet patient expectations. Despite the fact that this analysis yielded only a few Class B evidences and mostly expert opinions, it will guide practitioners to manage these patients and form the basis for future clinical trials.

Regional variation in vestibular schwannoma management and patient support group participation across the United States.

PURPOSE: The Acoustic Neuroma Association (ANA) represents the largest existing patient support organization for those diagnosed with vestibular schwannoma (VS) in the United States. Yet, the degree to which the ANA is actually utilized across the country is unknown. Moreover, evidence suggests that there may exist significant regional variation in management practices of VS across the United States. MATERIALS AND METHODS: Patient participation in the ANA by state and geographical region, as well as their management approach, was reviewed through a cross-sectional survey performed from February 2017 through January 2019, patients diagnosed with sporadic VS. RESULTS: Among 878 patients studied, the District of Columbia had the largest proportion of ANA patients relative to state population (0.85 per 100,000 persons), followed by New Hampshire (0.74), Maine (0.60), and New Jersey (0.42). Comparatively, Mississippi (0.03), Hawaii (0.07), and Rhode Island (0.09) harbored significantly lower participation rates (p = 0.001). Significant treatment variations were observed across the United States: in Maine, Iowa, Missouri, Kansas, and New Hampshire, an average of 73% (range, 70-75) of patients underwent microsurgery, whereas only 24% (range, 0-35) of patients in Colorado, New York, Massachusetts, Connecticut, and West Virginia underwent microsurgery (p < 0.001). After controlling for patient age and tumor size, patients were significantly more likely to undergo a retrosigmoid approach than the translabyrinthine approach for medium- to large-sized tumors in the Northeast (OR = 4.18; p = 0.001) and Western United States (OR = 2.94; p = 0.009). CONCLUSIONS: Large regional variation exists surrounding patient participation in the ANA as well as management practices across the United States.