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Objective: The aim of this study was to evaluate the efficacy and safety of transorbital neuroendoscopic surgery (TONES) in the management of sphenoid wing meningiomas (SWMs) with cavernous sinus and orbital invasion., Methods: The authors conducted a retrospective review of 32 patients with SWMs treated at Gazi University using TONES from October 2019 to May 2023. The study includes clinical applications to elucidate the endoscopic transorbital approach. Surgical techniques focused on safe subtotal resection, aiming to minimize residual tumor volume for subsequent radiosurgery. Data were collected on patient demographics, tumor characteristics, surgical procedures, complications, and postoperative outcomes, including radiological imaging and ophthalmological evaluations., Results: Surgical dissections delineated a three-phase endoscopic transorbital approach: extraorbital, intraorbital, and intracranial. In the clinical application, gross-total resection was not achieved in any patient because of planned postoperative Gamma Knife radiosurgery. The mean follow-up period was 16.3 months. Of 30 patients with preoperative proptosis, 25 experienced postoperative improvement. No new-onset extraocular muscle paresis or visual loss occurred postoperatively. The average hospital stay was 1.15 days, with minimal complications and no significant morbidity or mortality., Conclusions: Total resection of SWMs invading the cavernous sinus and orbit is associated with substantial risks, particularly cranial nerve deficits. TONES offers a minimally invasive alternative, reducing morbidity compared with transcranial approaches, and represents a significant advancement in the surgical management of SWMs, especially those extending into the cavernous sinus and orbit. The approach provides a safe, effective, and patient-centric approach, prioritizing subtotal resection to minimize neurological deficits while preparing patients for adjunctive radiosurgery. This study positions TONES as a transformative surgical technique, aligning therapeutic efficacy with neurovascular preservation and postoperative recovery.

Objective: CD44 is a major cell surface receptor involved in cell adhesion and migration. The overexpression of CD44 is a poor prognostic factor in many neoplasms, including meningiomas. The aim of this study was to investigate the association between CD44 gene expression and clinical signatures of primary meningiomas., Methods: CD44 gene expression was quantitatively evaluated by snap freezing tumor tissues obtained from 106 patients with primary meningioma. The relationships between CD44 expression and clinical signatures of meningiomas, including histological malignancy, tumor volume, and peritumoral brain edema (PTBE), were analyzed. PTBE was assessed using the Steinhoff classification (SC) system (from SC 0 to SC III)., Results: CD44 gene expression in WHO grade 2 and 3 meningiomas was significantly higher than that in grade 1 meningiomas. In addition, CD44 expression increased with the severity of PTBE. Particularly, among the grade 1 meningiomas or small-sized tumors (maximum tumor diameter < 43 mm), CD44 expression in tumors with severe PTBE (SC II or III) was significantly higher than that in tumors without or with mild PTBE (SC 0 or I). Multivariate logistic regression analysis also revealed that overexpression of CD44 was an independent significant factor of severe PTBE development in primary meningiomas., Conclusions: In addition to tumor cell aggressiveness, CD44 expression promotes the development of PTBE in meningioma. Since PTBE is a strong factor of tumor-related epilepsy or cognitive dysfunction in patients with meningioma, CD44 is thus a potential therapeutic target in meningioma with PTBE.

Objective: Tuberculum sellae meningioma (TSM) represents a complex skull base tumor. The primary goals of surgical treatment are represented by maximal safe resection and visual recovery; therefore, appropriate patient selection is critical to optimize results. In the last 2 decades, the endoscopic endonasal approach (EEA) has appeared as a successful and viable strategy for the management of these tumors. The authors identified preoperative factors associated with extent of resection and visual outcome after EEA for TSM., Methods: In this retrospective cohort study, the authors analyzed patients who underwent extended endoscopic endonasal surgery for TSM between January 2005 and April 2022 at the Division of Neurosurgery, Università degli Studi di Napoli Federico II, Naples, Italy. Tumor size, vessel encasement, and optic canal involvement were classified according to University of California, San Francisco, score. Visual acuity and visual fields were analyzed according to the visual impairment score (VIS), defined as a four-level classification: grade 1 (VIS 0-25), grade 2 (VIS 26-50), grade 3 (VIS 51-75), and grade 4 (VIS 76-100). Ophthalmological functions were tested preoperatively and during the early postoperative period (within 6 months after surgery) and late postoperative period., Results: A total of 48 patients were enrolled. Forty-one (85.4%) patients experienced blurred vision or visual field defect as a presenting sign. Gross-total resection was achieved in 40 (83.3%), near-total resection in 2 (4.2%), and subtotal resection in 6 (12.5%). Visual defect improved in 82.9% (34/41) of cases, 12.2% (5/41) had no significant changes, and 2.4% (1/41) had worsened visual defect. The mean change in VIS was 42% (95% CI 58.77-31.23). Visual outcome was poorer when preoperative VIS (VIS-pre) was greater than 25 (p = 0.02). Six postoperative CSF leaks occurred (12.5%), and 1 patient (2.1%) required revision surgery., Conclusions: The EEA is a safe and effective approach for TSM removal, with the advantage of preserving optic apparatus vascularization, and can promote gross-total resection and visual improvement. The authors have defined four categories based on VIS that relate to postoperative outcome: the lower the VIS-pre, the higher the rate of postoperative VIS improvement. This finding may be useful for predicting a patient's visual outcome at the preoperative stage.

Objective: Meningiomas invading the intracranial venous sinuses may cause intracranial venous hypertension, papilledema, and visual compromise. Sinus resection and graft reconstructions, however, add significant complexity to tumor surgery, with the potential for increased morbidity. In this study, the authors explored whether venous sinus stenting might provide an alternative means of controlling venous hypertension that would be sustainable over the long term., Methods: The authors performed a retrospective review of all 16 patients with intracranial meningiomas who underwent stenting at their institution for venous sinus compromise. At presentation, all had headache and 9 had papilledema. Thirteen patients had 1 meningioma and 3 had 2 or more. Three patients had had previous tumor resection and radiotherapy. One patient had been treated with a lumboperitoneal shunt and radiotherapy. The median length of clinical follow-up was 8 years (range 4 months-18 years)., Results: Venous sinus narrowing was often not confined to the site of meningioma, and bilateral transverse sinus narrowing, reminiscent of that seen in idiopathic intracranial hypertension, was present in 7 patients with sagittal sinus meningiomas. Eleven patients had stents placed solely across sinus narrowing caused by meningioma. Five patients had additional stents placed at other sites of venous narrowing at the same time: in one of these patients, a stent was placed across a defect in the sagittal sinus caused by previous surgery, and in the 4 other patients, stents were placed across nontumor narrowings of the transverse sinuses. In 1 patient, the jugular vein was also stented. Nine patients developed symptomatic in-stent restenosis at the meningioma site. Eight had further stenting procedures with variable success in restoring the in-stent lumen. The remaining patient, with a late partial relapse, is being reinvestigated. Papilledema resolved in all patients after stenting. Six patients experienced prolonged and very substantial relief of all symptoms. Five patients had persistent headache despite restoration of the sinus lumen. Five had persistent symptoms associated with resistant in-stent stenosis. There were no significant complications from any of the diagnostic or therapeutic procedures., Conclusions: In patients who are symptomatic with meningiomas obstructing the venous sinuses, successful stenting of the affected segment can give a good outcome, especially in terms of relieving papilledema. However, further procedures are often necessary to maintain stent patency, other areas of venous compromise frequently coexist, and some patients remain symptomatic despite apparently successful treatment of the index lesion. Long-term surveillance is a requirement.

Objective: Petroclival meningiomas (PCMs) are challenging lesions to treat because of their deep location and proximity to critical neurovascular structures. Patients with these lesions commonly present because of local mass effect. A symptom that proves challenging to definitively manage is trigeminal neuralgia (TN), which occurs in approximately 5% of PCM cases. To date, there is no consensus on whether microsurgical resection or stereotactic radiosurgery (SRS) leads to better outcomes in the treatment of TN secondary to PCM. In this systematic review and meta-analysis, the authors aimed to evaluate the available literature on the efficacy of microsurgical resection versus SRS for controlling TN secondary to PCM., Methods: The Embase, MEDLINE, Scopus, and Cochrane databases were queried from database inception to May 17, 2022, using the search terms "(petroclival AND meningioma) AND (trigeminal AND neuralgia)." Study inclusion criteria were as follows: 1) reports on patients aged ≥ 18 years and diagnosed with TN secondary to PCM, 2) cases treated with microsurgical resection or SRS, 3) cases with at least one posttreatment follow-up report of TN pain, 4) cases with at least one outcome of tumor control, and 5) publications describing randomized controlled trials, comparative or single-arm observational studies, case reports, or case series. Exclusion criteria were 1) literature reviews, technical notes, conference abstracts, or autopsy reports; 2) publications that did not clearly differentiate data on patients with PCMs from data on patients with different tumors or with meningiomas in different locations (other intracranial or spinal meningiomas); 3) publications that contained insufficient data on treatments and outcomes; and 4) publications not written in the English language. References of eligible studies were screened to retrieve additional relevant studies. Data on pain and tumor outcomes were compared between the microsurgical resection and SRS treatment groups. The DerSimonian-Laird random-effects model with Hartung-Knapp-Sidik-Jonkman variance correction was used to pool estimates from the included studies., Results: Two comparative observational studies and 6 single-arm observational studies describing outcomes after primary intervention were included in the analyses (138 patients). Fifty-seven patients underwent microsurgical resection and 81 underwent SRS for the management of TN secondary to PCM. By the last follow-up (mean 71 months, range 24-149 months), the resection group had significantly higher rates of pain resolution than the SRS group (82%, 95% CI 50%-100% vs 31%, 95% CI 18%-45%, respectively; p = 0.004). There was also a significantly longer median time to tumor recurrence following resection (43.75 vs 16.7 months, p < 0.01). The resection group showed lower rates of pain persistence (0%, 95% CI 0%-6% vs 25%, 95% CI 13%-39%, p = 0.001) and pain exacerbation (0% vs 12%, 95% CI 3%-23%, p = 0.001). The most common postintervention Barrow Neurological Institute pain score in the surgical group was I (66.7%) compared with III (27.2%) in the SRS group. Surgical reintervention was less frequently required following primary resection (1.8%, 95% CI 0%-37% vs 19%, 95% CI 1%-48%, p < 0.01)., Conclusions: Microsurgical resection is associated with higher rates of TN pain resolution and lower rates of pain persistence and exacerbation than SRS in the treatment of PCM. SRS with further TN management is a viable alternative in patients who are not good candidates for microsurgical resection.

Objective: Mutations in NF2 are the most common somatic driver mutation in sporadic meningiomas. NF2 mutant meningiomas preferentially arise along the cerebral convexities-however, they can also be found in the posterior fossa. The authors investigated whether NF2 mutant meningiomas differ in clinical and genomic features based on their location relative to the tentorium., Methods: Clinical and whole exome sequencing (WES) data for patients who underwent resection of sporadic NF2 mutant meningiomas were reviewed and analyzed., Results: A total of 191 NF2 mutant meningiomas were included (165 supratentorial, 26 infratentorial). Supratentorial NF2 mutant meningiomas were significantly associated with edema (64.0% vs 28.0%, p < 0.001); higher grade-i.e., WHO grade II or III (41.8% vs 3.9%, p < 0.001); elevated Ki-67 (55.0% vs 13.6%, p < 0.001); and larger volume (mean 45.5 cm3 vs 14.9 cm3, p < 0.001). Furthermore, supratentorial tumors were more likely to harbor the higher-risk feature of chromosome 1p deletion (p = 0.038) and had a larger fraction of the genome altered with loss of heterozygosity (p < 0.001). Infratentorial meningiomas were more likely to undergo subtotal resection than supratentorial tumors (37.5% vs 15.8%, p = 0.021); however, there was no significant difference in overall (p = 0.2) or progression-free (p = 0.4) survival., Conclusions: Supratentorial NF2 mutant meningiomas are associated with more aggressive clinical and genomic features as compared with their infratentorial counterparts. Although infratentorial tumors have higher rates of subtotal resection, there is no associated difference in survival or recurrence. These findings help to better inform surgical decision-making in the management of NF2 mutant meningiomas based on location, and may guide postoperative management of these tumors.

Objective: Skull base meningiomas (SBMs) involving the cavernous sinus encase the internal carotid artery (ICA) and may lead to stenosis of the vessel. Although ischemic stroke has been reported in the literature, there are to the authors' knowledge no reported studies quantifying the risk of stroke in these patients. The authors aimed to determine the frequency of arterial stenosis in patients with SBMs that encase the cavernous ICA and to estimate the risk of ischemic stroke in these patients., Methods: Records of all patients with SBM encasing the ICA whose cases were managed by the skull base multidisciplinary team at Salford Royal Hospital between 2011 and 2017 were reviewed using a two-stage approach: 1) clinical and radiological strokes were identified from electronic patient records, and 2) cases were reviewed to examine the correlation between ICA stenosis associated with SBM encasement and anatomically related stroke. Strokes that were caused by another pathology or did not occur in the perfusion territory were excluded., Results: In the review of patient records the authors identified 118 patients with SBMs encasing the ICA. Of these, 62 SBMs caused stenosis. The median age at diagnosis was 70 (IQR 24) years, and 70% of the patients were female. The median follow-up was 97 (IQR 101) months. A total of 13 strokes were identified in these patients; however, only 1 case of stroke was associated with SBM encasement, which occurred in the perfusion territory of a patient without stenosis. Risk of acute stroke during the follow-up period for the entire cohort was 0.85%., Conclusions: Acute stroke in patients with ICA encasement by SBMs is rare despite the propensity of these tumors to stenose the ICA. Patients with ICA stenosis secondary to their SBM did not have a higher incidence of stroke than those with ICA encasement without stenosis. The results of this study demonstrate that prophylactic intervention to prevent stroke is not necessary in ICA stenosis secondary to SBM.

Objective: The objective of this paper was to describe the volumetric natural history of meningiomas in patients with neurofibromatosis type 2 (NF2)., Methods: The authors performed a retrospective descriptive study by reviewing NF2 patients with meningiomas at their institution between 2000 and 2019. Demographic data were collected from the electronic medical records. Tumor volume was collected using volumetric segmentation software. Imaging characteristics including peritumoral brain edema (PTBE) and tumor calcification were collected for each patient from their first to most recent MRI at the authors' institution. An increase of 15% or more per year from original tumor size was used as the cutoff to define growth., Results: A total of 137 meningiomas from 48 patients were included in the analysis. The average number of tumors per person was 2.9. Ninety-nine (72.3%) tumors were in female patients. The median length of follow-up from first imaging to last imaging was 32 months (IQR 10.9, 68.3 months). Most tumors were located in the cerebral convexity (24.8%), followed by the falcine region (18.2%) and spine (10.2%). The median tumor growth was 0.12 cm3/yr (IQR 0.03, 0.52 cm3/yr). At the time of first imaging, 21.9% of tumors had calcifications, while 13.9% of meningiomas had PTBE. Of 137 tumors, 52 showed growth. Characteristics associated with tumor growth included PTBE (OR 9.12, 95% CI 1.48-56.4), tumor volume (per cm3) at first imaging (OR 0.91, 95% CI 0.83-0.99), and 10-year increased age at first imaging (OR 0.57, 95% CI 0.43-0.74). PTBE had the shortest median time to growth at 9.2 months., Conclusions: Although the majority of NF2-associated meningiomas do not grow in the short term, a wide range of growth patterns can be seen. Younger age at first imaging and presence of PTBE are associated with growth. Patients with these characteristics likely benefit from closer follow-up.

Objective: Tumors compressing the trigeminal nerve can cause facial pain, numbness, or paresthesias. Limited data exist describing how these symptoms change after resection and what factors predict symptom improvement. The objective of this study was to report trigeminal pain and sensory outcomes after tumor resection and identify factors predicting postoperative symptom improvement., Methods: This retrospective study included patients with tumors causing facial pain, numbness, or paresthesias who underwent resection. Trigeminal schwannomas were excluded. Logistic regression, recursive partitioning, and time-to-event analyses were used to report outcomes and identify variables associated with facial sensory outcomes., Results: Eighty-six patients met inclusion criteria, and the median follow-up was 3.1 years; 63 patients (73%) had meningiomas and 23 (27%) had vestibular schwannomas (VSs). Meningioma patients presented with pain, numbness, and paresthesias in 56%, 76%, and 25% of cases, respectively, compared with 9%, 91%, and 39%, respectively, for patients with VS. Most meningioma patients had symptoms for less than 1 year (60%), whereas the majority of VS patients had symptoms for 1-5 years (59%). The median meningioma and VS diameters were 3.0 and 3.4 cm, respectively. For patients with meningiomas, gross-total resection (GTR) was achieved in 27% of patients, near-total resection (NTR) in 29%, and subtotal resection (STR) in 44%. For patients with VS, GTR was achieved in 9%, NTR in 30%, and STR in 61%. Pain improved immediately after tumor resection in 81% of patients and in 92% of patients by 6 weeks. Paresthesias improved immediately in 80% of patients, increasing to 84% by 6 weeks. Numbness improved more slowly, with 52% of patients improving immediately, increasing to 79% by 2 years. Pain recurred in 22% of patients with meningiomas and 0% of patients with VSs. After resection, the Barrow Neurological Institute (BNI) facial pain intensity score improved in 73% of patients. The tumor diameter significantly predicted improvement in BNI score (OR 0.47/cm larger, 95% CI 0.22-0.99; p = 0.047). Complete decompression of the trigeminal nerve was associated with qualitative improvement in pain (p = 0.037) and decreased pain recurrence (OR 0.08, 95% CI 0.01-0.67; p = 0.024)., Conclusions: Most patients with facial sensory symptoms caused by meningiomas or VSs experienced improvement after resection. Surgery led to immediate and sustained improvement in pain and paresthesias, whereas numbness was slower to improve. Patients with smaller tumors and complete decompression of the trigeminal nerve were more likely to experience improvement in facial pain.

Objective: Patients with skull base meningioma (SBM) often require complex surgery around critical neurovascular structures, placing them at high risk of poor health-related quality of life (HRQOL) and possibly neurocognitive dysfunction. As the survival of meningioma patients is near normal, long-term neurocognitive and HRQOL outcomes are important to evaluate, including evaluation of the impact of specific tumor location and treatment modalities on these outcomes., Methods: In this multicenter cross-sectional study including patients 5 years or more after their last tumor intervention, Short-Form Health Survey (SF-36) and European Organisation for Research and Treatment of Cancer (EORTC) QLQ-BN20 questionnaires were used to assess generic and disease-specific HRQOL. Neurocognitive functioning was assessed with standardized neuropsychological assessment. SBM patient assessments were compared with those of 1) informal caregivers of SBM patients who served as controls and 2) convexity meningioma patients. In addition, the authors compared anterior/middle SBM patients with posterior SBM patients and anterior/middle and posterior SBM patients separately with controls. Multivariable and propensity score regression analyses were performed to correct for possible confounders., Results: Patients with SBM (n = 89) with a median follow-up of 9 years after the last intervention did not significantly differ from controls (n = 65) or convexity meningioma patients (n = 84) on generic HRQOL assessment. Statistically significantly but not clinically relevantly better disease-specific HRQOL was found for SBM patients compared with convexity meningioma patients. Anterior/middle SBM patients (n = 62) had significantly and clinically relevantly better HRQOL in SF-36 and EORTC QLQ-BN20 scores than posterior SBM patients (n = 27): physical role functioning (corrected difference 17.1, 95% CI 0.2-34.0), motor dysfunction (-10.1, 95% CI -17.5 to -2.7), communication deficit (-14.2, 95% CI -22.7 to -5.6), and weakness in both legs (-10.1, 95% CI -18.8 to -1.5). SBM patients whose primary treatment was radiotherapy had lower HRQOL scores compared with SBM patients who underwent surgery on two domains: bodily pain (-33.0, 95% CI -55.2 to -10.9) and vitality (-18.9. 95% CI -33.7 to -4.1). Tumor location and treatment modality did not result in significant differences in neurocognitive functioning, although 44% of SBM patients had deficits in at least one domain., Conclusions: In the long term, SBM patients do not experience significantly more sequelae in HRQOL and neurocognitive functioning than do controls or patients with convexity meningioma. Patients with posterior SBM had poorer HRQOL than anterior/middle SBM patients, and primary treatment with radiotherapy was associated with worse HRQOL. Neurocognitive functioning was not affected by tumor location or treatment modality.

Objective: Ménière's disease is an inner ear disorder classically characterized by fluctuating hearing loss, tinnitus, and aural fullness accompanied by episodic vertigo. While the pathogenesis of Ménière's remains under debate, histopathological analyses implicate endolymphatic sac dysfunction with inner ear fluid homeostatic dysregulation. Little is known about whether external impingement of the endolymphatic sac by tumors may present with Ménière's-like symptoms. The authors present a case series of 7 patients with posterior fossa meningiomas that involved the endolymphatic sac and new onset of Ménière's-like symptoms and review the literature on this rare clinical entity., Methods: A retrospective review of patients undergoing resection of a posterior petrous meningioma was performed at the authors' institution. Inclusion criteria were age older than 18 years; patients presenting with Ménière's-like symptoms, including episodic vertigo, aural fullness, tinnitus, and/or hearing loss; and tumor location overlying the endolymphatic sac., Results: There were 7 cases of posterior petrous face meningiomas involving the vestibular aperture presenting with Ménière's-like symptoms. Imaging and intraoperative examination confirmed no cranial nerve VIII compression or labyrinthine artery involvement accounting for audiovestibular symptoms. Of the 7 patients in the series, 6 experienced significant improvement or resolution of their vertigo, and all 7 had improvement or resolution of their tinnitus after resection. Of the 5 patients who had preoperative hearing loss, 2 experienced improvement or resolution of their ipsilateral preoperative hearing deficit, whereas the other 3 had unchanged hearing loss compared to preoperative evaluation., Conclusions: Petrous face meningiomas overlying the endolymphatic sac can present with a Ménière's syndrome. Early recognition and microsurgical excision of these tumors is critical for resolution of most symptoms and stabilization of hearing loss.

Objective: The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population., Methods: The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with "posterior fossa" or "spindle cell tumors" or "Ewing sarcoma" or "high-grade" or "spindle cell sarcoma" or "leptomeningeal melanocytoma" as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome., Results: The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease., Conclusions: A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.

Objective: Although meningiomas frequently involve the optic nerve, primary optic nerve sheath meningiomas (ONSMs) are rare, accounting for only 1% of all meningiomas. Given the high risk of vision loss with these tumors, surgical intervention is seldom considered, and radiation or observation is commonly applied. Here, the authors describe the visual outcomes for a series of patients who were treated with surgery aiming at maximal tumor resection and highlight their prognostic factors., Methods: The authors retrospectively analyzed the data for 8 patients with intracanalicular ONSMs who had been surgically treated by the senior author (O.A.) between 1998 and 2016. Meningiomas extending into the optic canal from the intracranial cavity (i.e., clinoid, sphenoid wing, tuberculum sellae, diaphragma sellae) were excluded. Diagnosis was based on ophthalmological, radiological, and intraoperative findings, which were confirmed by the typical histological findings. Preoperative, postoperative, and follow-up visual assessments were performed by neuro-ophthalmologists in all cases., Results: The patients included 7 females and 1 male. The mean age at diagnosis was 45.1 years (range 25.0-70.0 years). Mean duration of follow-up was 38.9 months (range 3.0-88.0 months). All patients reported visual complaints, and all had objective evidence of optic nerve dysfunction. Their evaluation included visual field, visual acuity, funduscopy, and retinal fiber thickness. Total resection was obtained in 4 cases. Comparing preoperative and postoperative visual function revealed that 4 patients had improvement at the last follow-up, 1 patient had stable vision, and 3 patients had decreased function but none had total vision loss. All patients with good preoperative visual acuity maintained this status following surgical treatment. There was no surgical mortality or infection. Operative complications included binocular diplopia in 4 patients, which remitted spontaneously., Conclusions: Surgery can play a beneficial role in the primary treatment of ONSM, especially lesions located in the posterior third of the nerve. Total removal can be achieved with vision preservation or improvement, without major surgical complications, especially at early stages of the disease. Patients with good preoperative vision and CSF flow in the optic sheath have better chances of a favorable outcome than those with poor vision.

In August 2016, an 11-year-old boy presented to the authors' institution with a right orbital tumor that was located superotemporally (superolaterally) and adherent to the sclera. The patient's past medical history revealed that he had undergone 2 previous craniotomies elsewhere in June 2008 and July 2010 for a superomedially located orbital lesion that had been histopathologically diagnosed as a neurothekeoma. After the second craniotomy, the patient underwent adjuvant intensity modulated radiotherapy (IMRT) to the right medial orbit. At the authors' institution, total excision of the orbital tumor was performed via an anterior conjunctival orbitotomy. Histopathological examination revealed a rhabdoid meningioma. Review of the histopathology obtained at the time of previous tumor excisions showed that the lesion was misdiagnosed as neurothekeoma and instead represented a meningioma from the beginning. The patient was started on a regimen of oral sunitinib and remained free of recurrence at 1.5 years of follow-up. Ectopic meningioma of the orbit is a rare entity. Rhabdoid meningioma is a rarely seen subtype of meningioma, accounting for 1%-3% of all intracranial meningiomas. To the best of the authors' knowledge, this is the first case of an ectopic orbital rhabdoid meningioma reported in the literature. They suspect that tumor seeding during the previous surgeries might have played a role in the occurrence of the tumor in an orbital location not targeted by IMRT.

OBJECTIVE Planum sphenoidale (PS) and tuberculum sellae (TS) meningiomas cause visual symptoms due to compression of the optic chiasm. The treatment of choice is surgical removal with the goal of improving vision and achieving complete tumor removal. Two options exist to remove these tumors: the transcranial approach (TCA) and the endonasal endoscopic approach (EEA). Significant controversy exists regarding which approach provides the best results and whether there is a subset of patients for whom an EEA may be more suitable. Comparisons using a similar cohort of patients, namely, those suitable for gross-total resection with EEA, are lacking from the literature. METHODS The authors reviewed all cases of PS and TS meningiomas that were surgically removed at Weill Cornell Medical College between 2000 and 2015 (TCA) and 2008 and 2015 (EEA). All cases were shown to a panel of 3 neurosurgeons to find only those tumors that could be removed equally well either through an EEA or TCA to standardize both groups. Volumetric measurements of preoperative and postoperative tumor size, FLAIR images, and apparent diffusion coefficient maps were assessed by 2 independent reviewers and compared to assess extent of resection and trauma to the surrounding brain. Visual outcome and complications were also compared. RESULTS Thirty-two patients were identified who underwent either EEA (n = 17) or TCA (n = 15). The preoperative tumor size was comparable (mean 5.58 ± 3.42 vs 5.04 ± 3.38 cm 3 [± SD], p = 0.661). The average extent of resection achieved was not significantly different between the 2 groups (98.80% ± 3.32% vs 95.13% ± 11.69%, p = 0.206). Postoperatively, the TCA group demonstrated a significant increase in the FLAIR/edema signal compared with EEA patients (4.15 ± 7.10 vs -0.69 ± 2.73 cm 3 , p = 0.014). In addition, the postoperative diffusion-weighted imaging signal of cytotoxic ischemic damage was significantly higher in the TCA group than in the EEA group (1.88 ± 1.96 vs 0.40 ± 0.55 cm 3 , p =0.008). Overall, significantly more EEA patients experienced improved or stable visual outcomes compared with TCA patients (93% vs 56%, p = 0.049). Visual deterioration was greater after TCA than EEA (44% vs 0%, p = 0.012). While more patients experienced postoperative seizures after TCA than after EEA (27% vs 0%, p = 0.038), there was a trend toward more CSF leakage and anosmia after EEA than after TCA (11.8% vs 0%, p = 0.486 and 11.8% vs 0%, p = 0.118, respectively). CONCLUSIONS In this small single-institution study of similarly sized and located PS and TS meningiomas, EEA provided equivalent rates of resection with better visual results, less trauma to the brain, and fewer seizures. These preliminary results merit further investigation in a larger multiinstitutional study and may support EEA resection by experienced surgeons in a subset of carefully selected PS and TS meningiomas.

OBJECTIVE In tuberculum sellae meningioma (TSM) surgery, endonasal approaches are claimed to have a superior visual outcome compared with transcranial approaches. The authors question whether this is always true and analyze their series of cases of endoscope-assisted transcranial TSM surgery with special regard to the postoperative visual outcome in order to explore this issue. METHODS All surgical procedures for TSM performed between 2003 and 2015 in the Department of Neurosurgery, University Medicine Greifswald, were retrospectively analyzed. Special attention was paid to the postoperative visual outcome. RESULTS During the study period, 15 patients (12 female and 3 male) underwent surgery for TSM. Gross-total resection was achieved in 14 cases (93.3%) and near-total resection in 1. One patient suffered from a major stroke during surgery and had to be excluded from further analyses. No other complications occurred. Preoperatively, visual acuity was disturbed in 12 patients (80%) and visual field deficits were present in 11 patients (73.3%). In 3 patients (20%), the TSM was an incidental finding. Postoperatively, ophthalmological examination revealed an improvement of visual acuity in 10 (90.9%) of 11 patients and improvement of visual field deficits in 9 (90%) of 10 patients; no deterioration of visual acuity or visual field was seen in any patient. Visual acuity and visual field improvement was observed in all patients who had surgery within 3 years after the onset of visual disturbances. No tumor recurrence was observed during follow-up (mean 32 months, range 3-134 months). TSMs were approached via a frontolateral craniotomy in 7 patients and via a supraorbital craniotomy in 8. The use of the endoscope as an assistive device led to improved tumor visualization and consequent removal in areas that were hidden in the microscopic view in 6 patients (40%). CONCLUSIONS The present series confirms a favorable visual outcome after TSM surgery via supraorbital or frontolateral endoscope-assisted approaches. With endoscopic visualization, major manipulation of the optic apparatus could be avoided, perhaps affecting the favorable visual outcome.

This video demonstrates a surgical technique of resecting dorsum sellae meningioma using a combined interhemispheric translamina terminalis approach and pterional approach with clinoidectomy. The tumor, 5 cm in maximum diameter, originated from the dorsum sellae, compressed the third ventricle and the midbrain, and displaced the pituitary stalk ventrally. Feeding arteries of the tumor were bilateral meningohypophyseal trunks, mainly from the right side. The authors performed devascularization of the tumor via a right pterional approach following frontotemporal craniotomy, and debulking of the tumor via an interhemispheric translamina terminalis approach following bifrontal craniotomy. These procedures with two separate craniotomies enabled safe and effective resection of the tumor. The video can be found here: https://youtu.be/DEnKOC5zQ&#95;M .

Meningiomas are the most prevalent primary intracranial tumor, with 3%-10% arising from the tuberculum sellae. Optimal management consists of total microsurgical resection with preservation of surrounding structures. The authors present a 64-year-old woman with progressive bilateral vision loss, including visual acuity deficits and bitemporal hemianopsia. MRI revealed a 2-cm tuberculum sellae meningioma causing optic apparatus compression. An extended endoscopic endonasal transtuberculum approach was utilized for gross-total resection, including microdissection of tumor from the optic chiasm and infundibulum. Closure was performed with multilayer tensor fascia lata autograft and a pedicled nasal-septal flap. The patient's postoperative exam showed visual improvement and normal pituitary function. The video can be found here: https://youtu.be/ZfNB&#95;rhlyeI .

A 49-year-old man presented with headache and cognitive difficulty. MRI demonstrated a meningioma in the falcotentorial region with compression of the internal cerebral veins, basal veins of Rosenthal, and vein of Galen. It was a removed via a left-sided occipital interhemispheric approach, performed with the patient in the lateral position. After tumor debulking and removal of its attachment to the tentorium and anterior falx, the tumor was resected. All venous structures were preserved. The video demonstrates the technical nuances and strategy for removal of tumors in a region with complicated venous anatomy that must be preserved. The video can be found here: https://youtu.be/wKqAn3dYu4E .

A 69-year-old female with incidental diagnosis of a dorsum sellae meningioma had shown significant tumor growth after initial conservative management. The procedure started with a microscopic sublabial transsphenoidal approach to the sella and the suprasellar space. Due to limitations to a safe dissection and removal of the retrosellar component, the surgery was converted to a purely endoscopic endonasal approach with left hemi-transposition of the pituitary gland, followed by drilling of the dorsum sellae and removal of the left posterior clinoid process. A complete tumor resection was achieved, and a multilayer skull base reconstruction was performed without complications. The video can be found here: https://youtu.be/BEolyK-To&#95;A .

Tuberculum sella meningiomas can be approached through lateral approaches including pterional/orbitozygomatic craniotomies, frontobasal craniotomy, or through expanded endoscopic transsphenoidal approaches. The authors present the case of a 60-year-old male who presented with bitemporal hemianopia and significant right-side visual acuity compromise due to a large tuberculum sella meningioma. The tumor had an important extension to the posterior fossa. A right orbitozygomatic trans-sylvian approach was deemed most suitable to tackle the posterior extension of the tumor. Some operative nuances are detailed in the video including dissection of the tumor off the carotid artery, basilar bifurcation, and small thalamoperforators attached to the tumor. Postoperatively, the patient's bilateral hemianopia improved significantly, but his right visual activity remained unchanged. The remaining part of the tumor in the sella and midclivus was addressed with a second-stage expanded endoscopic transclival approach. The video can be found here: https://youtu.be/KbewhlT2FWs .

A 38-year-old woman had a 3-week gradual onset of right-sided weakness in the upper and lower extremities. MRI showed a large left petro-clival meningioma encasing the basilar and left superior cerebellar artery and compressing the brainstem. A posterior transpetrosal approach, with a left temporal and retrosigmoid craniotomy and mastoidectomy, was performed. The tumor was removed in a gross-total resection with questionable remnants adherent to the brainstem. Intraoperative partial iatrogenic injury to the left oculomotor nerve was repaired with fibrin glue. Postoperatively, the hemiparesis improved, and the patient was discharged to the rehabilitation center with left oculomotor and abducens palsies. A postoperative MRI scan showed complete resection of tumor with no remnants on the brainstem. A 6-month follow-up examination showed complete resolution of motor symptoms and complete recovery of cranial nerve (CN) palsies affecting CN III and CN VI. The video can be found here: https://youtu.be/vOu6YFA8uoo .

Surgical removal of foramen magnum meningiomas poses great challenges due their deep location within the central skull base and their proximity to vital neurovascular structures. This video depicts the operative nuances of surgical management for a 59-year-old female who presented with a right-sided spinocranial meningioma. Simpson Grade I resection was achieved through a right transcondylar approach. The patient's postoperative period was unremarkable, and she was discharged home on postoperative Day 5 for periodic follow-up. The transcondylar approach safely exposes the craniocervical junction at the anterior aspect of the neuraxis and still allows the surgeon to access the tumor through a parallel plane, with minimum morbidity. The video can be found here: https://youtu.be/P0-kXjAkw9U .

The authors present the case of a 56-year-old patient who presented with a large petroclival meningioma and clinical symptoms of brainstem compression and hydrocephalus. The authors describe a two-stage combined petrosal approach for complete resection of the tumor. The nuances of the approach and microsurgical technique are discussed, as well as the radiographic and clinical outcomes. The video can be found here: https://youtu.be/JxoTXS-uiHM .

Extradural anterior clinoidectomy is a versatile technique to increase exposure of the sellar and parasellar region. It is of particular use in the resection of clinoidal meningiomas, as sphenoidal and clinoidal hyperostosis can cause compression of the optic nerve. Extradural clinoidectomy follows a series of steps, consisting of (1) unroofing of the superior orbital fissure, (2) unroofing of the optic canal, (3) removal of the optic strut, and (4) removal of the anterior clinoid process. The authors show these steps in detail, as well as their application to the resection of a large clinoidal meningioma. The video can be found here: https://youtu.be/O1Fcef29ETg .

Clear cell meningioma (CCM) is an uncommon variant of meningioma. The authors describe a case of a pediatric CCM localized to the lumbar spine. After resection, sequencing revealed an inactivating mutation in the SWI/SNF chromatin remodeling complex subunit SMARCE1, with loss of the second allele in the tumor. The authors present a literature review of this mutation that is associated with CCM and a family history of spine tumors.

Object: The authors aimed to assess whether the prevalence of intracranial aneurysms in patients with intracranial meningiomas was higher than that in a healthy population., Methods: The authors performed a hospital-based case-control study of 300 patients with newly diagnosed intracranial meningiomas and 900 age- and sex-matched controls without a history of brain tumors to evaluate any associations between intracranial aneurysms and intracranial meningiomas. Unconditional multivariate logistic regression models were used for case-control comparisons., Results: Intracranial aneurysms were identified in 23 patients (7.7%) and 24 controls (2.7%; p < 0.001). There was a significant association between intracranial aneurysms and intracranial meningiomas (OR 2.913, 95% CI 1.613-5.261) and hypertension (OR 1.905, 95% CI 1.053-3.446). In a subgroup analysis of the patients with newly diagnosed intracranial meningiomas, there was a significant association between intracranial aneurysms and hypertension (OR 2.876, 95% CI 1.125-7.352) and tumor volume (OR 1.012, 95% CI 1.001-1.024). These patients were also more likely than controls to have other intracranial vascular diseases (p < 0.001), such as isolated occlusion of the intracranial vessels, excluding intracranial aneurysms., Conclusions: The prevalence of intracranial aneurysms was higher in patients with intracranial meningiomas. Hypertension and tumor volume appear to be associated with the formation of intracranial aneurysms in these patients.

Object: To assess the long-term outcomes of stereotactic radiosurgery (SRS) for cerebellopontine angle (CPA) meningiomas, the authors retrospectively reviewed data from a 20-year experience. They evaluated progression-free survival as well as improvement, stabilization, or deterioration in clinical symptoms., Methods: Seventy-four patients with CPA meningiomas underwent SRS involving various Gamma Knife technologies between 1990 and 2010. The most common presenting symptoms were dizziness or disequilibrium, hearing loss, facial sensory dysfunction, and headache. The median tumor volume was 3.0 cm(3) (range 0.3-17.1 cm(3)), and the median radiation dose to the tumor margin was 13 Gy (range 11-16 Gy). The median follow-up period was 40 months (range 4-147 months)., Results: At last imaging follow-up, the tumor volume had decreased in 46 patients (62%), remained stable in 26 patients (35%), and increased in 2 patients (3%). The progression-free survival after SRS was 98% at 1 year, 98% at 3 years, and 95% at 5 years. At the last clinical follow-up, 23 patients (31%) showed neurological improvement, 43 patients (58%) showed no change in symptoms or signs, and 8 patients (11%) had worsening symptoms or signs. The neurological improvement rate after SRS was 16% at 1 year, 31% at 3 years, and 40% at 5 years. The post-SRS deterioration rate was 5% at 1 year, 10% at 3 years, and 16% at 5 years. A multivariate analysis demonstrated that trigeminal neuralgia was the symptom most likely to worsen after SRS (HR 0.08, 95% CI 0.02-0.31; p = 0.001). Asymptomatic peritumoral edema occurred in 4 patients (5%) after SRS, and symptomatic adverse radiation effects developed in 7 patients (9%)., Conclusions: Stereotactic radiosurgery for CPA meningiomas provided a high tumor control rate and relatively low risk of ARE. Tumor compression of the trigeminal nerve by a CPA meningioma resulted in an increased rate of facial pain worsening in this patient experience.

Meningioma is the second most common type of adult intracranial neoplasm. A substantial subset of patients present with peritumoral brain edema (PTBE), which can cause significant morbidity via mass effect, complicate surgical management, and impact the safety of stereotactic radiosurgery. Recent studies suggest a close relationship between vascular endothelial growth factor-A (VEGF-A) expression and PTBE development in meningiomas. The authors performed a systematic review of the literature on the pathogenesis of PTBE in meningiomas, the effectiveness of steroid therapy, the role played by VEGF-A, and the current clinical evidence for antiangiogenic therapy to treat peritumoral brain edema. Mounting evidence suggests VEGF-A is secreted directly by meningioma cells to induce angiogenesis and edemagenesis of tumoral as well as peritumoral brain tissue. The VEGF-A cascade results in recruitment of cerebral-pial vessels and disruption of the tumor-brain barrier, which appear to be requisite for VEGF-A to have an edemagenic effect. Results of preliminary clinical studies suggest VEGF-directed therapy has modest activity against recurrent and progressive meningioma growth but can alleviate PTBE in some patients. A comprehensive understanding of the VEGF-A pathway and its modulators may hold the key to an effective therapeutic approach to treating PTBE associated with meningiomas. Further clinical trials with larger patient cohorts and longer follow-up periods are warranted to confirm the efficacy of VEGF-directed therapy.

Object: The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors., Methods: The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age ≤ 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort. Data regarding symptoms, diagnosis, tumor characteristics, and NF2 status were collected and analyzed., Results: Sixty patients (55% female, 45% male) harboring 87 tumors were identified. The mean age at diagnosis was 12.8 years (median 14.0 years, range 0.9-18.9 years). Schwannomas were the most commonly identified lesions (57 of 87 tumors, including 52 vestibular, 3 facial, and 2 trigeminal schwannomas), followed by meningiomas (5 of 87) and epidermoid cysts (4 of 87). Six malignant tumors were diagnosed, including small-cell sarcoma, squamous cell carcinoma, malignant meningioma, atypical rhabdoid-teratoid tumor, endolymphatic sac tumor, and malignant ganglioglioma. Headache, followed by hearing loss and imbalance, was the most common presenting symptom, whereas dysphagia, otalgia, and facial pain were uncommon. Neurofibromatosis Type 2 was diagnosed in 20 (61%) of 33 patients with vestibular schwannoma (VS), while the other 13 patients (39%) had sporadic tumors. Nineteen of the 20 patients with NF2 met the diagnostic criteria for that disorder on initial presentation, and 15 of them presented with bilateral VS. At the last follow-up, 19 of the 20 patients subsequently diagnosed with NF2 demonstrated bilateral VSs, whereas 1 patient with a unilateral VS and multiple other NF2-associated tumors has yet to demonstrate a contralateral VS to date. Only 1 patient presenting with an isolated unilateral VS and no family history of NF2 demonstrated a contralateral VS on subsequent radiological screening., Conclusions: Cerebellopontine angle and IAC tumors in the pediatric population are rare. There are several noteworthy differences between the adult and pediatric populations harboring these lesions. While VS is the most common pathology in both age groups, the lesion was found in only 60% of the pediatric patients in the present study. Unlike in adults, VSs in the pediatric population were associated with NF2 in over one-half of all cases. The majority of pediatric patients with NF2 fulfilled the diagnostic criteria at initial presentation; however, approximately 7% of patients presenting with a seemingly sporadic (no family history of NF2) unilateral VS will meet the criteria for NF2 later in life. Finally, malignancies account for a significantly higher percentage (10%) of cases among pediatric patients. These findings underscore the importance of early screening and close radiological follow-up and may be helpful in patient counseling.

Meningiomas rarely cause CSF dissemination, and CSF seeding to the optic nerve (ON) is extremely rare. This is the first report of 2 cases of atypical meningioma with subacute visual loss due to ON seeding. The authors present the genetic characteristics of these atypical meningiomas with CSF dissemination. The patient in Case 1 was a 36-year-old woman with a 1.5-cm mass within the left ON, and the patient in Case 2 was a 70-year-old woman with a 0.9-cm mass around the right ON. Both individuals had undergone multiple surgeries for primary lesions and local recurrent lesions. They presented with subacute visual loss, and both tumors were completely resected. The pathological diagnosis was atypical meningioma with high MIB-1 indices and p53-positive cell ratios in each case. Comparative genomic hybridization showed significant chromosomal copy number alterations similar to the results of previous surgeries, confirming that the tumors were disseminated lesions. The present findings suggest that genetic characteristics, such as 1p and 10qcen-23 losses and 17q and 20 gains, shared by the 2 cases might be associated with CSF dissemination of meningiomas.

Object: Calcified meningiomas are an uncommon type of meningioma. This study details the clinical features, treatment, and follow-up of 11 calcified meningiomas treated from 2002 to 2009, for the purpose of providing general information, describing the skill required for the surgery, and detailing the imaging study of these tumors., Methods: Between 2002 and 2009, 11 patients underwent surgery for the treatment of calcified meningiomas. All were treated by the same group of doctors at the same institution, including surgery and rehabilitation after surgery. The minimum 3-year (> 36 months) follow-up data from the 11 patients were detailed. Neurological function was evaluated twice, based on the Frankel scale and Japanese Orthopaedic Association scoring system. The first evaluation occurred before surgery and the second 3 years after surgery., Results: In 3 cases, the Frankel score decreased by 1 level. In a comparison of the duration of preoperative symptoms, age, degree of canal stenosis, and intraoperative blood loss, it was found that the greater the degree of canal stenosis, the poorer the outcome of the patient. Calcified meningiomas were more likely to adhere to the nerves and dura, a finding that might explain the high incidence of neurological dysfunction and CSF leakage after surgery., Conclusions: Calcified meningiomas are the most rare of all meningiomas. It appears that a greater degree of canal stenosis can lead to a poorer outcome. Computed tomography scans and MRI with contrast enhancement are recommended for intraspinal tumors before surgery to exclude the possibility of calcification. For calcified meningiomas, precise tumor resection, dura repair during surgery, and medical care after surgery are important for achieving an acceptable outcome.

Object: The objective of this study was to evaluate the ophthalmological outcome, nonvisual morbidity, and surgical complications after tuberculum sellae meningioma (TSM) removal using a superior interhemispheric approach., Methods: In the last decade, 20 consecutive patients with TSM underwent operations using the superior interhemispheric approach. Visual acuity, visual field, and ocular fundus examination were assessed both preoperatively and 6-months postoperatively. Nonvisual morbidity was determined at an early postoperative period and at 6 months based on assessment of the Karnofsky Performance Scale score, leakage of CSF, endocrinological status, and olfactory function, which was assessed using a visual analog scale (VAS). The potential brain injury related to the approach was assessed by MRI at 6 months. Magnetic resonance imaging was then performed yearly to detect a recurrence. The mean follow up was 56.3 ± 34 months., Results: The primary presenting symptom for diagnosis of TSM in 20 patients (female:male ratio of 6.6:1, mean age 59.1 ± 11.1 years) was visual disturbance in 12 patients (60%), headache in 4 (20%), cognitive alteration in 1 (5%), epilepsy in 2 (10%), and accidental in 1 (5%). In a total of 40 eyes, 17 eyes in 11 patients presented with preoperative deterioration of visual acuity. Postoperatively, the visual acuity improved in 13 eyes in 8 patients (72.8%), remained unchanged in 3 eyes in 2 patients (18.2%) and deteriorated in 1 patient (9%). The nonvisual morbidity included olfactory deterioration in 7 patients (35%), and panhypopituitarism in 1 patient (5%). No patients experienced a CSF leak. The impact of olfactory deterioration on the quality of life, as estimated by a VAS score (range 0-10), was a mean of 5.7 ± 2.2 (95% CI 4.1-7.3). On the follow-up MRI, no additional lesions or recurrences were observed on the medial aspect of the frontal lobe along the surgical corridor., Conclusions: The superior interhemispheric approach appears to be effective in resolving the problem of visual deterioration due to a TSM, without inducing surgical injury on the brain surface along the surgical corridor. Olfactory deterioration remained the challenging predominant nonvisual morbidity using this approach.

The authors present the case of an 11-year-old boy with an intraventricular chordoid meningioma, which is a rare presentation of prolonged fever of unknown origin due to a rare tumor in a rare location. The fever resolved after excision of the lesion. Subsequent imaging revealed recurrence at 1 year. After a repeat excision and fractionated radiotherapy, the patient has remained disease free 5 years after the first surgery. Very few cases of intraventricular chordoid meningioma have been reported to date. The pathological features and clinical course are described. A review of the literature describing management options for this tumor type, recently found to have a higher recurrence rate, is described herein.

Meningioangiomatosis consists of benign hamartomatous lesions of the brain and the leptomeninges, which typically present with seizure. Management is predicated on resection and control of seizures with medication. Lesions are typically solitary. Multifocal meningioangiomatosis is extremely rare, with only 2 cases reported in adults and none in children. The authors report the first case, to their knowledge, of multifocal meningioangiomatosis in a child. This unique case highlights therapeutic challenges associated with these lesions and demonstrates that multifocality is possible in the pediatric population. This finding has implications for diagnosis and follow-up for children afflicted with these tumors.

Object: Various surgical approaches, such as uni- and bifrontal, frontolateral, and pterional approaches, have been advocated for tuberculum sellae meningiomas. The authors retrospectively reviewed the effectiveness of a bilateral subfrontal approach for tuberculum sellae meningiomas with special attention to ophthalmological outcomes and complications., Methods: Between 1993 and 2009, 34 patients underwent surgery for removal of tuberculum sellae meningiomas at Osaka City University. Tumor size ranged from 14 to 45 mm. Thirty-two of 34 patients presented with visual disturbances before the surgery. The visual functions in all patients were assessed using a visual impairment score (VIS) before and after surgery. Postoperative visual examination was performed 2 weeks after surgery. Long-term follow-up examinations were conducted 1 year after surgery., Results: Radical resection (Simpson Grades I and II) was accomplished in 27 patients, and subtotal or partial resection (Simpson Grades III and IV) was achieved in 7. There was no deterioration in postoperative visual outcome. Twenty-nine (90.6%) of 32 patients showed improved VIS compared with preoperative VIS. The average VIS was 38.1 preoperatively, 23.5 in the short-term postoperative period, and 21.8 in the long-term postoperative period. In the short-term postoperative period, the visual function in 6 patients normalized, and visual problems persisted in the remaining 26. Six (23%) of 26 patients showed further improvement in VIS during the long-term follow-up period, and no patient exhibited a worsened VIS during this time. One patient complained of hyposmia after surgery, but there was no indication of related complications such as CSF leakage or frontal brain contusion., Conclusions: The bilateral subfrontal approach was previously avoided because of the relatively high rate of complications in earlier surgical series of tuberculum sellae meningiomas. However, after developments in microsurgical techniques in recent years, the bilateral subfrontal approach can now provide satisfactory visual outcomes with minimal postoperative complications. Careful preservation of the blood supply to optic apparatus and early unroofing of the optic canal using a bilateral subfrontal approach led to further improvement in long-term postoperative visual outcome.

Pneumosinus dilitans (PSD) is enlargement of the sinuses of the skull base and is frequently seen with meningiomas. Identifying PSD on imaging can assist with operative planning and preparation. Meningiomas associated with PSD are not more commonly high grade, and complete resection is frequently possible.

Object: Juxtasellar meningiomas frequently extend into the optic canal. Removing these meningiomas from the optic canal is crucial for favorable visual outcome., Methods: The authors performed a retrospective analysis of 45 patients with anterior and middle fossa meningiomas with involvement of the optic pathway in whom surgery was performed by the senior author (O.A.M.) during the period from 1993 to 2007. Extent of resection and recurrence rates were determined by pre- and postoperative MR imaging studies. Visual outcomes were evaluated with full ophthalmological examinations performed before and after surgery., Results: Forty-five patients (31 women and 14 men) were involved in this study; their mean age was 51.6 years. Patients were followed for a mean of 29.8 months (range 6-108 months). No surgery-related death occurred. The average tumor size was 3.1 cm. Total resection of the tumor (Simpson Grade I) was achieved in 32 patients (71.1%). Gross-total resection (Simpson Grades II and III) was achieved in 13 patients (28.9%). Only 1 patient harboring a left cavernous sinus meningioma had tumor recurrence and underwent repeat resection. Meningiomas extended into 58 optic canals in these cases; 13 patients showed extension into both optic canals. Visual disturbance was the main presenting symptom in 37 patients (82.2%); 8 patients had normal vision initially. Visual improvement after surgery was seen in 21 (57%) of 37 patients and in 27 (34.6%) of 78 affected eyes. Vision remained unchanged in 48 (61.5%) of 78 eyes. Transient postoperative visual deterioration occurred in 2 eyes (2.6%), with recovery to baseline over time. Only 1 (1.3%) of 78 eyes had permanent visual deterioration after surgery. The visual outcome was affected mainly by the tumor size, the preoperative visual status, and the duration of symptoms., Conclusions: Involvement of the optic canal in meningiomas is frequent. It occurs in a wide variety of anterior skull base meningiomas and it can be bilateral. It is a prominent factor that affects the preoperative visual status and postoperative recovery. Decompression of the optic canal and removal of the tumor inside is a crucial step in the surgical management of these tumors to optimize visual recovery and prevent tumor recurrence.

Neuropathic facial pain can be a debilitating condition characterized by stabbing, burning, dysesthetic sensation. With a large range of causes and types, including deafferentation, postherpetic, atypical, and idiopathic, both medicine and neurosurgery have struggled to find effective treatments that address this broad spectrum of facial pain. The authors report the use of motor cortex stimulation to alleviate 3 distinct conditions associated with intractable facial pain: trigeminal deafferentation pain following rhizotomy, deafferentation pain secondary to meningioma, and postherpetic neuralgia. Functional MR imaging was used to localize facial areas on the precentral gyrus prior to surgery. All 3 patients experienced long-lasting complete or near-complete resolution of pain following electrode implantation. Efficacy in pain reduction was achieved through variation of stimulation settings over the course of treatment, and it was assessed using the visual analog scale and narrative report. Surgical complications included moderate postsurgical incisional pain, transient cerebral edema, and intraoperative seizure. The authors' results affirm the efficacy and broaden the application of motor cortex stimulation to several forms of intractable facial pain.

This 11-year-old boy presented with a rare case of Castleman syndrome caused by a clear cell meningioma manifesting as persistent fever of unknown origin, 2 years after glomerulonephritis. Laboratory investigation of the patient showed an increased inflammatory reaction, as well as elevated polyclonal gamma globulin titer and serum level of C-reactive protein. Magnetic resonance imaging revealed a tumor at the cerebellopontine angle. Neurosurgical intervention was performed under the presumptive diagnosis of Castleman syndrome caused by intracranial tumor. Histological examination of the tumor verified that it was clear cell meningioma with infiltration of lymphoplasma cells, and surgical removal resulted in complete resolution of the patient's symptoms and biochemical abnormalities. The present case of clear cell meningioma manifesting as Castleman syndrome shows that the possibility of a brain tumor should be considered in patients presenting with fever of unknown origin, anemia, hypergammaglobulinemia, or other systemic illness.

Primary sarcomas of the CNS are rare and are sometimes associated with chronic subdural effusion (SDE). Approximately 10 patients with such presentations have been reported. The authors report the case of a 5-year-old boy with multiple subdural masses and SDE. An SDE had been diagnosed when the patient was 2 months old, and he received a subduroperitoneal shunt when he was 5 months old. Since then, he had been clinically stable and well for 5 years. When he presented with acute headache, nausea, and vomiting, a newly developed tumor was found. Near-total resection of the tumor was performed, and the mass was diagnosed as an embryonal-type rhabdomyosarcoma. The child was given radiation therapy and 13 cycles of chemotherapy and is still free of disease 13 months after surgery.

Object: This study was focused on the development of models with which to predict the occurrence of intracranial edema after Gamma Knife surgery (GKS) of meningiomas, based on clinical and imaging data collected in a large group of patients., Methods: Data in 368 patients with 381 meningiomas treated using the Leksell Gamma Knife unit were analyzed. Follow up of more than 24 months was available in 331 patients (90%); this time period ranged from 24 to 120 months (median 51 months). The actuarial tumor control rate was 97.9% at 5 years. Perilesional edema after GKS was radiologically confirmed in 51 patients (15.4%) and 32 of them (9.7%) were symptomatic; symptoms were temporary in 23 (6.9%) and permanent in nine (2.7%). Ten different factors were proposed as potential predictors for the occurrence of the intracranial edema after GKS: patient's sex, patient's age, previous surgery, edema before GKS treatment, lobulated margin of meningioma, heterogeneous appearance of the tumor, tumor volume, tumor location, maximum dose to the tumor, and dose to the tumor margin. To identify factors having influence on edema occurrence, univariate and multivariate statistical analyses were performed. There was a significant difference in the incidence of edema for different patient age groups and a significantly higher incidence of edema occurrence in patients in whom no surgical procedure was performed before GKS, those with edema present before GKS, those with a tumor volume larger than 10 cm3, those in whom the tumor was located in the anterior fossa, those in whom the maximum dose to the tumor was higher than 30 Gy, and for different tumor margin doses. A binary logistic regression multifactorial prediction model was used to identify the following significant factors to predict of edema occurrence after GKS: previous surgery, edema before the treatment, tumor volume, tumor location, and tumor margin dose., Conclusions: Based on these models estimates of the occurrence of edema after the GKS can be made, and consequently treatment parameters can be adjusted to reduce the occurrence of edema. These results may provide grounds for additional patient care such as more frequent follow up or possibly administration of steroids.

Meningiomas are the most common tumors affecting the cavernous sinus (CS). Despite advances in microsurgery and radiosurgery, treatment of CS meningiomas remains difficult and controversial. As in cases of other meningiomas, the goal of treatment for CS meningioma is long-term growth control and preservation of neural function. Gross-total resection, the ideal treatment for meningioma, is not always possible to obtain in patients with CS meningiomas with an acceptable level of morbidity. Therefore, microsurgery and radiosurgery have recently been advocated as a combined therapy to achieve good control of tumor growth and favorable functional outcome. The authors describe a technique in which tumor volume can be reduced to a minimal residual amount, while preserving cranial nerve function. This enables the smallest field to be treated radiosurgically. The optic nerve is decompressed, and the tumor mass is reduced to provide at least a 5-mm interpositional distance between the optic nerve and the residual lesion. Direct decompression of the CS, with opening of the lateral and superior sinus walls, and piecemeal removal of the tumor in "safe" locations are performed to facilitate an improvement in cranial nerve function. The authors describe the use of this technique in a series of patients and demonstrate improvement of cranial nerve function in a subset of these patients.

Object: The extent of peritumoral brain edema (PTBE) associated with meningiomas can be highly variable. The authors studied the correlation between the development of efferent venous drainage from the tumor and the degree of PTBE that occurs in intracranial meningiomas., Methods: Twenty-five patients with 27 intracranial supratentorial meningiomas were investigated to identify the correlation between the efferent venous drainage system of the tumor and peritumoral edema. The overall mean age of the patients was 54 years. Seventeen patients (68%) were female and eight (32%) were male. All patients underwent magnetic resonance imaging and digital subtraction angiography. In each meningioma, superselective angiography of the dominant feeding artery was performed, including the late venous phase to evaluate the development of the tumor's draining vein. An edema index (EI) was introduced to serve as an objective means by which to judge the extent of PTBE. Eleven meningiomas (41%), in which the mean EI was 0.14 +/- 0.10, clearly showed dominant draining veins originating from the tumor itself. In the other 16 meningiomas (59%), superselective angiography demonstrated no efferent venous drainage from the tumor, which could account for this group's mean EI value of 1.49 +/- 1.05 (p < 0.001)., Conclusions: The current results suggest that hypoplasia of the efferent draining vein from the meningioma itself contributes to PTBE formation. The development of an efferent venous system mitigates against the formation of PTBE. Intratumoral venous congestion can be considered the main cause of PTBE in meningiomas.

Although spinal meningioma is a common benign neoplasm, the ossified variant is rare. No more than 20 sporadic cases were reported in the literature between 1977 and January 2005. Recently, the authors treated a patient with a symptomatic ossified meningioma located in the posterior aspect of T-11 and an associated osteoporotic T-11 burst fracture. The tumor was completely removed by T10-11 laminectomy and transpedicular vertebroplasty was performed. The kyphotic deformity of the T-11 burst fracture was partially reduced and maintained for at least 2 months after vertebroplasty and laminectomy. The clinical presentation and management of this case are reported.

The authors present the case of a 60-year-old woman who was admitted to their institution after suffering a subarachnoid hemorrhage (SAH). Neuroimaging data demonstrated an olfactory groove meningioma surrounded by a slight edema, but there was no evidence of SAH, although results of the lumbar puncture demonstrated xanthochromic cerebrospinal fluid. Angiography confirmed the diagnosis of meningioma, but results of magnetic resonance imaging led the authors to suspect a cavernoma within the meningioma. This diagnosis was established by pathological examination of the resected lesion. The patient did well and was discharged soon after surgery. This very rare association and the propensity of each of these lesions to be revealed by hemorrhage are discussed.

Relapsing polychondritis (RP) is a rare systemic disease characterized by recurrent inflammation of the cartilaginous structures and connective tissue. Central nervous system lesions in association with RP have occasionally been reported, but intracranial mass lesions have not been described. The authors report the first such case, in which a 51-year-old man presented with parasagittal meningeal plasma cell granuloma with RP. The mass was subtotally resected and adjuvant radiotherapy was administered. The patient did not experience any recurrence of the lesion during an 8-year follow-up period. In this case, the exact diagnosis of RP was made based on symptoms of respiratory tract chondritis, which was successfully treated by the placement of tracheobronchial stents.

Object: The occurrence of meningiomas in children younger than 20 years of age is rare, accounting for less than 3% of all childhood tumors of the central nervous system. The authors of this study sought to add to the limited available information regarding clinicopathological factors that influence outcome, disease progression, and survival in children with meningiomas., Methods: Eighty-seven cases of childhood meningiomas were identified and classified according to World Health Organization (WHO) 2000 criteria. In addition to the WHO classification, the following potential prognostic factors were analyzed: age, sex, extent of resection, history of radiotherapy, diagnosis of neurofibromatosis Type 2 (NF2) or other inherited syndromes, and the presence of a comorbidity. There was a sex predilection for male patients (35 females and 52 males). Patient age ranged from 5 months to 20 years (mean 14 years). The most common clinical presentations were seizures (33%), headaches (13%), ataxia (10%), and hemiparesis (10%). Nine patients had NF2 and two had Gorlin syndrome. Seven patients had undergone radiotherapy for a prior neoplasm. Tumor location was supratentorial in 64% of the patients, infratentorial in 16%, intraventricular in 12%, and spinal in 8%. Fifty-three patients (62%) underwent gross-total resection and 28 (33%) underwent subtotal resection. Histopathological analysis revealed 62 (71%) WHO Grade I, 21 (24%) Grade II, and four (5%) Grade III meningiomas. One patient received adjuvant chemotherapy and four received radiotherapy., Conclusions: Using survival data from this unique patient cohort, the authors found that recurrence-free survival time was significantly related to WHO grade (p = 0.002), but overall survival time was not significantly linked to any of the potential prognostic factors considered in this study (p = 0.06).