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A 62-year-old man presented with a history of atypical meningioma (World Health Organization grade II) and recurrent as anaplastic meningioma (World Health Organization grade III). His previous treatments included multiple surgical resections, fractionated radiation therapy, stereotactic radiosurgery, everolimus/octreotide long-acting release, bevacizumab, and hydroxyurea. Magnetic resonance imaging revealed rapid volumetric progression over the prior 9 months, with a near tripling in size from 29.9 cm3 to 80.4 cm3. Indium In 111 octreotide scanning confirmed the presence of somatostatin receptors within the tumor. Lutetium Lu 177 dotatate was administered intravenously at a dose of 200 mCi per dose every 8 weeks for 4 cycles. Treatment was tolerated very well, with no notable adverse events. Tumor volume initially increased to 98.3 cm3 after cycle 1 of treatment and subsequently decreased to 91.2 cm3 after cycle 2. Eight months after treatment onset, the tumor volume remained stable (93.4 cm3).

Meningioma is a common benign intracranial neoplasm. The incidence of extracranial extension to other sites is rare. Due to the neglected intracranial component, the chances of an under diagnosis or a misdiagnosis of the extracranial component is there, which may adversely affect the management and therefore, the prognosis of the patient.

BACKGROUND There are examples of incongruence between the WHO grade and clinical course in meningioma patients. This incongruence between WHO grade and recurrence has led to search for other prognostic histological markers. OBJECTIVE To study the correlation between the Ki-67 proliferative index (PI), risk of recurrence, and recurrence rates in meningioma patients. METHODS We prospectively collected pathological diagnosis of de novo consecutive meningiomas. In total, we followed 159 patients with clinical controls until recurrence, death, or emigration. We estimated the correlation between risk of recurrence and Ki-67 PI when adjusted for age at diagnosis, sex, WHO grade, extent of surgical resection, and tumor location. We estimated the cumulative incidence of recurrence when considering death without recurrence a competing risk. We report recurrence rates per 100 person-years. RESULTS A 1%-point increase of Ki-67 PI yielded a hazard ratio of 1.12 (95% CI: 1.01-1.24) in a multivariate analysis. The cumulative incidence of recurrence was 3% for Ki-67 0% to 4% vs 19% for Ki-67 > 4% meningiomas after 1 yr, but 24% vs 35%, respectively, after 10 yr. There was no significant difference in mean Ki-67 PI between nonrecurrent and recurrent meningioma in a 2-sample t-test (P = .08). The strongest relationship was detected between Ki-67 PI and time to recurrence: Ki-67

Meningiomas are the most common primary intracranial tumors, but meningioma metastases are rare. Accordingly, the clinical workup, diagnostic testing, and molecular classification of metastatic meningioma is incompletely understood. Here, we present a case report of multiply recurrent meningioma complicated by liver metastasis. We discuss the patient presentation, imaging findings, and conventional histopathologic characterization of both the intracranial lesion and the metastatic focus. Further, we perform multiplatform molecular profiling, comprised of DNA methylation arrays and RNA-sequencing, of six stereotactically-guided samples from the intracranial meningioma and a single ultrasound-guided liver metastasis biopsy. Our results show that DNA methylation clusters distinguish the liver metastasis from the intracranial meningioma samples, and identify a small focus of hepatocyte contamination with the liver biopsy. Nonetheless, DNA methylation-based classification accurately identifies the liver metastasis as a meningioma with high confidence. We also find that clustering of RNA-sequencing results distinguishes the liver metastasis from the intracranial meningiomas samples, but that differential gene expression classification is confounded by hepatocyte-specific gene expression programs in the liver metastasis. In sum, this case report sheds light on the comparative biology of intracranial and metastatic meningioma. Furthermore, our results support methylation-based classification as a robust method of diagnosing metastatic lesions, underscore the broad utility of DNA methylation array profiling in diagnostic pathology, and caution against the routine use of bulk RNA-sequencing for identifying tumor signatures in heterogeneous metastatic lesions.

A 42-year-old female patient with a history of cervicothoracic junction meningioma World Health Organization grade I, resected in early 2011, was admitted to the hospital with intractable headache and lower extremity weakness. Magnetic resonance imaging (MRI) showed an epidural mass compressing the spinal cord at the prior surgical site, which was interpreted as recurrent meningioma. Following surgical resection, histopathological analysis revealed desmoid fibromatosis (desmoid tumor). In retrospect, dynamic contrast-enhanced magnetic resonance imaging performed prior to surgery should have allowed for prospective exclusion of meningioma as the recurrent mass and suggested an alternative diagnosis such as post-traumatic desmoid fibromatosis or the need for biopsy to confirm diagnosis.

Meningiomas are the most common intracranial extra-axial tumor. While the literature is abundant on the therapeutic management of meningioma recurrence after the initial surgery, the natural history of repeated recurrences is poorly described, as well as and their respective management. A partly retrospective, partly prospective review was conducted in a Norwegian cohort of 1469 consecutive cases of meningioma surgically treated, totaling 11 414 patient-years of follow-up. 114 recurrences (7.7%) were treated surgically with a risk a surgical retreatment of 1% per patient-year of follow-up. 36 patients were operated on 3 times or more. The time-to-retreatment (TTR) decreased significantly and steadily between surgeries, from 4.3 ± 4 years after the first surgery to 2.4 ± 2.9 years after the third surgery. The primary driver for recurrence was the WHO grade (OR 7.13 [4.40;11.55], p

Treatment for recurrent intracranial neoplasms is often difficult and less standardized. Since its approval by the Food & Drug Administration (FDA), GammaTileTM (GT, GT Medical Technologies, Tempe, AZ), a novel collagen tile cesium brachytherapy, has been investigated for use in this population. This study presents the initial experience with the use of GT for patients with recurrent intracranial neoplasms at a tertiary referral center. A retrospective analysis of all patients with GT implantation from November 2019 to July 2021 was performed. Information regarding demographics, clinical history, imaging data, prior tumor treatment, dosing, surgical complications, and outcomes was collected. Twelve patients were included in this study. Pathologies included gliomas (five patients), meningiomas (five patients), and metastatic tumors (two patients). The median tumor volume treated was 24 cc (IQR: 21.2 - 31.3 cc), and patients had a median of 7.5 tiles implanted (IQR: 5.4 - 10.3). One patient had a delayed epidural hematoma requiring reoperation, which was unrelated to GT implantation. Median follow-up was seven months (IQR: 3 -10), with the longest follow-up time of 20 months. Two patients have had local disease recurrence and three patients have had systemic progression of their disease. Three patients are deceased with survivals of 2.9, 4.8, and 5.8 months. Collagen tile brachytherapy is a safe and viable option for patients with recurrent intracranial tumors. Our data are consistent with early results seen at other institutions. Long-term data with larger patient populations are required to assess efficacy, safety, and indications for the use of this novel technology.

PURPOSE OF REVIEW Although most meningiomas are slow growing tumors mainly controlled by surgery with or without radiotherapy, aggressive meningiomas that fail these conventional treatments constitute a rare situation, a therapeutic challenge and an unmet need in neuro-oncology. RECENT FINDING Mutational landscape in recurrent high-grade meningiomas includes mainly NF2 mutation or 22q chromosomal deletion, whereas telomerase reverse transcriptase promoter, BAP-1 and CDK2NA mutations were also found in aggressive meningiomas. Pi3K-Akt-mTOR pathway is currently the most relevant intracellular signaling pathway target in meningiomas with preliminary clinical activity observed. Assessment of drug activity with progression free survival rate at 6 months is challenging in regard to meningioma growth rate heterogeneity, so that 3-dimensional growth rate before and during treatment could be considered in the future to selected new active drugs. SUMMARY Despite a low evidence level, some systemic therapies may be considered for patients with recurrent meningioma not amenable to further surgery or radiotherapy. In recurrent high-grade meningioma, everolimus-octreotide combination, bevacizumab, sunitinib and peptide receptor radionuclide therapy exhibit a signal of activity that may justify their clinical use. Despite a lack of clear signal of activity to date, immunotherapy may offer new perspectives in the treatment of these refractory tumors.

Simple Summary This study investigated patients who underwent more than one operation for a meningioma, a type of brain tumor. Currently, there is little evidence available for this specific patient group. The purpose of this study was to determine if patients had an improvement or deterioration following a second operation for a recurrent meningioma, and to identify any factors that may influence this change. The results demonstrated that following a second operation for meningioma, patients have poorer outcomes. The findings of this study provide supporting information for surgeons and patients, thereby informing decisions related to patient care and re-operation. Abstract The outcomes following re-operation for meningioma are poorly described. The aim of this study was to identify risk factors for a performance status outcome following a second operation for a recurrent meningioma. A retrospective, comparative cohort study was conducted. The primary outcome measure was World Health Organization performance. Secondary outcomes were complications, and overall and progression free survival (OS and PFS respectively). Baseline clinical characteristics, tumor details, and operation details were collected. Multivariable binary logistic regression was used to identify risk factors for performance status outcome following a second operation. Between 1988 and 2018, 712 patients had surgery for intracranial meningiomas, 56 (7.9%) of which underwent a second operation for recurrence. Fifteen patients (26.8%) had worsened performance status after the second operation compared to three (5.4%) after the primary procedure (p = 0.002). An increased number of post-operative complications following the second operation was associated with a poorer performance status following that procedure (odds ratio 2.2 [95% CI 1.1–4.6]). The second operation complication rates were higher than after the first surgery (46.4%, n = 26 versus 32.1%, n = 18, p = 0.069). The median OS was 312.0 months (95% CI 257.8–366.2). The median PFS following the first operation was 35.0 months (95% CI 28.9–41.1). Following the second operation, the median PFS was 68.0 months (95% CI 49.1–86.9). The patients undergoing a second operation for meningioma had higher rates of post-operative complications, which is associated with poorer clinical outcomes. The decisions surrounding second operations must be balanced against the surgical risks and should take patient goals into consideration.

BACKGROUND Meningioma is the commonest primary brain tumour. Despite surgery, meningiomas can recur. Surgery is usually the first line treatment for recurrent meningioma. The aim was to determine the risk factors associated with clinical outcomes (performance status, morbidity, mortality, recurrence) following re-operation for recurrence of intracranial meningioma. MATERIAL AND METHODS Retrospective cohort study (1998–2018). Eligible patients had reoperation for local recurrence of a previously operated meningioma. Collected data included baseline clinical and imaging characteristic. Primary outcome measure was performance status after each reoperation. Secondary outcome measures were medical and surgical morbidity, recurrence-free survival (RFS) and overall survival (OS). RESULTS Fifty-eight patients were eligible (37 female, mean age at 1st re-operation 56.1 years (SD=11.6)). Eleven patients (19.6%) had 2 re-operations and 3 patients (5.4%) had 3 re-operations. Median follow up was 128.5 months (IQR=73–194.5). Median time to 1st recurrence and 1st re-operation were 36.5 (IQR=24.3–81.0) and 43.8 months (IQR=20.3–103.4), respectively. Fifteen patients (26.8%) had worse performance status after 1st reoperation, compared to 5.4% (n=3) after the primary operation. Complication rate was 32.1% (n=18) after the primary operation compared to 48.2% (n=27) after 1st reoperation. At primary operation, there were 29 (51.8%) grade 1, 24 (42.9%) grade 2, and 1 (1.8%) grade 3 tumours. Median RFS after first re-operation was 36.5 months (95% CI 29.3–43.9). Median OS was 312 months (95 % CI 257.8–366.2). Increased number of post-operative complications were a risk factor for worsened performance status following reoperation (OR 2.2 [95% CI 1.1–4.6], P=0.029). CONCLUSION Re-operation is associated with a worse performance status and increased risk of complications. Re-operating meningiomas for radiological recurrence without symptoms increases patient morbidity. Shared-care management decision should be made with patients when considering operating for radiological recurrence only.

Meningiomas are the most common primary central nervous system tumors, as they can account for up to one-third of all primary brain tumors. Most meningiomas are benign, although up to one-fourth of such tumors are classified as atypical or malignant. Atypical and malignant meningiomas are associated with an increased risk of local recurrence and decreased overall survival. Our patient is a 57-year-old male with a history of recurrent malignant meningioma, with metastasis to the liver. He underwent multiple surgical interventions, radiation treatments, and systemic therapies for a malignant meningioma, ultimately requiring transfer to hospice care. Not only did a positive novel coronavirus (COVID-19) infection delay his ability to receive radiation therapy, the infection in itself may have had an impact on the course of care for this patient. Treatment targeting the patient's COVID-19 infection may have suppressed the immune system, and as a result, caused the progression of metastatic disease. Palliative care was needed in the setting of losing all functional goals for quality of life due to malignant neoplasm.

Background En plaque meningiomas are a rare subtype of meningiomas that are frequently encountered in the spheno-orbital region. Characterized by a hyperostotic and dural invasive architecture, these tumors present unique diagnostic and treatment considerations. Objective The authors conduct a narrative literature review of clinical reports of en plaque meningiomas to summarize the epidemiology, clinical presentation, diagnostic criteria, and treatment considerations in treating en plaque meningiomas. Additionally, the authors present a case from their own experience to illustrate its complexity and unique features. Methods A literature search was conducted using the MEDLINE database using the following terminology in various combinations: meningioma, meningeal neoplasms, en plaque, skull base, spheno-orbital, and sphenoid wing. Only literature published in English between 1938 and 2018 was reviewed. All case series were specifically reviewed for sufficient data on treatment outcomes, and all literature was analyzed for reports of misdiagnosed cases. Conclusion En plaque meningiomas may present with a variety of symptoms according to their location and degree of bone invasion, requiring a careful diagnostic and treatment approach. While early and aggressive surgical resection is generally accepted as the optimal goal of treatment, these lesions require an individualized approach, with further investigation needed regarding the role of new therapies.

Introduction Several radiolabeled somatostatin analogues have been developed for molecular imaging of neuroendocrine tumors (NETs) with single-photon emission computed tomography (SPECT) and positron-emission tomography (PET). The aim of the present study was to report our first results using 64Cu-DOTATOC in patients with NETs. Methods Thirty-three patients with NETs (15 female, 18 male; mean age 64 ± 13 years) were included in this retrospective study. 64Cu-DOTATOC PET-CT scans were performed on all patients. Results Five out of 33 patients with a history of NET after surgical removal of the primary lesion showed no pathological lesions on PET-CT imaging and 8/33 patients had enhanced uptake in the area of recurrent meningioma at the skull base. The remaining 20/33 patients had a history of neuroendocrine tumor in the gastrointestinal tract (GEP-NET) and were presented with at least one pathological lesion. Conclusion The high detection rate of suspected lesions in patients with NETs and the high target-to-background contrast found in this study hold promise for the safe application of 64Cu-DOTATOC in patients with NET.

OBJECTIVERecurrent meningiomas are primarily managed with radiation therapy or repeat resection. Surgical morbidity after reoperation for recurrent meningiomas is poorly understood. Thus, the objective of this study was to report surgical outcomes after reoperation for recurrent non–skull base meningiomas.METHODSA retrospective review of patients was performed. Inclusion criteria were patients with recurrent meningioma who had prior resection and supratentorial non–skull base location. Univariate and multivariate logistic regression and recursive partitioning analysis were used to identify risk factors for surgical complications.RESULTSThe authors identified 67 patients who underwent 111 reoperations for recurrent supratentorial non–skull base meningiomas. The median age was 53 years, 49% were female, and the median follow-up was 9.8 years. The most common presenting symptoms were headache, weakness, and seizure. The WHO grade after the last reoperation was grade I in 22% of cases, grade II in 51%, and grade III in 27%. The tumor grade increased at reoperation in 22% of cases. Tumors were located on the convexity (52%), parasagittal (33%), falx (31%), and multifocal (19%) locations. Tumors involved the middle third of the sagittal plane in 52% of cases. In the 111 reoperations, 48 complications occurred in 32 patients (48%). There were 26 (54%) complications requiring surgical intervention. There was no perioperative mortality. Complications included neurological deficits (14% total, 8% permanent), wound dehiscence/infection (14%), and CSF leak/pseudomeningocele/hydrocephalus (9%). Tumors that involved the middle third of the sagittal plane (OR 6.97, 95% CI 1.5–32.0, p = 0.006) and presentation with cognitive changes (OR 20.7, 95% CI 2.3–182.7, p = 0.001) were significantly associated with complication occurrence on multivariate analysis. The median survival after the first reoperation was 11.5 years, and the 2-, 5-, and 10-year Kaplan-Meier survival rates were 91.0%, 68.8%, and 50.0%, respectively.CONCLUSIONSReoperation for recurrent supratentorial non–skull base meningioma is associated with a high rate of complications. Patients with cognitive changes and tumors that overlap the middle third of the sagittal plane are at increased risk of complications. Nevertheless, excellent long-term survival can be achieved without perioperative mortality.

BackgroundAstroblastoma (ABM) is a rare glial brain tumor. Recurrent meningioma 1 (MN1) alterations have been recently identified in most pediatric cases. Adolescent and adult cases, however, remain molecularly poorly defined.Materials and MethodsWe performed clinical and molecular characterization of a retrospective cohort of 14 adult and 1 adolescent ABM.ResultsStrikingly, we found that MN1 fusions are a rare event in this age group (1/15). Using methylation profiling and targeted sequencing, most cases were reclassified as either pleomorphic xanthoastrocytomas (PXA)-like or high-grade glioma (HGG)-like. PXA-like ABM show BRAF mutation (6/7 with V600E mutation and 1/7 with G466E mutation) and CD34 expression. Conversely, HGG-like ABM harbored specific alterations of diffuse midline glioma (2/5) or glioblastoma (GBM; 3/5). These latter patients showed an unfavorable clinical course with significantly shorter overall survival (p = .021). Mitogen-activated protein kinase pathway alterations (including FGFR fusion, BRAF and NF1 mutations) were present in 10 of 15 patients and overrepresented in the HGG-like group (3/5) compared with previously reported prevalence of these alterations in GBM and diffuse midline glioma.ConclusionWe suggest that gliomas with astroblastic features include a variety of molecularly sharply defined entities. Adult ABM harboring molecular features of PXA and HGG should be reclassified. Central nervous system high-grade neuroepithelial tumors with MN1 alterations and histology of ABM appear to be uncommon in adults. Astroblastic morphology in adults should thus prompt thorough molecular investigation aiming at a clear histomolecular diagnosis and identifying actionable drug targets, especially in the mitogen-activated protein kinase pathway.Implications for PracticeAstroblastoma (ABM) remains a poorly defined and controversial entity. Although meningioma 1 alterations seem to define a large subset of pediatric cases, adult cases remain molecularly poorly defined. This comprehensive molecular characterization of 1 adolescent and 14 adult ABM revealed that adult ABM histology comprises several molecularly defined entities, which explains clinical diversity and identifies actionable targets. Namely, pleomorphic xanthoastrocytoma-like ABM cases show a favorable prognosis whereas high-grade glioma (glioblastoma and diffuse midline gliome)-like ABM show significantly worse clinical courses. These results call for in-depth molecular analysis of adult gliomas with astroblastic features for diagnostic and therapeutic purposes.

Meningiomas are the most common tumours of the central nervous system in adults. In clinical routine, their diagnostic workup prior to subsequent therapies such as resection and radiotherapy usually consists of contrast-enhanced MRI and CT of the brain. However, there are several diagnostic uncertainties in the clinical workup, which standard morphological imaging fails to resolve. Molecular imaging has an emerging role for the diagnosis of meningiomas, which characteristically show high expression of the somatostatin receptor subtype 2 (SSR). PET imaging with selective ligands can visualize and quantify this expression against low background signal in healthy brain. Moreover, SSR-directed radioligands labeled with beta-emitters can be effective for radiopeptide therapy (RPT) in patients with recurrent or refractory meningioma. A literature search on the PubMed literature database was conducted using the terms “meningioma”, “PET”, “somatostatin receptor”, “SS(T)R”, “DOTATATE”, “DOTATOC”, “Radiopeptide therapy”, “imaging”, “therapy” alone and in combination, extending until February 2019. The search results were augmented by the authors’ own literature files. We summarize the current state of SSR-directed imaging in patients with meningioma regarding the distinct clinical applications for initial diagnosis, differential diagnosis, surgery and radiotherapy planning. Our review also summarizes SSR imaging for the differentiation of recurrent meningioma tissue from post-therapeutic changes within the individual follow-up. Moreover, we discuss the clinical value and place of SSR-directed RPT in patients with refractory or recurrent meningioma. Molecular imaging with SSR-directed radioligands contributes to the diagnostic work-up of meningioma patients by providing information that is absent from structural MR or CT imaging. Targeted SSR RPT offers well-tolerated treatment options in patients with refractory or recurrent meningioma.

Objective Meningiomas are among the most frequent intracranial tumors. Although the majority of meningiomas can be cured by surgical resection, up to 20% of the patients develop an aggressive clinical course with tumor recurrence or progressive disease. Cytogenetically, meningiomas frequently harbour a normal karyotype or monosomy of chromosome 22 as the sole anomaly. However, progression of meningiomas is associated with a non-random pattern of secondary losses of the chromosomes and chromosomal regions 1p, 6, 10, 14, 18, and 19. There is evidence, that loss of chromosome 17 might be involved in the clonal cytogenetic evolution of recurrent meningiomas. The aim of this study was to determine the role of deletions in the 17q chromosomal region in patients with recurrent meningiomas. Results The authors retrospectively reviewed all patients that underwent repeated surgery for recurrent meningiomas between 1999 and 2015 at the Department of Neurosurgery of the Saarland University Hospital. Patients were included in this study if tumor samples from two or more different meningiomas were available. A total of 7 patients underwent repeated surgery for recurrent meningiomas (4 males, 3 females, mean age: 45.4 years at the date of surgery) between 1999 and 2015. Collectively, 22 biopsies were analyzed with FISH (fluorescence-in-situ-hybridization) for the chromosomal region 17q23.3. In 20/22 (90.1%) specimens, the tumor samples harboured a significant deletion in the chromosomal region 17q (range: 10 to 63% of the cells). In 3/3 (100%) cases, deletion in the 17q chromosomal region was detectable in the primary tumor. In the tumor evolution, there was no steady in- or decrease in the percentage of this deletion. Conclusion Deletion in the 17q chromosomal region was present in the patients’ primary tumors as well as in late recurrences. Overall, a significant deletion in the 17q chromosomal region was detected in 90.1% of the tumors. Thus, the authors assume that deletion in the 17q chromosomal region displays rather an early event in meningioma progression. Accordingly, deletion in the 17q chromosomal region might clinically serve as a potential early marker for malignancy and a higher risk for recurrence in meningiomas.

BACKGROUND: Recurrent atypical and malignant meningiomas have poor outcomes with surgical therapy alone. Neither adjuvant chemotherapy nor postoperative radiation therapy remedies this problem. OBJECTIVE: To evaluate our experience with the treatment of 15 patients treated with I-125 or Cs-131 brachytherapy radiation seeds as an adjuvant in these difficult cases. METHODS: Patients with high-grade recurrent meningioma who underwent resection and intraoperative placement of brachytherapy seeds at our institution from 2002 to 2014 were identified and studied by retrospective chart review. RESULTS: Fifteen patients with median age of 68.8 yr were treated with I-125 (n = 13) or Cs-131 (n = 2) brachytherapy seeds for cases of recurrent, grade II (n = 8), or grade III (n = 7) meningioma at our institution from 2002 to 2014. These lesions originated from a variety of locations including, convexity (3), falcine (3), frontal (2), occipital (1), parietal (2), 2 sphenoid wing (2), and temporal (2), based recurrent meningiomas. Patients had a median of 2 prior open surgical interventions and received local radiation therapy with a median dose of 55 Gy prior to brachytherapy. Survival at 2.5 yr was 56% for grade II and 17% for grade III lesions. Survival was significantly associated with patient age but not tumoral pathology. Forty percent of patients required reoperations for wound complications following brachytherapy. CONCLUSION: Brachytherapy with implantation of permanent radiation seeds provides a viable alternative treatment for recurrent meningioma while carrying a significant clinical risk of wound infection and need for reoperation.

Background The endoscopic endonasal approach to the infratemporal fossa (ITF) has gained popularity. However, the inferior turbinate and/or lacrimal duct are often removed when performing endoscopic medial maxillectomy for ITF approach, and there is potential risk for empty nose syndrome or epiphora. Although the endoscopic prelacrimal recess approach was introduced to avoid these complications, there were some limitations associated with surgical freedom. We report a 2-port endoscopic technique using both prelacrimal recess and antral window as a means to preserve the inferior turbinate and lacrimal duct, while facilitating instrument availability during ITF tumor resection. Methods We retrospectively reviewed 3 patients between September 2016 and May 2018 who were treated with a modified 2-port technique for ITF tumors. Results There was 1 case of trigeminal schwannoma originating in the mandibular nerve, 1 recurrent meningioma, and 1 paraganglioma. The 2-port technique was not initially planned in these 3 cases, but it was decided to use the technique during surgery because tumors were extensively attached to surrounding muscles and had profuse bleeding. After tumor resection, sinonasal anatomy including inferior turbinate and lacrimal duct was well preserved. Conclusions We propose a hybrid endoscopic surgical procedure for ITF tumors using both endoscopic prelacrimal recess approach and transantral window. This technique provides surgeons an adequate working space via a bimanual technique through 2 different ports, while preserving normal sinonasal structures.

Background: Extracranial metastasis from intracranial meningioma is a very rare condition. A current literature review reveals that only few cases are documented with extensive pulmonary involvement >10 years after initial intracranial meningioma resection. Diagnosis of pulmonary meningioma is often confirmed by computed tomography chest-guided core biopsies. The prognosis of extensive metastatic pulmonary meningioma, however, is unknown and there is no gold standard treatment option. Case Description: We present a case of multiple pulmonary meningioma metastases developing 13 years after initial resection of left occipital parafalcine World Health Organization Grade I intracranial meningioma. Conclusion: There are no established guidelines for the optimal management or surveillance of extensive pulmonary metastatic meningioma. In patients with high-grade meningioma and multiple cannonball pulmonary lesions, metastatic meningioma should be considered as part of the differential diagnosis. Metastatic meningioma may occur even a decade after initial tumour resection.

Meningiomas are primary CNS tumors that arise from the arachnoid layer of the meninges. Genomic sequencing has revealed that NF2 mutations are the most common genetic alteration seen in meningiomas. Meningiomas although usually low grade, can sometimes progress to high grade. A patient who had several recurrences of meningiomas since childhood presented with recurrent headaches. Imaging showed that he had another recurrence of a meningioma. He underwent surgery for resection of the meningioma and histopathology showed NF2 mutation. He was started on everolimus and bevacizumab with good effect. Studies have shown that NF-2 mutated meningiomas have a good response to everolimus and bevacizumab with increased progression-free survival time and progression-free survival time at 6 months.

Background: Over 200 human telomerase reverse transcriptase (hTERT) polymorphism combinations have been implicated in the development of cancer. This study aimed to evaluate hTERT mutations in meningioma tissue and its association with meningioma. Material and Methods: A total of 90 patients who underwent surgery between 2006 and 2015 and were histopathologically diagnosed with meningioma (WHO 2016) were included. Results: Among the 90 participants included herein, 50 (55.5%) and 40 (44.5%) were female and male, respectively, with an average age of 56.2 ± 14 years. Mean Ki-67 values were 10.56% (SD 12.41, range 0-60), while the mean follow-up duration was 39.1 months (SD 26.3). Low- and high-grade patients had a mean Ki-67 score of 4.31% (SD 3.58, range 0-16) and 19.92% (SD 14.91, range 2-60) (p = 0.0001). Our results showed a moderate positive correlation between Ki-67 score and the presence of hTERT mutation (Pearson correlation test, r = 0.5161; p = 0.0001). Patients with an hTERT mutation > 30% had significantly higher risk for reoperation than those with lower levels of mutation (p = 0.016, chi square test). None of the patients requiring reoperation had an hTERT mutation < 10%. Moreover, high-grade patients had a 7.2 times higher risk of reoperation than those with an hTERT mutation > 30%. Conclusion: The presence of hTERT mutation, in addition to high Ki-67, indicated a more aggressive meningioma disease course and potentially increased risk of recurrence.

BACKGROUND Medical therapies for recurrent brain tumors are limited. Abemaciclib is a small molecule CDK4/6 inhibitor that has demonstrated antitumor activity in multiple cancer types and crosses the blood-brain barrier. METHODS We conducted a phase II trial of single-agent abemaciclib in patients with recurrent primary brain tumors utilizing a novel CNS basket trial design with multiple tumor types accrued to separate cohorts including patients with recurrent IDH-wildtype gliomas (Cohort A), any recurrent gliomas requiring cytoreductive surgery (Cohort B), and any other recurrent primary brain tumors (Cohort C) including IDH-mutant gliomas, meningiomas, and other tumor types. In all patients, abemaciclib was administered orally at 200mg twice daily for each 28-day cycle. In cohort B abemaciclib was administered 4-7 days prior to surgery then resumed after recovery. Neuroimaging disease assessments were performed every two cycles. Cohorts were individually assessed for efficacy, tumoral molecular characteristics, and exploratory biomarker analyses. Next generation sequencing was performed on patients who had prior surgery. RESULTS To date, a total of 61 patients have enrolled and initiated treatment with abemaciclib. Cohort A enrolled 9 patients with IDH-wildtype WHO grade II and III astrocytomas. Cohort B enrolled 10 patients with astrocytomas of varying IDH-status. Cohort C is a diverse group of 42 patients including 22 treatment-refractory meningiomas, 10 IDH-mutant gliomas (5 astrocytomas, 5 oligodendrogliomas), 3 ependymomas, 3 primary CNS lymphomas, 2 pituitary tumors, 1 glioneuronal rosette forming tumor, and 1 diffuse midline glioma. A total of 7 grade 3 toxicities occurred in 6 patients: fatigue (3), neutropenia (2), colitis (1) and seizure (1); no grade 4 toxicities occurred. CONCLUSIONS We present the results of a novel CNS basket trial looking at the efficacy of abemaciclib across multiple recurrent primary brain tumors. Efficacy results will be presented, highlighting an update on promising results in the 22 patients with recurrent meningiomas.

Aims: This retrospective observational study documents our experience in the CyberKnife® treatment of relapsed atypical meningioma (WHO Grade II) and their response to irradiation via this technique.

Meningiomas are common intracranial tumors that can be treated successfully in most cases with surgical resection and/or adjuvant radiotherapy. However, approximately 20% of patients show an aggressive clinical course with tumor recurrence or progressive disease, resulting in significant morbidity and increased mortality. Despite several studies that have investigated different cytotoxic agents in aggressive meningiomas in the past several years, limited evidence of efficacy and clinical benefit has been reported thus far. Novel molecular alterations have been linked to a particular clinicopathological phenotype and have been correlated with grading, location, and prognosis of meningiomas. In this regard, SMO, AKT, and PIK3CA mutations are typical of anterior skull base meningiomas, whereas KLF4 mutations are specific for secretory histology, and BAP1 alterations are common in progressive rhabdoid meningiomas. Alterations in TERT, DMD, and BAP1 correlate with poor outcomes. Moreover, some actionable mutations, including SMO, AKT1, and PIK3CA, regulate meningioma growth and are under investigation in clinical trials. PD-L1 and/or M2 macrophage expression in the microenvironment provides evidence for the investigation of immunotherapy in progressive meningiomas.