scholarly journals MNG-07 A CASE OF TRANSFORMED ANAPLASTIC MENINGIOMA IN 18 YEARS

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii36-ii36
Author(s):  
Yu Fujii ◽  
Toshihiro Ogiwara ◽  
Daisuke Yamazaki ◽  
Kohei Nagamine ◽  
Tetsuyoshi Horiuchi
Keyword(s):  
Malignant Transformation ◽  
Histopathological Diagnosis ◽  
Ki 67 ◽  
Who Grade ◽  
Anaplastic Meningioma ◽  
Sagittal Sinus ◽  
Simpson Grade ◽  
Perifocal Edema ◽  
Consciousness Disturbance ◽  
Surgical Manipulation

Abstract INTRODUCTION Although 95% of meningiomas are benign tumor (WHO grade I), they rarely exist malignant transformation. It is well known that WHO II or III meningiomas rapidly grow and recurrent frequently. However, there are no common sense about duration of malignant transformation between previous reports. Here we report a case of anaplastic meningioma which showed malignant transformation in 18 years. CASE The patient was 77-year-old man. He was pointed out as having a 20-mm mass in the left occipital convexity at brain checkup 19 years before. After 6 months, the mass was totally removed (Simpson grade I) at previous hospital. Histopathological diagnosis revealed meningioma with psammoma body, whose Ki-67 index was below 1 %. Postoperative course was good and periodic follow up was abandoned. The patient presented with consciousness disturbance and right hemiparesis after 18 years from the first operation. CT scan showed a 30-mm mass with intratumoral hemorrhage and perifocal edema in the left occipital lesion. Tumor removal was performed. The border of the tumor was clear and the macroscopic view was compatible with meningioma at intraoperative findings. The tumor was removed as much as possible, but partially remained which were extended to superior sagittal sinus (Simpson grade III). Histopathological findings were anaplastic meningioma with sarcoid change, whose Ki-67 index was 70%. His neurological abnormality improved postoperatively. DISCUSSION In the previous report, anaplastic meningioma is a rare tumor, which is 1% of whole meningioma. The rate of malignant transformation is reported 12–38%. The cause of malignant transformation is reported to be radiation therapy, mechanical stimulation by surgical manipulation, viral infection, and loss of chromosome. Although recurrence rate is low at total resection of meningioma, there rarely exists malignant transformation after a long period of time like our case.

scholarly journals PATH-05. A CASE OF PILOCYTIC ASTROCYTOMA HARBORING THE FGFR1 GENE MUTATION WITH A PREDOMINANT OLIGODENDROGLIOMA-LIKE COMPONENT

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii425-iii425
Author(s):  
Nobuyoshi Sasaki ◽  
Tomohiro Chiba ◽  
Kuniaki Saito ◽  
Keiichi Kobayashi ◽  
Yoshiaki Shiokawa ◽  
...  
Keyword(s):  
Pilocytic Astrocytoma ◽  
Parietal Lobe ◽  
Karnofsky Performance Status ◽  
Performance Status ◽  
Histopathological Diagnosis ◽  
Diagnostic Challenge ◽  
Ki 67 ◽  
Who Grade ◽  
Idh1 R132h ◽  
Perifocal Edema

Abstract Pilocytic astrocytomas rarely present with oligodendroglioma-like morphological features, which gives rise to a diagnostic challenge. In this report we present a case of pilocytic astrocytoma harboring the FGFR1 mutation, accompanied with a predominant oligodendroglioma-like component, thus initially diagnosed as oligodendroglioma. A 14-year-old female presented with syncope and simple partial seizure involving her right upper limb. Contrast-enhanced MRI revealed an enhancing lesion with substantial cystic portion and perifocal edema in the left parietal lobe. Open surgery was performed and a gross total resection of the tumor was achieved. On initial histopathological diagnosis, tumor cells with monotonous round nuclei and perinuclear halo predominated with branching capillaries, which were strongly suggestive for oligodendroglioma. Immunohistochemically, IDH1 R132H was negative, and Ki-67 index was around 5%. The patient was thus initially diagnosed as oligodendroglioma, WHO grade II, based on the 2007 WHO classification criteria. However, histopathological re-review revealed a minor astrocytic component with Rosenthal fibers and rare eosinophilic granular bodies, thus the diagnosis was changed as pilocytic astrocytoma. FGFR1 K654E mutation was confirmed by Sanger sequencing. Although she postoperatively developed mild sensory disturbance in her right hands, finger agnosia, and left-right disorientation, her symptoms had gradually improved, and she was discharged on day 17 with a Karnofsky performance status (KPS) of 90 and no cognitive decline. Without any adjuvant therapies, she has remained recurrence-free for 85 months. While the diagnosis of pilocytic astrocytoma with predominant oligodendroglioma-like component can be challenging, analysis of IDH1 and FGFR1 mutations can be beneficial in certain cases.

scholarly journals Multiple Pulmonary Metastases following Total Removal of a Bilateral Parasagittal Meningioma with Complete Occlusion of the Superior Sagittal Sinus: Report of a Case

2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Masataka Nakano ◽  
Toshihide Tanaka ◽  
Aya Nakamura ◽  
Mitsuyoshi Watanabe ◽  
Naoki Kato ◽  
...  
Keyword(s):  
Pulmonary Metastases ◽  
Superior Sagittal Sinus ◽  
Labeling Index ◽  
Ki 67 ◽  
Who Grade ◽  
Lung Lesions ◽  
Sagittal Sinus ◽  
Arterial Lumen ◽  
Hypervascular Tumor ◽  
Parasagittal Meningioma

Pulmonary metastases of benign meningiomas are extremely rare. The case of a 34-year-old man with bilateral parasagittal meningioma who developed pulmonary metastases is described. The meningioma was an enormous hypervascular tumor with invasion of the superior sagittal sinus. The tumor was resected completely and histologically diagnosed as transitional meningioma. The Ki-67 labeling index was 5%. Four months after operation, the patient subsequently developed bilateral multiple lung lesions later identified as metastases. The lung lesions were partially removed surgically and histologically diagnosed as meningothelial meningioma WHO grade I. The Ki-67 labeling index was 2%. The histological findings demonstrated that the tumor occupied the arterial lumen and the perivascular space, suggesting that pulmonary tumors might metastasize via the vascular route. The histopathological features and mechanisms of metastasizing meningiomas are reviewed and discussed.

Novel histopathological classification of meningiomas based on dural invasion

2020 ◽  
pp. jclinpath-2020-206592
Author(s):  
Makoto Murase ◽  
Ryota Tamura ◽  
Yuki Kuranari ◽  
Mizuto Sato ◽  
Kentaro Ohara ◽  
...  
Keyword(s):  
Brain Parenchyma ◽  
Ki 67 ◽  
Who Grade ◽  
Histopathological Classification ◽  
Simpson Grade ◽  
Grade Ii ◽  
Meningioma Recurrence

AimsHistological invasion into the adjacent brain parenchyma is frequently investigated in meningioma because it is an important morphological criterion for grade II meningioma according to the 2016 WHO classification. However, few studies have focused on dural invasion of meningiomas. Herein, we propose a novel histopathological classification based on dural invasion of meningiomas.MethodsForty-nine cases with WHO grade I meningiomas who underwent Simpson grade I removal were collected. After the meningeal layer (ML) and periosteal layer (PL) of dura mater were visualised by Masson’s trichrome stain, we evaluated the depth (to the ML and PL) and the patterns (1, expanding; 2, infiltrating) of dural invasion of meningiomas using serial paraffin sections. Invasion-associated markers, including Ki-67, matrix metalloproteinase (MMP)-1, MMP-9 and MMP-13, aquaporin 1 and Na-K-2Cl cotransporter, were quantitatively analysed by immunohistochemistry.ResultsThirty-five cases (71.4%) showed the dural invasion. In 27 of these 35 cases (77.1%), dural invasion was localised in ML. Type 1 (expanding type) and type 2 (infiltrating type) invasions were observed in 23 and 12 cases, respectively. The recurrence rate in cases with type 2 invasion was significantly higher than that in cases with type 1 invasion. The percentage of MMP-1-positive tumour cells was also significantly higher in cases with dural invasion than those without, suggesting involvement of MMP-1 in dural invasion.ConclusionsWe quantitatively evaluated the depth and patterns of dural invasion in meningiomas. The patterns of dural invasion were associated with meningioma recurrence.

scholarly journals Prognostic Significance of Preoperative Neutrophil-to-Lymphocyte Ratio in Patients With Meningiomas

2020 ◽  
Vol 10 ◽  
Author(s):  
Yuki Kuranari ◽  
Ryota Tamura ◽  
Noboru Tsuda ◽  
Kenzo Kosugi ◽  
Yukina Morimoto ◽  
...  
Keyword(s):  
Prognostic Significance ◽  
Laboratory Data ◽  
Progression Free Survival ◽  
Cost Effective ◽  
Tumor Location ◽  
Neutrophil To Lymphocyte Ratio ◽  
Ki 67 ◽  
Who Grade ◽  
Lymphocyte Ratio ◽  
Subtotal Removal

BackgroundMeningiomas are the most common benign intracranial tumors. However, even WHO grade I meningiomas occasionally show local tumor recurrence. Prognostic factors for meningiomas have not been fully established. Neutrophil-to-lymphocyte ratio (NLR) has been reported as a prognostic factor for several solid tumors. The prognostic value of NLR in meningiomas has been analyzed in few studies.Materials and MethodsThis retrospective study included 160 patients who underwent surgery for meningiomas between October 2010 and September 2017. We analyzed the associations between patients’ clinical data (sex, age, primary/recurrent, WHO grade, extent of removal, tumor location, peritumoral brain edema, and preoperative laboratory data) and clinical outcomes, including recurrence and progression-free survival (PFS).ResultsForty-four meningiomas recurred within the follow-up period of 3.8 years. WHO grade II, III, subtotal removal, history of recurrence, Ki-67 labeling index ≥3.0, and preoperative NLR value ≥2.6 were significantly associated with shorter PFS (P < 0.001, < 0.001, 0.002, < 0.001, and 0.015, respectively). Furthermore, NLR ≥ 2.6 was also significantly associated with shorter PFS in a subgroup analysis of WHO grade I meningiomas (P = 0.003). In univariate and multivariate analyses, NLR ≥2.6 remained as a significant predictive factor for shorter PFS in patients with meningioma (P = 0.014).ConclusionsNLR may be a cost-effective and novel preoperatively usable biomarker in patients with meningiomas.

scholarly journals Pilocytic Midbrain Astrocytoma Presenting with Fresh Bleed after Twenty-one-years Survival Following First Surgery: A Unique Case of Longest Brainstem Glioma Survival

2016 ◽  
Vol 07 (S 01) ◽  
pp. S088-S090 ◽  
Author(s):  
Guru Dutta Satyarthee ◽  
M. D. Sudhan ◽  
V. S. Mehta
Keyword(s):  
Gamma Knife ◽  
Pilocytic Astrocytoma ◽  
Brainstem Glioma ◽  
Prolonged Survival ◽  
Histopathological Diagnosis ◽  
Large Tumor ◽  
Who Grade ◽  
Mri Brain ◽  
First Case ◽  
Gamma Knife Therapy

ABSTRACTBrainstem glioma usually carries a poor prognosis and prolonged survival is very infrequent. In a detailed Pubmed, Medline search for prolonged survival, authors could got a longest survival only up to seventeen years, reported by Umehara et al, who was subjected to gamma knife therapy and got symptomatic, MRI brain reveled large tumor growth during pregnancy necessitating emergency surgery and histopathological diagnosis was pilocytic astrocytoma. Authors report an interesting case of midbrain glioma diagnosed 21 years back, who underwent gross resection in the year 1993, histopathology was pilocytic astrocytoma, WHO grade I, and received gamma knife surgery for residual subsequently and he presented with sudden onset left sided hemiplegia on the current admission. The cranial MRI imaging revealed an infarct involving right hemi midbrain, contrast MRI brain revealed no residual glioma. To the best knowledge of authors such prolonged survival is not reported with a case of brainstem glioma survived twenty- one years with non residual tumor on the last imaging study represents first case of its kind in the western literature and probably developed hemiplegia due to bleed, highlighting bleed as delayed complication following gamma knife therapy for cranial tumors

A radiopathological classification of dural tail sign of meningiomas

2012 ◽  
Vol 117 (4) ◽  
pp. 645-653 ◽  
Author(s):  
Song-tao Qi ◽  
Yi Liu ◽  
Jun Pan ◽  
Silky Chotai ◽  
Lu-xiong Fang
Keyword(s):  
Tumor Cells ◽  
Histopathological Examination ◽  
Who Grade ◽  
Simpson Grade ◽  
Mri Studies ◽  
Dural Tail Sign ◽  
Significant Difference ◽  
Different Types ◽  
Dural Tail

Object The completeness of meningioma resection depends on the resection of dura mater invaded by the tumor. The pathological changes of the dura around the tumor can be interpreted by evaluating the dural tail sign (DTS) on MRI studies. The goal of this study was to clarify the pathological characteristics of the DTSs, propose a classification based on the histopathological and radiological correlation, and identify the invasive range of tumor cells in different types of DTS. Methods The authors retrospectively reviewed 179 patients with convexity meningiomas who underwent Simpson Grade I resection. All patients underwent an enhanced MRI examination preoperatively. The convexity meningiomas were dichotomized into various subtypes in accordance with the 2007 WHO classification of tumors of the CNS, and the DTS was identified based on the Goldsher criteria. The range of resection of the involved dura was 3 cm from the base of the tumor, which corresponded with the length of DTS on MRI studies. Histopathological examination of dura at 0.5, 1.0, 1.5, 2.0, 2.5, and 3.0 cm from the base of the tumor was conducted, and the findings were correlated with the preoperative MRI appearance of the DTS. Results A total of 154 (86%) of 179 convexity meningiomas were classified into WHO Grade I subtype, including transitional (44 [28.6%] of 154), meningothelial (36 [23.4%] of 154), fibrous (23 [14.9%] of 154), psammomatous (22 [14.3%] of 154), secretory (10 [6.5%] of 154), and angiomatous (19 [12.3%] of 154). The other 25 (14%) were non–Grade I (WHO) tumors, including atypical (12 [48%] of 25), anaplastic (5 [20%] of 25), and papillary (8 [32%] of 25). The DTS was classified into 5 types: smooth (16 [8.9%] of 179), nodular (36 [20.1%] of 179), mixed (57 [31.8%] of 179), symmetrical multipolar (15 [8.4%] of 179), and asymmetrical multipolar (55 [30.7%] of 179). There was a significant difference in distribution of DTS type between Grade I and non–Grade I tumors (p = 0.004), whereas the difference was not significant among Grade I tumors (0.841) or among non–Grade I tumors (p = 0.818). All smooth-type DTSs were encountered in Grade I tumors, and the mixed DTS (52 [33.8%] of 154) was the most common type in these tumors. Nodular-type DTS was more commonly seen in non–Grade I tumors (12 [48%] of 25). Tumor invasion was found in 88.3% (158 of 179) of convexity meningiomas, of which the range of invasion in 82.3% (130 of 158) was within 2 cm and that in 94.9% (150 of 158) was within 2.5 cm. The incidence of invasion and the range invaded by tumor cells varied in different types of DTS, and differences were statistically significant (p < 0.001). Conclusions Nodular-type DTS on MRI studies might be associated with non–Grade I tumors. The range of dural resection for convexity meningiomas should be 2.5 cm from the tumor base, and if this extent of resection is not feasible, the type of DTS should be considered. However, for skull base meningiomas, in which mostly Simpson Grade II resection is achieved, the use of this classification should be further validated. The classification of DTS enables the surgeon to predict preoperatively and then to achieve the optimal range of dural resection that might significantly reduce the recurrence rate of meningiomas.

scholarly journals Laporan Kasus Butterfly gliobasltoma pada Laki-Laki Usia 24 Tahun

2021 ◽  
Vol 8 (3) ◽  
pp. 395-411
Author(s):  
Dewa Kartika ◽  
Baskoro Nurdopo
Keyword(s):  
White Matter ◽  
Corpus Callosum ◽  
Ct Scan ◽  
Pilocytic Astrocytoma ◽  
High Grade ◽  
Who Grade ◽  
High Grade Astrocytoma ◽  
Perifocal Edema

Pendahuluan Butterfly Glioma adalah high grade astrocytoma, biasanya glioblastoma (WHO grade IV), yang melintasi garis tengah melalui corpus callosum. Komissura white matter lainnya kadang juga terlibat. Istilah kupu-kupu mengacu pada ekstensi yang melewati garis tengah seperti sayap. Butterfly Glioma paling sering terjadi di lobus frontal, melintasi garis tengah melalui genu corpus callosum, namun butterfly glioma posterior kadang juga ditemui. Laporan kasus Seorang pasien laki-laki usia 24 tahun dengan keluhan utama 9 bulan, yang lalu. Penglihatan kabur, konsentrasi menurun. Kejang(-). Kemudian 3 bulan yang lalu mata tidak bisa melihat. Dan 1 bulan yang lalu tubuh lemas susah digerakkan Pemeriksaan patologi anatomi menunjukkan Pylocytic Astrocytoma. Pemeriksaan CT scan kepala menunjukkan  Massa solid inhomogen intraxial ( ukuran ± AP 7,6 x 8,9 x CC 6,2 cm ) disertai kalsifikasi di dalamnya pada corpus callosum yang tampak cross mid line ( sisi kiri lebih dominan ) membentuk gambaran butterfly sign dengan perifocal edema à curiga gambaran glioblastoma multiformis.   Pembahasan Hasil pemeriksaan anamnesis dan pemeriksaan fisik pasien ini menunjukkan kecurigaan adanya SOL. Pemeriksaan CT scan kepala menunjukkan  Massa solid inhomogen intraxial disertai kalsifikasi di dalamnya pada corpus callosum yang tampak cross mid line ( sisi kiri lebih dominan ) membentuk gambaran butterfly sign dengan perifocal edema à curiga gambaran glioblastoma multiformis. Dari PA didapatkan hasil Pilocytic astrocytoma. Sedangkan gambaran radiologi Pilocytic astrocytoma berupa lesi kistik dengan nodul mural yang enhanced. Kasus ini secara radiologis lebih mengarah ke Butterfly Glioblastoma dengan adanya lesi yang melewati garis tengah, serta ada komponen nekrotik dan perdarahan.. Modalitas imejing pilihan yang dapat dilakukan pada kasus Butterfly Glioblastoma adalah CT scan dan MRI. Kesimpulan Kasus ini secara radiologis lebih mengarah ke Butterfly Glioblastoma dengan adanya lesi yang melewati garis tengah, serta ada komponen nekrotik dan perdarahan. Dan pemeriksaan radiologis yang dapat digunakan pada Butterfly Glioblastoma adalah CT scan dan MRI.

scholarly journals Establishment of age group classification for risk stratification in glioma patients

2020 ◽  
Author(s):  
Zhiying Lin ◽  
Runwei Yang ◽  
Yawei Liu ◽  
Kaishu Li ◽  
Guozhong Yi ◽  
...  
Keyword(s):  
Molecular Markers ◽  
Risk Stratification ◽  
Tumor Size ◽  
Age Groups ◽  
Tumor Location ◽  
Group Classification ◽  
Age Group ◽  
Ki 67 ◽  
Who Grade ◽  
Age Group Classification

Abstract Objective: Age is associated with the prognosis of glioma patients, but there is no uniform standard of age-group classification to evaluate the prognosis of glioma patients. In this study, we aimed to establish an age group classification for risk stratification in glioma patients. Methods: A total of 1502 patients diagnosed with gliomas at Nanfang Hospital between 2000 and 2018 were enrolled. The WHO grade of glioma was used as a dependent variable to evaluate the effect of age on risk stratification. The evaluation model was established by logistic regression, and the Akaike information criterion (AIC) value of the model was used to determine the optimal cutoff points for age-classification. The differences in gender, WHO grade, pathological subtype, tumor cell differentiation direction, tumor size, tumor location, and molecular markers between different age groups were analyzed. The molecular markers included GFAP, EMA, MGMT, p53, NeuN, Oligo2, EGFR, VEGF, IDH1, Ki-67, 1p/19q, PR, CD3, H3K27M, and TS. Results: The proportion of men with glioma was higher than that of women with glioma (58.3% vs 41.7%). Analysis of age showed that appropriate classifications of age group were 0-14 years old (pediatric group), 15-47 years old (youth group), 48-63 years old (middle-aged group), and ≥64 years old (elderly group).The proportions of glioblastoma and large tumor size (4-6 cm) increased with age (p = 0.000, p = 0.018, respectively ). Analysis of the pathological molecular markers across the four age groups showed that the proportion of patients with larger than 10% area of Ki-67 expression or positive PR expression increased with age (p = 0.000, p = 0.017, respectively). Conclusion: Age was effective evaluating the risk of glioblastoma in glioma patients. Appropriate classifications of age group for risk stratification were 0-14 years old (pediatric group), 15-47 years old (young group), 48-63 years old (middle age group) and ≥ 64 years old (elderly group). There was significant heterogeneity in WHO grade, tumor size, tumor location and some molecular markers among the four age groups.

scholarly journals Huge heterogeneity in survival in a subset of adult patients with resected, wild-type isocitrate dehydrogenase status, WHO grade II astrocytomas

2019 ◽  
Vol 130 (4) ◽  
pp. 1289-1298 ◽  
Author(s):  
Gaëtan Poulen ◽  
Catherine Gozé ◽  
Valérie Rigau ◽  
Hugues Duffau
Keyword(s):  
Radiation Therapy ◽  
Malignant Transformation ◽  
Isocitrate Dehydrogenase ◽  
Adult Patients ◽  
World Health ◽  
Wild Type ◽  
Who Grade ◽  
Grade Ii ◽  
The Individual

OBJECTIVEWorld Health Organization grade II gliomas are infiltrating tumors that inexorably progress to a higher grade of malignancy. However, the time to malignant transformation is quite unpredictable at the individual patient level. A wild-type isocitrate dehydrogenase (IDH-wt) molecular profile has been reported as a poor prognostic factor, with more rapid progression and a shorter survival compared with IDH-mutant tumors. Here, the oncological outcomes of a series of adult patients with IDH-wt, diffuse, WHO grade II astrocytomas (AII) who underwent resection without early adjuvant therapy were investigated.METHODSA retrospective review of patients extracted from a prospective database who underwent resection between 2007 and 2013 for histopathologically confirmed, IDH-wt, non–1p19q codeleted AII was performed. All patients had a minimum follow-up period of 2 years. Information regarding clinical, radiographic, and surgical results and survival were collected and analyzed.RESULTSThirty-one consecutive patients (18 men and 13 women, median age 39.6 years) were included in this study. The preoperative median tumor volume was 54 cm3 (range 3.5–180 cm3). The median growth rate, measured as the velocity of diametric expansion, was 2.45 mm/year. The median residual volume after surgery was 4.2 cm3 (range 0–30 cm3) with a median volumetric extent of resection of 93.97% (8 patients had a total or supratotal resection). No patient experienced permanent neurological deficits after surgery, and all patients resumed a normal life. No immediate postoperative chemotherapy or radiation therapy was given. The median clinical follow-up duration from diagnosis was 74 months (range 27–157 months). In this follow-up period, 18 patients received delayed chemotherapy and/or radiotherapy for tumor progression. Five patients (16%) died at a median time from radiological diagnosis of 3.5 years (range 2.6–4.5 years). Survival from diagnosis was 77.27% at 5 years. None of the 21 patients with a long-term follow-up greater than 5 years have died. There were no significant differences between the clinical, radiological, or molecular characteristics of the survivors relative to the patients who died.CONCLUSIONSHuge heterogeneity in the survival data for a subset of 31 patients with resected IDH-wt AII tumors was observed. These findings suggest that IDH mutation status alone is not sufficient to predict risk of malignant transformation and survival at the individual level. Therefore, the therapeutic management of AII tumors, in particular the decision to administer early adjuvant chemotherapy and/or radiation therapy following surgery, should not solely rely on routine molecular markers.

P14.57 WHO grade II and III meningioma: a large mono-institutional retrospective series

2021 ◽  
Vol 23 (Supplement_2) ◽  
pp. ii48-ii49
Author(s):  
G Simonetti ◽  
P Gaviani ◽  
M Farinotti ◽  
F Legnani ◽  
B Pollo ◽  
...  
Keyword(s):  
Disease Control ◽  
Aggressive Behavior ◽  
Survival Data ◽  
Large Population ◽  
Future Research ◽  
Ki 67 ◽  
Who Grade ◽  
Risk Of Death ◽  
Molecular Features ◽  
Grade Ii

Abstract BACKGROUND Meningiomas are usually considered benign lesions, however a part of them can show very aggressive behavior with tendency to metastasize. This subgroup is known as high-grade meningiomas (HGM). Due to the rarity of the disease, effective medical treatments are lacking, especially at the time of recurrence. We aim to describe the clinical, radiological and molecular features of a large population of HGM diagnosed between 2010 and 2018. The secondary aim was to evaluate survival (PFS) and overall survival (OS). MATERIAL AND METHODS We collected clinical and survival data from primitive WHO grade II and III meningioma patients treated at Fondazione IRCCS Istituto Neurologico Carlo Besta from January 2010 to December 2018. Records were collected on a web-based platform (Microsoft Excel) that was customized for this study. The database, contained epidemiological, diagnostic (radiological and histological/molecular), surgical, therapeutic and recurrence information, as well as survival data. RESULTS 183 patients (105 females and 78 males), with median age of 58 years (25–88), were included; 168 were atypical, 12 anaplastic, 3 rhabdoid. Overall, m-PFS was 4.2 years, and m-OS was 10.3 years. Gross-total resection had a 5-year survival rate of 95% compared with subtotal/partial resection (86% and 67%) (p=0.002). Higher expression of Ki-67/MIB-1 seems associated with higher risk of death (HR:1.06 with 95% CI, 1.00–1.12, p=0.03). No statistically significant differences were seen in survival between the group managed with a wait-and-see strategy vs the group treated with RT/SRS while a difference on PFS was seen (4.1 years vs 5.2 years p=0.03). After second recurrence, the most employed treatments were systemic therapies with a very limited effect on disease control. CONCLUSION Data confirmed the aggressive behavior of HGM. The extent of resection seems to correlate with a favorable outcome regardless histological subtypes. The role of RT/SRS remains controversial, with no statistically significant impact on OS but a possible role on PFS. At relapse, no chemotherapies are able to achieve disease control and recurrent HGM remains the real challenge for future research, focusing on biological/molecular predictors in order to achieve a patient-tailored treatment.This retrospective study allowed to analyzed the largest national recent series of aggressive meningiomas with the purpose of identify relevant outcome measures for future prospective studies. Our findings could be important to give a snapshot of the current attitude to treat aggressive meningiomas but also to inspire national and international collaboration in order to provide evidence-based management strategies trying to obtain a standard of care.

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