AB051. Multiple meningiomas developed outside the radiation field after cranial irradiation: a case report.

BACKGROUND: Cranial irradiation has well-known long-term side effects, including radiation-induced neoplasms and vasculopathy. This report describes a case of aggressive and rapid-growing multiple meningiomas developed outside the radiation field after the treatment of medulloblastoma. CASE DESCRIPTION: A 6-year-old boy underwent surgery (gross total resection) and radiotherapy (19.8 Gy for posterior fossa only) against medulloblastoma in the 4th ventricle. The patient could not receive further craniospinal irradiation because of ventriculoperitoneal shunt-related complications. Eighteen years after the radiotherapy, the first meningioma developed in the right temporal convexity, without recurrence of medulloblastoma. It was left untreated because it was asymptomatic. Three years later, the meningioma grew from 0.6 to 6.3 cm3 in volume and another large meningioma (22.1 cm3) developed in the left temporal convexity with additional small meningioma in the right frontal convexity. The left large temporal meningioma showed aggressive nature invading the adjacent temporal bone and temporalis muscle. It was completely resected and the histology revealed as transitional meningioma with 2% of Ki-67. Another new meningioma was identified on the right cerebellar convexity three years post-craniotomy. Subsequent follow-up indicated a progressive increase in the tumor size and gamma knife radiosurgery was performed with right frontal convexity small meningioma. The patient is currently under ongoing surveillance through follow-up assessments. CONCLUSIONS: For patients who received radiotherapy at a young age, clinicians should consider the possibility of secondary neoplasm development even outside the radiation field. Careful imaging follow-up and surgical management are warranted because of the aggressive nature of secondary tumors even though benign in histology.

Metachronous intracranial meningiomas without dural attachment in a child - Rare case report and review of literature.

INTRODUCTION: Meningiomas in children are rare, constituting less than 5% of all paediatric brain tumours and less than 2% of all meningiomas. Multiple meningiomas (synchronous or metachronous) are even more uncommon, typically occurring due to radiation exposure or in patients with phacomatoses like Neurofibromatosis II. This report presents the case of a child with metachronous meningiomas without dural attachment in unusual locations, along with their management. PURPOSE: This report aims to describe a rare paediatric case of metachronous meningiomas without dural attachment, detailing their presentation, treatment, and outcomes. CASE DETAILS: A 2-year-old female presented with headaches, irritability, and excessive crying for one year. A CT scan revealed a mass in the fourth ventricle, causing obstruction, which was surgically decompressed. The biopsy confirmed a clear cell meningioma, WHO grade II. A follow-up MRI identified a new lesion in the suprasellar area six months later, for which she underwent right pterional craniotomy and gross total resection, which turned out to be a clear cell meningioma, WHO grade II. The patient recovered well and remained asymptomatic, with no recurrence on MRI at one-year follow-up. CONCLUSION: This case highlights the unusual presentation of metachronous clear cell meningiomas without dural attachment in a young child. Surgical excision resulted in a favourable outcome, though long-term follow-up is essential due to the high propensity for recurrence.

Comprehensive genomic analysis reveals clonal origin and subtype-specific evolution in a case of sporadic multiple meningiomas.

Meningioma is the most common primary intracranial tumor in adults, with up to 10% manifesting as multiple tumors. Data on the genomic and molecular changes in sporadic multiple meningiomas are scarce, leading to ongoing debates regarding their evolutionary processes. A comprehensive genetic analysis of a large number of lesions, including precursor lesions, is necessary to explore these two possible origins: clonal and independent. In the present study, we performed whole-exome sequencing and analyzed somatic single-nucleotide variants (SNVs), insertions/deletions (INDELs), and copy number alterations (CNAs) in a patient with sporadic multiple meningiomas. These meningiomas included two mass-forming lesions of different histological subtypes (transitional and chordoid) and two small meningothelial nests. Genetic analysis revealed CNAs on chromosomes 22q and Y as common abnormalities in the two largest tumors. Furthermore, we identified SNV/INDELs unique to each focus, with NF2 mutation prevalent in the transitional meningioma and CREBBP mutation in the chordoid meningioma. Loss of chromosome 22 was detected in two small meningothelial nests. Overall, we elucidated the clonal origin and subtype-specific evolution of multiple meningiomas in this case. CNAs may serve as the initial driving event in meningioma development.

Multiple meningiomas: Epidemiology, management, and outcomes.

Meningiomas are the most common nonmalignant brain tumor in adults, with an increasing incidence of asymptomatic meningiomas diagnosed on more ubiquitous neuroimaging. A subset of meningioma patients bear 2 or more spatially separated synchronous or metachronous tumors termed "multiple meningiomas" (MM), reported to occur in only 1%-10% of patients, though recent data indicate higher incidence. MM constitute a distinct clinical entity, with unique etiologies including sporadic, familial and radiation-induced, and pose special management challenges. While the pathophysiology of MM is not established, theories include independent origin in disparate locations through unique genetic events, and the "monoclonal hypothesis" of a transformed neoplastic clone with subarachnoid seeding precipitating numerous distinct meningiomas. Patients with solitary meningiomas carry the risk of long-term neurological morbidity and mortality, as well as impaired health-related quality of life, despite being a generally benign and surgically curable tumor. For patients with MM, the situation is even less favorable. MM should be regarded as a chronic disease, and in many cases, the management goal is disease control, as cure is seldom possible. Multiple interventions and lifelong surveillance are sometimes necessary. We aim to review the MM literature and create a comprehensive overview, including an evidence-based management paradigm.

Intracranial Rosai Dorfman disease - A rare differential diagnosis of multiple meningiomas: a case report.

Rosai Dorfman Destombes (RDD) disease is a non-Langerhans histiocytosis. The central nervous system is affected in < 5% of cases. We report the case of a 59-year-old man, who began 8 months before admission with headache, diminished visual acuity in the temporal hemifields, hyposmia, and seizures. Magnetic resonance imaging showed three midline skull-base lesions in anterior, media, and posterior fossae. We performed a complete resection of symptomatic lesions using a bifrontal craniotomy. The histopathological analysis determined RDD, therefore, we started steroid treatment. Our case description is due to the diagnosis and location, one of the rarest reported to date in the literature.

La enfermedad de Rosai-Dorfman-Destombes (RDD) es una histiocitosis no Langerhans. El SNC se ve afectado en menos del 5% de los casos. Presentamos el caso de un hombre de 59 años quien inició ocho meses previos al ingreso con cefalea, hemianopsia bitemporal, hiposmia y convulsiones. La resonancia magnética mostró tres lesiones de la base del cráneo en las fosas anterior, media y posterior. Realizamos una resección completa de las lesiones sintomáticas mediante una craneotomía bifrontal. El análisis histopatológico determinó RDD. Nuestro caso es debido al diagnóstico y localización, uno de los más raros reportados hasta la fecha en la literatura.

Neurofibromatosis Type-2 presenting with vision impairment.

Neurofibromatosis Type-2 (NF2) is an autosomal dominant genetic tumour-predisposing condition caused by mutations in the NF2 gene located on chromosome 22q12. It is characterized by multiple benign tumours of the central and peripheral nervous systems and meninges, causing high morbidity. Herein, presentation of a rare case of NF2 in a 36-year-old female who presented with right eye visual disturbances, followed by tinnitus with hearing impairment. The visual disturbance developed into blindness. Magnetic resonance imaging (MRI) was performed, which showed a right-side cerebellopontine angle vestibular schwannoma and multiple meningiomas around the brain. According to the MRI findings, the patient was diagnosed with NF2. This case report aims to elucidate the importance of early brain imaging in any visual disturbances in young adults and to highlight the key role of medical imaging in the diagnosis of rare cases. Moreover, this describe the MRI features and the diagnostic accuracy for the tumours occurring in NF2 in detail.

Multiple Meningioma Resection by Bilateral Extended Rostrotentorial Craniotomy with a 3D-Print Guide in a Cat.

A 13-year-old castrated male domestic shorthair cat was referred for the surgical removal of multiple meningiomas. The cat experienced generalized tonic-clonic seizures, altered mentation, mild proprioceptive ataxia, and circling. Magnetic resonance imaging (MRI) revealed two round, solitary, well-delineated, space-occupying lesions suggestive of multiple meningiomas in the right frontal and occipital lobes. Before surgery, patient-specific three-dimensional (3D) printed models and guides were produced using a 3D program based on MRI and computed tomography (CT), and a rehearsal surgery was performed. With a 3D guide to find the location of the craniotomy lines, bilateral extended rostrotentorial craniotomy allowed en bloc resection of multiple meningiomas. The bone fragment was replaced and secured to the skull with a craniofacial plate and screws with an artificial dura. All of the surgical steps were performed without complications. The preoperative presenting signs were resolved by the time of follow-up examinations 2 weeks after surgery. Twelve months after the removal of the multiple meningiomas, the cat survived without further neurological progression. For the resection of multiple meningiomas, surgery can result in large bone defects and risk of massive hemorrhage. For this challenging surgery, patient-specific 3D models and guides can be effective for accurate and safe craniotomies.

Multiple ossified spinal meningiomas in the thoracic spine: A case report and literature review.

Background: Ossified spinal meningioma (OSM) is a rare form of a spinal tumor. The surgical strategies and pathologic findings related to OSM have been investigated in recent years. However, multiple OSMs are rarely reported. Here, we intend to present a rare case of multiple OSMs and review the relevant published literature. Case Presentation: A 76-year-old woman experienced a progressive sensorimotor disturbance in her bilateral lower limbs for the past 2 years. She complained of inability to walk, urinary incontinence, and chronic constipation when referred to our hospital. A neurological examination revealed a diminished sensation below the bilateral T7, and her neurological status was Nurick Grade 6. Magnetic resonance imaging (MRI) revealed multiple intradural-extramedullary neoplasms at the T7-T11 level. Computed tomography (CT) scans showed five high-density masses of varying sizes in the spinal canal at the T7-T12 level. The patient underwent tumor resection through T7-T11 laminectomy. A histopathological examination revealed multiple OSMs. Conclusion: We reported a rare case of multiple OSMs in an elderly patient. After one-stage complete resection, the patient recovered with satisfactory curative effect. Although elderly patients will face various postoperative complications due to their poor physical condition, we still recommend one-stage complete resection of multiple OSMs to reduce recurrence.

Case report and literature review: Treatment of multiple meningiomas combined with multiple unruptured aneurysms in a single operation.

Background: Although the incidence of a single meningioma or a single aneurysm is common, cases of multiple meningiomas combined with multiple aneurysms are rarely reported, and surgical treatment of the coexisting situation is also relatively uncommon. Case presentation: A 38-year-old male patient presented to the neurosurgery department of our center with a headache. Examination revealed only symptoms of headache. Laboratory tests showed only decreased total protein and albumin. Magnetic resonance imaging showed preoccupation with the frontal lobe and the right temple bone. Magnetic resonance angiography and digital subtraction angiography showed two aneurysms in the anterior communicating artery and right anterior cerebral artery. Based on a combination of the patient's history and imaging, we hypothesized that the patient was simultaneously suffering from meningioma and an aneurysm, and both of them are multiple. The patient underwent tumor resection and clipping procedure based on this hypothesis in one surgery. Intraoperative biopsy proved to be a meningioma. The patient was discharged on the 10th postoperative day, and a postoperative follow-up suggested no complications. Conclusion: Multiple meningiomas combined with multiple aneurysms are rare to be reported in the same patient. For those unruptured intracranial aneurysms (UIAs) located in the visual field of craniotomy prepared for brain tumorlike meningioma, it is possible to do the clipping as well. When the meningiomas are multiple, fitted with the surgical indication, and located in a position that cannot be treated in one surgery, this may lead to a two-stage operation, no matter where the UIAs are located.

Genomic profiling of sporadic multiple meningiomas.

BACKGROUND: Multiple meningiomas (MMs) rarely occur sporadically. It is unclear whether each individual tumor in a single patient behaves similarly. Moreover, the molecular mechanisms underlying the formation of sporadic MMs and clonal formation etiology of these tumors are poorly understood. METHODS: Patients with spatially separated MMs without prior radiation exposure or a family history who underwent surgical resection of at least two meningiomas were included. Unbiased, comprehensive next generation sequencing was performed, and relevant clinical data was analyzed. RESULTS: Fifteen meningiomas and one dural specimen from six patients were included. The majority of tumors (12/15) were WHO Grade I; one patient had bilateral MMs, one of which was Grade II, while the other was Grade I. We found 11/15 of our cohort specimens were of NF2-loss subtype. Meningiomas from 5/6 patients had a monoclonal origin, with the tumor from the remaining patient showing evidence for independent clonal formation. We identified a novel case of non-NF2 mutant MM with monoclonal etiology. MMs due to a monoclonal origin did not always display a homogenous genomic profile, but rather exhibited heterogeneity due to branching evolution. CONCLUSIONS: Both NF2-loss and non-NF2 driven MMs can form due to monoclonal expansion and those tumors can acquire inter-tumoral heterogeneity through branched evolution. Grade I and II meningiomas can occur in the same patient. Thus, the molecular make-up and clinical behavior of one tumor in MMs, cannot reliably lend insight into that of the others and suggests the clinical management strategy for MMs should be tailored individually.

Multiple Intracranial Meningiomas in Absence of Neurofibromatosis Type 2: A Case Report and Literature Review.

Meningiomas are one of the most common primary intracranial tumors known to exist since pre-historic times. Most of these tumors are benign, sporadic, and solitary. Multiple meningiomas are rare and have mostly been described in patients with neurofibromatosis type 2 (NF2). The presence of multiple lesions poses a unique challenge in strategizing the treatment. We present a rare case of multiple intracranial meningiomas in the absence of NF2, which we treated at Tata Main Hospital, Jamshedpur. The relevant literature has also been discussed.

Neurofibromatosis type 2-associated meningiomas: an international multicenter study of outcomes after Gamma Knife stereotactic radiosurgery.

OBJECTIVE: The management of neurofibromatosis type 2 (NF2)-associated meningiomas is challenging. The role of Gamma Knife radiosurgery (GKRS) in the treatment of these tumors remains to be fully defined. In this study, the authors aimed to examine the role of GKRS in the treatment of NF2-associated meningiomas and to evaluate the outcomes and complications after treatment. METHODS: Seven international medical centers contributed data for this retrospective cohort. Tumor progression was defined as a ≥ 20% increase from the baseline value. The clinical features, treatment details, outcomes, and complications were studied. The median follow-up was 8.5 years (range 0.6-25.5 years) from the time of initial GKRS. Shared frailty Cox regression was used for analysis. RESULTS: A total of 204 meningiomas in 39 patients treated with GKRS were analyzed. Cox regression analysis showed that increasing the maximum dose (p = 0.02; HR 12.2, 95% CI 1.287-116.7) and a lower number of meningiomas at presentation (p = 0.03; HR 0.9, 95% CI 0.821-0.990) were predictive of better tumor control in both univariable and multivariable settings. Age at onset, sex, margin dose, location, and presence of neurological deficit were not predictive of tumor progression. The cumulative 10-year progression-free survival was 94.8%. Radiation-induced adverse effects were noted in 4 patients (10%); these were transient and managed medically. No post-GKRS malignant transformation was noted in 287 person-years of follow-up. CONCLUSIONS: GKRS achieved effective tumor control with a low and generally acceptable rate of complications in NF2-associated meningiomas. There did not appear to be an appreciable risk of post-GKRS-induced malignancy in patients with NF2-treated meningiomas.

The impact of multiple lesions on progression-free survival of meningiomas: a 10-year multicenter experience.

OBJECTIVE: Multiple meningiomas (MMs) occur in as many as 18% of patients with meningioma, and data on progression-free survival (PFS) are scarce. The objective of this study was to explore the influence of the number of lesions and clinical characteristics on PFS in patients with WHO grade I meningiomas. METHODS: The authors retrospectively reviewed the records of all adults diagnosed with a meningioma at their three main sites from January 2009 to May 2020. Progression was considered the time from diagnosis until radiographic growth of the originally resected meningioma. A secondary analysis was performed to evaluate the time of diagnosis until the time to second intervention (TTSI). Univariable and multivariable analyses were conducted to assess whether the number of lesions or any associated variables (age, sex, race, radiation treatment, tumor location, and extent of resection) had a significant impact on PFS and TTSI. RESULTS: Eight hundred thirty-eight patients were included. Use of a log-rank test to evaluate PFS and TTSI between a single and multiple lesions showed a significantly shorter progression for MM (p < 0.001 and p < 0.001, respectively). Multivariable Cox regression analysis showed significantly inferior PFS on MM compared to a single lesion (hazard ratio [HR] 2.262, 95% confidence interval [CI] 1.392-3.677, p = 0.001) and a significantly inferior TTSI for patients with MM when compared to patients with a single meningioma (HR 2.377, 95% CI 1.617-3.494, p = 0.001). By testing the number of meningiomas as a continuous variable, PFS was significantly inferior for each additional meningioma (HR 1.350, 95% CI 1.074-1.698, p = 0.010) and TTSI was significantly inferior as well (HR 1.428, 95% CI 1.189-1.716, p < 0.001). African American patients had an inferior PFS when compared to non-Hispanic White patients (HR 3.472, 95% CI 1.083-11.129, p = 0.036). CONCLUSIONS: The PFS of meningiomas appears to be influenced by the number of lesions present. Patients with MM also appear to be more prone to undergoing a second intervention for progressive disease. Hence, a closer follow-up may be warranted in patients who present with multiple lesions. These results show a decreased PFS for each additional lesion present, as well as a shorter PFS for MM compared to a single lesion. When assessing associated risk factors, African American patients showed an inferior PFS, whereas older age and adjuvant therapy with radiation showed an improved PFS.

High Copy-Number Variation Burdens in Cranial Meningiomas From Patients With Diverse Clinical Phenotypes Characterized by Hot Genomic Structure Changes.

Meningiomas, as the most common primary tumor of the central nervous system, are known to harbor genomic aberrations that associate with clinical phenotypes. Here we performed genome-wide genotyping for cranial meningiomas in 383 Chinese patients and identified 9,821 copy-number variations (CNVs). Particularly, patients with diverse clinical features had distinct tumor CNV profiles. CNV burdens were greater in high-grade (WHO grade II and III) samples, recurrent lesions, large tumors (diameter >4.3 cm), and those collected from male patients. Nevertheless, the level of CNV burden did not relate to tumor locations, peritumoral brain edema, bone invasion, or multiple lesions. Overall, the most common tumor CNVs were the copy-number gain (CNG) at 22q11.1 and the copy-number losses (CNLs) at 22q13.2, 14q11.2, 1p34.3, and 1p31.3. Recurrent lesions were featured by the CNLs at 1p31.3, 6q22.31, 9p21.3, and 11p12, and high-grade samples had more CNVs at 4q13.3 and 6q22.31. Meanwhile, large tumors were more likely to have the CNVs at 1p31.3 and 1p34.3. Additionally, recurrence prediction indicated the CNLs at 4p16.3 (p = 0.009, hazard ratio = 5.69) and 10p11.22 (p = 0.037, hazard ratio = 4.53) were candidate independent risk factors.

Multiple meningiomas: does quantity matter? a population-based survival analysis with underlined age and sex differences.

INTRODUCTION: Intracranial meningiomas rarely present with multiple lesions. To the best of our knowledge, current literature regarding meningiomatosis (MM) is mostly comprised of small case series and individual reports. Hence, survival outcome data are limited. The Objective of this study is to explore the influence of sex, age, and number of lesions on overall survival (OS) in patients with MM. METHODS: We obtained demographic and clinical data from the surveillance, epidemiology, and end results program (SEER) on adult patients diagnosed with meningiomas from 1975 to 2017. Univariable and multivariable analyses were conducted to assess whether number of lesions, age, and sex had a significant influence on OS. RESULTS: 99,918 cases were included. Results showed that MM patients had a significantly decreased OS when compared to patients with a single lesion (median OS of 94 and 180 months, respectively; p < 0.001). Further analysis showed a progressive decrease on OS for every additional lesion; 2 (HR 1.659 [CI 95% 1.612-1.708], p < 0.001), 3 (HR 1.877 [CI 95% 1.773-1.988], p < 0.001), and ≥ 4 (HR 2.116 [CI 95% 1.886-2.373], p < 0.001). When assessing for sex differences, female patients had increased OS (HR 0.778 [CI 95% 0.743-0.815], p < 0.001) and decreased risk of developing MM (HR 0.809 [CI 95% 0.784-0.835], p < 0.001). CONCLUSION: Increasing number of meningiomas has a significant negative impact on OS, with a progressive decrease on survival for every additional lesion. Furthermore, female patients had increased OS and decreased risk to develop MM.

Tumor-to-Tumor Metastasis of Multiple Meningiomas and Clear Cell Renal Cell Carcinoma Metastasis as First Clinical Appearance of Kidney Cancer: A Case Report and Analysis.

Background Meningioma accounts for more than 35% of all diagnosed brain tumors of the central nervous system and, moreover, it is the most common benign recipient of tumor-to-tumor metastasis. Several cases with tumor-to-meningioma metastasis by breast, lung, and intestinal cancer have been described before. Case description The case of a patient with a longstanding history of multiple meningiomas ( n = 4) that suddenly became symptomatic and progressive in size is presented. Following extirpation of the two largest meningiomas, a histological examination revealed two separate tumor-to-meningioma metastases of clear cell renal cell carcinoma that was undiagnosed before. Post-surgical computed tomography scan then confirmed tumor-suspect lesions in both kidneys. After recovery and rehabilitation, adjuvant radio-chemo-therapy was applied according to protocols for kidney cancer. No other tumor-to-tumor-suspect event occurred since then for the remaining two meningiomas. Conclusion Review of literature and our case strengthens the idea of meningioma as a favorable premetastatic niche. Considering that the patient lived with a stable disease for many years, a sudden progress of tumor size in association with neurological deterioration was highly suspected for malign involvement, including the possibility of tumor-to-tumor metastasis. Physicians should be aware about this phenomenon and treat patients accordingly to the underlying disease.

Whole exome sequencing of multiple meningiomas with varying histopathological presentation in one patient revealed distinctive somatic mutation burden and independent clonal origins.

Background: Although meningiomas are common intracranial tumors, multiple meningiomas (MMs) are rare entities in patients without neurofibromatosis type 2. Previous studies suggest most sporadic MMs are of monoclone in origin. Objective: To elucidate the clonal relationship between two sporadic meningiomas from the same patient by using the next-generation sequencing (NGS) platform. Methods: Two MMs, located frontally and parietally on the right side, were surgically removed from a 52-year-old male. Pathological examinations and whole exome sequencing were performed on tumor samples, followed by Sanger sequencing validation. Results: MMs were diagnosed as secretory and fibrous subtypes, respectively, on histology (WHO grade I) and tumor DNA exhibited distinctive somatic mutation patterns. Specifically, the secretory subtype carried more single nucleotide variant while the fibrous subtype had much higher copy number variation. Besides, the two tumors demonstrated different mutation profiles in predisposing genes and known driver mutations. For example, the secretory subtype had missense mutations in TRAF7 and KLF4, while the fibrous subtype had frameshift deletion of NF2 gene in addition to copy number loss of NF2 and SMARCB1, genetic events that have already been associated with the development of meningiomas. Significantly mutated gene analysis revealed novel mutations of LOC729159 in the secretory subtype and RPGRIP1L and DPP6 in the fibrous subtype. Sanger sequencing validated important point mutations in TRAF7 (c.1678G>A, p.G560S), KLF4 (c.1225A>C, p.K409Q) and CDH11 (c.169T>G, p.W57G). Conclusion: Our data suggest the two meningiomas might develop independently in this patient and molecular subtyping by NGS is a valuable supplement to conventional pathology. Further study is needed to ascertain whether these novel genetic events are tumorigenic or simply passenger mutations, as well as their clinical implications.

Somatic SMARCB1 Mutation in Sporadic Multiple Meningiomas: Case Report.

Background: Multiple intracranial meningiomas account for <10% of all meningiomas. Familial multiple meningiomas have been linked to germline mutations in two genes: neurofibromatosis type 2 (NF2) and SWIch/Sucrose Non-Fermentable (SWI/SNF)-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1). Sporadic multiple meningiomas have been associated with somatic NF2 mutations and, to date, there has been no case related to somatic SMARCB1 mutations. Here, we describe the first case. Case Report: A 45-year-old female suffered a head trauma while snowboarding. Subsequent to her injury, she experienced persistent headache, nausea, vomiting, dizziness, and flashing lights in the right eye. Magnetic resonance imaging (MRI) of her brain revealed multiple intracranial meningiomas. She underwent a two-staged craniotomy to remove frontal/parietal/temporal and occipital extra-axial tumors. Pathology confirmed the masses as meningiomas, WHO Grade I. Tumor genetic testing was positive for SMARCB1 mutation but blood genetic testing was negative for SMARCB1 mutation. Conclusion: In sporadic multiple meningiomas, somatic NF2 mutations are usually the suspected genetic alternations. Our case illustrates that somatic SMARCB1 mutation is another genetic risk factor for sporadic multiple meningiomas, albeit rare.