Rhabdoid meningiomas (RM) are a rare meningioma subtype with a heterogeneous clinical course which is more frequently associated with recurrence, even among tumors undergoing-complete surgical removal. Here, we retrospectively analyzed the clinical-histopathological and cytogenetic features of 29 tumors, from patients with recurrent (seven primary and 14 recurrent tumors) vs. non-recurrent RM (n = 8). Recurrent RM showed one (29%), two (29%) or three (42%) recurrences. BAP1 loss of expression was found in one third of all RM at diagnosis and increased to 100% in subsequent tumor recurrences. Despite both recurrent and non-recurrent RM shared chromosome 22 losses, non-recurrent tumors more frequently displayed extensive losses of chromosome 19p (62%) and/or 19q (50%), together with gains of chromosomes 20 and 21 (38%, respectively), whereas recurrent RM (at diagnosis) displayed more complex genotypic profiles with extensive losses of chromosomes 1p, 14q, 18p, 18q (67% each) and 21p (50%), together with focal gains at chromosome 17q22 (67%). Compared to paired primary tumors, recurrent RM samples revealed additional losses at chromosomes 16q and 19p (50% each), together with gains at chromosomes 1q and 17q in most recurrent tumors (67%, each). All deceased recurrent RM patients corresponded to women with chromosome 17q gains, although no statistical significant differences were found vs. the other RM patients.

UNASSIGNED: Rhabdoid meningiomas (RMs) are a rare type of malignant meningioma. Here, we report a case of intracranial RM presenting with visual disturbance and prominent hyperintensity in the optic nerve (ON).
UNASSIGNED: A 20-year-old female presented with a 1-year history of headache. At presentation, her visual acuity (VA) was 20/50 on the right side and 20/40 on the left, with an intraocular pressure of 17 mmHg on both sides. Cerebral magnetic resonance imaging revealed a broad-based tumor in the right frontal convexity. It measured 82 mm × 65 mm × 70 mm in diameter, accompanied by cystic components, and was inhomogeneously enhanced. The intraorbital ONs demonstrated prominent intramedullary hyperintensity on the constructive interference steady-state sequence. Gross total tumor resection was performed and the pathology was consistent with RM. Immediately after surgery, her VA and IOP were 20/17 and 10 mmHg, respectively, with a remarkable resolution of the intramedullary hyperintensity.
UNASSIGNED: Prominent hyperintensity in the ON identified in patients with chronic intracranial hypertension may be an indicator of visual disturbance. It can rapidly resolve after resolution of intracranial hypertension with functional recovery.

Rhabdoid meningiomas (RM) shows heterogeneous histological findings, and a wide variety of chromosomal copy number alterations (CNA) are associated with an unpredictable course of the disease. In this study, we analyzed a series of 305 RM samples from patients previously reported in the literature and 33 samples from 23 patients studied in our laboratory. Monosomy 22-involving the minimal but most common recurrent region loss of the 22q11.23 chromosomal region was the most observed chromosomal alteration, followed by losses of chromosomes 14, 1, 6, and 19, polysomies of chromosomes 17, 1q, and 20, and gains of 13q14.2, 10p13, and 21q21.2 chromosomal regions. Based on their CNA profile, RM could be classified into two genetic subgroups with distinct clinicopathologic features characterized by the presence of (1) chromosomal losses only and (2) combined losses and gains of several chromosomes. The latter displays a higher frequency of WHO grade 3 tumors and poorer clinical outcomes.

Meningioma is the most common primary brain tumor, and recurrence risk increases with increasing WHO Grade from I to III. Rhabdoid meningiomas are a subset of WHO Grade III tumors with rhabdoid cells, a high proliferation index, and other malignant features that follow an aggressive clinical course. Some meningiomas with rhabdoid features either only focally or without other malignant features are classified as lower grade yet still recur early. Recently, inactivating mutations in the tumor suppressor gene BAP1 have been associated with poorer prognosis in rhabdoid meningioma and meningioma with rhabdoid features, and germline mutations have been linked to a hereditary tumor predisposition syndrome (TPDS) predisposing patients primarily to melanoma and mesothelioma. We present the first report of a familial BAP1 inactivating mutation identified after multiple generations of a family presented with meningiomas with rhabdoid features instead of with previously described BAP1 loss-associated malignancies. A 24-year-old female presented with a Grade II meningioma with rhabdoid and papillary features treated with subtotal resection, adjuvant external beam radiation therapy, and salvage gamma knife radiosurgery six years later. Around that time, her mother presented with a meningioma with rhabdoid and papillary features managed with resection and adjuvant radiation therapy. Germline testing was positive for a pathogenic BAP1 mutation in both patients. Sequencing of both tumors demonstrated biallelic BAP1 inactivation via the combination of germline BAP1 mutation and either loss of heterozygosity or somatic mutation. No additional mutations implicated in oncogenesis were noted from either patient\'s germline or tumor sequencing, suggesting that the inactivation of BAP1 was responsible for pathogenesis. These cases demonstrate the importance of routine BAP1 tumor testing in meningioma with rhabdoid features regardless of grade, germline testing for patients with BAP1 inactivated tumors, and tailored cancer screening in this population.

Meningioma is the most common central nervous system tumor that usually behaves benignly and has a good prognosis after treatment with tumor gross resection and with or without adjuvant therapy. Malignancy in meningioma is very rare and extracranial metastasis to cervical lymph nodes is even rarer. We report a case of a 40-year-old woman diagnosed with metastatic rhabdoid meningioma. She had recurrent primary disease and metastasis to bilateral cervical lymph nodes. She previously had intracranial tumor twice resected. We also review relevant, previously published cases in the literature. I hope you find these suggestions helpful.

Rhabdoid Meningiomas (RM) are rare malignant type of meningiomas, classified as grade III in the WHO classification. Only a few case series have been reported, and factors affecting prognosis are still unclear.
We did a retrospective chart review of all the RMs diagnosed in our institute between 2007 and 2019. Demographic profile, clinical status, imaging, surgical procedures used, post-operative course, adjuvant therapy and follow-ups were reviewed. Histopathological slides were also reviewed.
There were 11 patients with RM who underwent 17 surgical procedures between them. Median age was 26 years. On imaging, four had lesions in skull base, three in convexity and four in parasagittal region. Five patients had lesions which had bled and two had leptomeningeal dissemination. Two patients underwent Simpson\'s grade 1 excision, seven underwent grade 2 and one patient each underwent grade 3 and 5 excisions. One patient presented with poor sensorium and underwent surgery but ultimately succumbed. All reported patients had Rhabdoid features (>50%). Features of anaplasia were seen in four cases and atypical meningioma in others. The median progression-free-survival and overall survival was 6 months and 9 months, respectively. Female gender (n = 5; p = 0.032) and patients who received radiotherapy (p = 0.030) had a survival advantage. Location of the tumor (p = 0.43), presence of hemorrhage in the lesion (p = 0.49), grade of excision (p = 0.40) and WHO pathological grade (p = 0.11) did not have a statistically significant survival benefit.
Female gender and adjuvant radiotherapy were associated with survival advantage in our sample. Large studies are required to establish the factors associated with survival.

Malignant intraventricular meningiomas (IVMs) are very rare with only a few reported cases. A midline search up to December 2020 selected 40 articles for a total of 65 patients. The inclusion criteria were series and case reports in English language, as well as papers written in other languages, but with abstracts written in English. Malignant IVMs at the first diagnosis (group A, 50 patients) and those with anaplastic transformation from previous WHO grades I and II tumors (group B, 15 patients) were separately analyzed. The unique personal case among 1285 meningiomas (0.078%) is also added. Malignant IVMs mainly occur in women (61%) with a median age of 45 years and are mainly located in the lateral ventricle (93%) and trigonal region (74%), with no cases in the fourth ventricle. Irregular borders (80%), heterogeneous enhancement (83%), and perilesional edema (76%) are the most frequent radiological findings. The histology was mainly pure anaplastic (85%), whereas papillary (7%), rhabdoid (5%), and mixed forms (3%) are very rare. The CSF spread was found in 60% of the cases. The prognosis is very dismal, with an overall median survival of 17.5 months after surgery for the anaplastic forms. Malignant IVMs at initial diagnosis (group A) show better overall survival (25 months) than those occurring from anaplastic transformation of lower grade tumors (group B) (10.1 months).

Meningiomas are the most common primary brain tumors and are generally considered benign. However, a rare subgroup of meningiomas, classified as World Health Organization (WHO) grade III meningiomas, can show extremely aggressive behavior and high rates of recurrence. Despite ongoing research, data on the clinical outcome of this subgroup of meningiomas are still limited.
Medical records of patients with WHO grade III meningiomas diagnosed between 2000 and 2018 at the Coimbra University Hospital Center were retrospectively reviewed and several variables of interest and their relation to patients\' survival were analyzed.
Of the 26 patients included in the final analysis, 23 had anaplastic meningiomas, 2 had papillary meningiomas, and 1 had a rhabdoid meningioma. Median overall survival and median progression-free survival were 2.45 and 1.22 years, respectively. Overall survival at 1, 2 and 5 years was 73%, 57%, and 35%, respectively. Adjuvant radiotherapy correlated with improved survival for subtotally resected meningiomas but not for gross totally resected meningiomas. There was a trend toward improved overall survival with gross total resection versus subtotal resection, but this difference failed to reach statistical significance.
This study provides insight into the clinical outcomes of WHO grade III meningiomas and suggests that adjuvant radiotherapy may not be beneficial for patients who underwent gross total resection. This rare subset of meningiomas still portends a devastating prognosis and the impact of extent of resection and adjuvant therapies in these patients needs further clarification.

UNASSIGNED: Spheno-orbital meningiomas (SOMs) are often benign. The association of meningioma and systemic lupus erythematosus (SLE) is rarely discussed in the literature. Here, we report two patients with high-grade, SOMs with a prolonged history of SLE and review the literature.
UNASSIGNED: The first case is a 52-year-old female patient with a 15-year history of SLE diagnosis who was referred to our center with a 1-year history of proptosis and excessive tearing of the left eye. This patient was operated for the left SOM with histopathological diagnosis of the World Health Organization (WHO) Grade III rhabdoid meningioma. The second case is a 36-year-old female patient with a 12-year history of SLE diagnosis who presented to our clinic with a 5-year-history of progressive right eye proptosis and occasional headaches. She was operated for the right SOM with histopathological diagnosis of the WHO Grade II chordoid meningioma.
UNASSIGNED: Rhabdoid and chordoid SOMs are uncommon and no previous report discussed their occurrence in patients with SLE. The association of high-grade meningiomas and SLE deserves further exploration.

An 8-year-old girl was admitted with four limb weakness for 2 months. Hyperactive reflexes were observed in all four limbs, and positive Hoffmann\'s signs were revealed. An MRI spine with the coronal slide detected two tumors, first in the intradural and intramedullary space at the C2-C4 level and the second in the intradural and extramedullary space at the C5-C7 level. Axial T1W contrast MRI brain showed a tumor in the left parietal region. The patient underwent spine surgery first and following by brain surgery. No invasive lesions were remarked during surgery. Histological findings showed meningothelial meningioma and ependymoma from the spine and rhabdoid meningioma from the brain. Two months after the second surgery, the patient recovered fully with no symptoms and was able to participate in all regular activities in life. This work is the first report of a mixed tumor with distinct spinal meningioma and ependymoma components occurring in the cervical spinal cord at the C2-C7 level and coexisting with cranial meningioma. The remarkable result was that the patient fully recovered without any symptoms after receiving two surgeries.