Clinical, Histopathologic and Genetic Features of Rhabdoid Meningiomas.

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.

Malignant intraventricular meningioma: literature review and case report.

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).

Meningioma with rhabdoid features combined with meningioangiomatosis in infancy: a novel combination.

Meningioangiomatosis is a rare histologically distinct abnormality that is occasionally associated with intracranial meningioma. The rhabdoid variant of meningioma is also uncommon and is classified as a World Health Organization Grade III tumour. We report a case of meningioangiomatosis in conjunction with a meningioma with prominent rhabdoid features, in an infant male who underwent complete surgical resection of the lesion. The patient has been followed up for 6 years with no disease recurrence. To our knowledge, this is the first report in the literature describing meningioangiomatosis combined with a meningioma with rhabdoid features.

Paired Primary and Recurrent Rhabdoid Meningiomas: Cytogenetic Alterations, BAP1 Gene Expression Profile and Patient Outcome.

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.

Rhabdoid meningioma with a history of Budd-Chiari syndrome: a case report and review of the literature.

Background: Rhabdoid meningioma and Budd-Chiari syndrome are both extremely rare, and there is no report describing the two diseases occurring in the same patient thus far. Herein, we showed an unusual case of rhabdoid meningioma with a history of Budd-Chiari syndrome. Case presentation: The man was found to have abnormal liver function during physical examination in 2016 at 36 and was not paid attention to it. In 2019, he went to Beijing YouAn Hospital Affiliated to Capital Medical University for the decompensation of cirrhosis and was diagnosed with Budd-Chiari syndrome, subsequent angiography of the inferior vena cava combined with balloon dilatation were performed, the anticoagulation and hepatoprotective therapy were performed for a long time. When he turned 40 who had magnetic resonance imaging (MRI) that showed a left frontotemporal lobe space-occupying lesion, and postoperative pathological examination confirmed rhabdoid meningioma. He underwent surgery and postoperative adjuvant radiotherapy, but then he developed severe psychiatric symptoms and eventually succumbed to a lung infection two months after treatment. Conclusions: Budd-Chiari syndrome and Rhabdoid meningiomas are both extremely rare diseases. To the best of our knowledge, there is no report that the two rare diseases occurred in the same patient, and this is the first case. However, whether there is any link between the two diseases is unclear, more researches are needed to confirm it in the future.

Large rhabdoid meningioma presenting prominent hyperintensity in the optic nerve: An indicator of visual disturbance on constructive interference steady-state sequence?

Background: 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). Case Description: 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. Conclusion: 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 Meningioma Metastases Cervical Lymph Nodes: A Rare Clinical Case Report and Treatment Outcome.

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: Clinicopathological analysis of a rare variant of meningioma.

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: Female gender and adjuvant radiotherapy were associated with survival advantage in our sample. Large studies are required to establish the factors associated with survival.

World Health Organization Grade III Meningiomas: A Retrospective Study at an Academic Medical Center.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Germline BAP1 Mutation in a Family With Multi-Generational Meningioma With Rhabdoid Features: A Case Series and Literature Review.

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.

Triad of meningothelial meningioma, rhabdoid meningioma and ependymoma: successful management of an extremely rare case.

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.

Prognostic factors and long-term outcomes of primary intracranial rhabdoid meningioma: A systematic review.

Primary intracranial Rhabdoid meningioma (PIRM) is an uncommon subtype of WHO grade III meningioma. Given its rarity, its risk factors and management strategies are still unclear. Therefore, we aimed to assess the risk factors and outcomes for patients with PIRM and proposed an appropriate treatment. Ovid, Medline, Embase, Pubmed, Web of Science and Cochrane database were used to search for articles published between January 1998 and October 2019. Search terms combined "intracranial", "brain", and "cerebral" with "rhabdoid meningioma" or "WHO grade III meningioma". The entire cohort included 27 males (51.9 %) and 25 females (48.1 %) with an age ranging from 2 to 77 years (median 44 years). The size of tumor ranged from 1.3 to 7.4 cm (mean 4.3 cm). The Ki-67 proliferation index ranged from 1 % to 90 % (mean 15 %). In the whole cohort, gross total resection (GTR) and non-GTR were achieved in 63.5 % (33 cases) and 36.5 % (19 cases) patients, respectively. Twenty-five patients (48.1 %) had the postoperative radiotherapy, and 5 patients (9.6 %) had postoperative chemotherapy. Nineteen patients (39.6 %) developed recurrences, 4 patients (7.7 %) developed distant metastasizes, and 13 patients (25.0 %) died. GTR was associated with favorable overall survival (p = 0.008). The 1-, 3-, and 5-year progression-free survival rates were 84.6 %, 59.4 %, and 49.6 %, respectively; and the 1-, 3- and 5- year overall survival rates in the entire group were 91.4 %, 83.5 % and 68.9 %, respectively. GTR is recommend as the initial treatment option for PIRMs, contributing to acute histological diagnosis and prolonging long-term survival.

High-grade spheno-orbital meningioma in patients with systemic lupus erythematosus: Two case reports and literature review.

BACKGROUND: 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. CASE DESCRIPTION: 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. CONCLUSION: 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.

Dural-based atypical teratoid/rhabdoid tumor in an adult: DNA methylation profiling as a tool for the diagnosis.

Atypical teratoid/rhabdoid tumor (ATRT) is a malignant CNS embryonal tumor that mostly occurs in childhood, adult cases are rare. We report a case of a 23-year-old male with an extra-axial dura-based lesion in the left frontal area, previously diagnosed as gliosarcoma. After 6 years, the patient had a recurrence and the previous slides were reviewed. Tumor was positive for vimentin and negative for INI1. The differential diagnosis for this extra-axial tumor with long survival was rhabdoid meningioma with INI1 loss or ATRT. DNA methylation profiling was performed to reach the final and the most definitive diagnosis; the result was ATRT. Our case suggests the usefulness of DNA methylation profiling for diagnosing challenging CNS tumors.

Pathology of meningiomas.

Meningiomas are a diverse group of neoplasms that exhibit a wide range of morphologies and clinical behavior. They are generally accepted to originate from arachnoid cap cells within the leptomeninges. Classic histologic features include whorl formations, psammoma bodies, nuclear holes, and nuclear pseudoinclusions. Meningiomas are classified as benign, atypical, or anaplastic (grades I, II, or III) based on histologic features including mitotic activity, brain invasion, and presence of other minor criteria. There are numerous histologic variants of meningiomas, and some are associated with worse clinical outcomes and therefore are assigned a higher grade. The majority of meningiomas show diffuse positivity for vimentin and epithelial membrane antigen, supporting the dual mesenchymal and epithelial nature of meningothelial cells. The presence of an elevated proliferation index (as measured by Ki-67 immunohistochemical stain) and loss of progesterone receptor expression are associated with the higher grade. Pathologic features including histologic variants, grading criteria, and ancillary tests such as special and immunohistochemical stains are discussed.

Pediatric Rhabdoid Meningioma with Extension to the Heart: A First Case Report and Literature Review.

BACKGROUND: Meningiomas are the most common benign intracranial neoplasms in adults, but they have a lower incidence in children. Rhabdoid meningioma is a rare subtype of meningioma and is classified as World Health Organization grade III. CASE DESCRIPTION: We present a very rare case of a 9-year-old boy who presented to our institution with a history of headache, dizziness, and vomiting without neurologic deficit. The investigation showed a posterior fossa tumor with hemorrhage inside and hydrocephalus. He underwent tumor resection, and pathology showed rhabdoid meningioma. The patient had extensive recurrence after only 5 months, including extension to the neck, mediastinal veins, and heart. He was treated surgically and received adjuvant chemotherapy followed by radiation therapy. CONCLUSIONS: Rhabdoid meningioma is a malignant subtype of meningioma that occurs very rarely in pediatric patients. Additionally, rhabdoid meningioma, when it does occur in pediatric patients, has a high tendency to recur. Radical surgical resection with adjuvant radiotherapy is essential to prolonging survival. This is the first case with extracranial extension to the mediastinal veins and heart.

Primary Pulmonary Meningioma With Rhabdoid Features.

Only 1% to 2% of meningiomas have primary extrameningeal location, which is mostly head and neck region. Primary pulmonary meningiomas (PPMs) are even more uncommon with up to 50 cases reported in the literature. Only 5 cases of PPM with confirmed or possible malignancy have been previously described. Three-grade classification of meningiomas with the accordingly growing risk of aggressive behavior of the tumor has been proposed by the World Health Organization. As it is based on correlations between morphological and clinical features of intracranial meningiomas, the analogous prediction of ectopic tumors prognosis remains questionable due to scarce number of cases. In this article, we present a rare case of PPM with rhabdoid features (World Health Organization grade III), which lacked other signs of malignancy. The patient is doing well for 2 years after the thoracoscopic wedge resection without evidence of the disease recurrence.

World Health Organization Grade III (Nonanaplastic) Meningioma: Experience in a Series of 23 Cases.

OBJECTIVE: Rhabdoid meningioma (RM) and papillary meningioma (PM) are rare variants of World Health Organization grade III meningiomas. In this study, we presented a series of 23 cases from our institution to investigate adverse factors of and appropriate treatment for RM and PM. METHODS: Clinical data from 23 cases of PM and RM between January 2011 and December 2015 were retrospectively reviewed. RESULTS: The median follow-up was 38.0 months for World Health Organization grade III meningiomas. The mean progression-free survival (PFS) was 37.6 months, with 1-year, 3-year, and 5-year PFS of 78.3%, 50.8%, and 43.6%, respectively. The mean overall survival (OS) was 48.8 months, with 1-year, 3-year, and 5-year OS of 95.7%, 82.6%, and 44.0%, respectively. Univariate analysis showed that a ki-67 proliferation index >20% (hazard ratio [HR], 4.190; 95% confidence interval [CI], 1.033-17.001; P = 0.045) and PM (HR, 3.375; 95% CI, 0.998-11.408; P = 0.005) were related to worse PFS. Patients administered postoperative radiotherapy (PRT) after surgery had longer OS than did patients who did not receive PRT (median, 60.7 vs. 35.1 months; P = 0.029). Multivariate analysis showed that PRT was an independent factor for PFS (HR, 0.147; 95% CI, 0.033-0.657; P = 0.012) and OS (HR, 0.130; 95% CI, 0.025-0.691; P = 0.017) and that RM was an independent factor for PFS (HR, 7.312; 95% CI, 1.587-33.688; P = 0.011) and OS (HR, 6.447; 95% CI, 1.310-31.740; P = 0.022). CONCLUSIONS: We recommended adjuvant radiation regardless of the extent of resection. Individuals with PM or RM were at increased risk of recurrence and death; appropriate treatment for these patients should be further studied, and close follow-up is needed.

Ectopic rhabdoid meningioma of the orbit in a child: case report and review of the literature.

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

Rhabdoid Meningioma of Brain - A Rare Aggressive Tumor.

Rhabdoid meningioma is a rare aggressive variant of meningioma, regarded as WHO Grade III type. Histologically and cytologically, it is distinctive type having abundant eosinophilic cytoplasm, cytoplasmic inclusion with eccentrically placed vesicular nuclei and prominent nucleoli. High recurrence rate and poor outcome are important features. Here, we are presenting a rare case of rhabdoid meningioma found in a recurrent meningioma of the posterior fossa in a middle-aged female. We emphasized the squash cytology and histology finding of the rare neoplasm.