Neurofibromatosis meningioma. Medical search. Frequent questions

Meningiomas are the second most common tumor in the intradural extramedullary location, second only to tumors of the nerve sheath. (medscape.com)
Meningiomas account for approximately 25% of all spinal tumors. (medscape.com)
[ 7 ] In the United States, meningioma accounts for 37% of primary brain tumors, and cancerous meningiomas constitute approximately 1% of all primary brain tumors. (medscape.com)
There are many genetic factors associated with intradural tumors, most commonly neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2), and Von-Hippel Lindau (VHL) syndrome. (wikipedia.org)
The most common type of intradural-extramedullary tumors are meningiomas and nerve-sheath tumors. (wikipedia.org)
Intradural-extramedullary tumors are located within the dura but outside of the spinal cord parenchyma, with the most common being meningiomas and nerve sheath tumors (e.g. schwannomas, neurofibromas). (wikipedia.org)
Neurofibromatosis type 2 is a disorder characterized by the growth of noncancerous tumors in the nervous system. (medlineplus.gov)
The most common tumors associated with neurofibromatosis type 2 are called vestibular schwannomas. (medlineplus.gov)
Tumors that form on the membrane that covers the brain and spinal cord (meninges) are also common in neurofibromatosis type 2. (medlineplus.gov)
These tumors are called meningiomas. (medlineplus.gov)
Research suggests that the loss of merlin allows cells, especially Schwann cells, to multiply too frequently and form the tumors characteristic of neurofibromatosis type 2. (medlineplus.gov)
Almost everyone who is born with one NF2 variant acquires a second variant (known as a somatic variant) in these cells and develops the tumors characteristic of neurofibromatosis type 2. (medlineplus.gov)
Meningiomas are the most common benign tumors of the brain . (wikidoc.org)
Meningioma tumors in the brain of a neurofibromatosis type 2 (NF2) patient. (wustl.edu)
Schwannomas and meningiomas are nervous system tumors that can occur sporadically or in patients with neurofibromatosis type 2 (NF2). (medworm.com)
Mutations of the Neurofibromatosis 2 (NF2) gene are frequently observed in these tumors. (medworm.com)
Treatment with antiangiogenesis drugs may improve the effectiveness of radiation treatment of nervous system tumors that interfere with the hearing of patients with the genetic disorder neurofibromatosis 2, investigators report. (medworm.com)
Although the majority of meningiomas are benign , these tumors can grow slowly until they are very large, if left undiscovered, and, in some locations, can be severely disabling and life-threatening. (aans.org)
These tumors grow at a faster rate than benign meningiomas and are often characterized by brain invasion. (aans.org)
NEW YORK - New research from a University of California at San Francisco-led team has identified three DNA methylation-based meningioma groups with an epigenetic profiling study that took into account other molecular features including copy number variant (CNV) profiles of the primary intracranial tumors. (genomeweb.com)
On the other hand, the team classified 28 percent of the tumors into a hypermitotic meningioma group linked to cytotoxic therapy resistance and poor outcomes. (genomeweb.com)
The remaining tumors - almost 40 percent of the meningioma set - fell into an immune-enriched group marked by immune cell infiltration, lymphatic vessel features, and intermediate survival outcomes. (genomeweb.com)
Meningiomas represent approximately 20% of all primary brain tumors and occur most commonly in middle-aged women. (indiasurgerytour.com)
Some inherited conditions increase the risk of brain tumors, including neurofibromatosis, Von Hippel-Lindau syndrome, Li-Fraumeni syndrome, and Turcot's syndrome. (indiasurgerytour.com)
Neurofibromatosis 1 is associated with a higher incidence of primary central nervous system tumors. (medlink.com)
I've devoted a good part of my career to researching neurofibromatosis type 2, a genetic disease that causes complex tumors to grow in the brain and spinal cord. (nyulangone.org)
This review will cover the histo- and molecular pathology of meningiomas, including recent 2016 updates to the WHO classification of CNS tumors. (scienceopen.com)
We report genomic analysis of 300 meningiomas, the most common primary brain tumors, leading to the discovery of mutations in TRAF7, a proapoptotic E3 ubiquitin ligase, in nearly one-fourth of all meningiomas. (scienceopen.com)
Although most meningiomas are encapsulated and benign tumors with limited numbers of genetic aberrations, their intracranial location often leads to serious and potentially lethal consequences. (scienceopen.com)
Because women are twice as likely as men to develop meningiomas and these tumors harbor hormone receptors, an etiologic role for hormones (both endogenous and exogenous) has been hypothesized. (scienceopen.com)
Meningiomas account for approximately 13-19% of all brain tumors. (medscape.com)
Neurofibromatosis (NF) is a group of neurocutaneous syndromes characterized by generally non-cancerous tumors in the nervous system and skin. (asperbio.com)
3) Schwannomatosis (SWN) is characterized by multiple schwannomas (benign tumors of the nervous system) and, less frequently, meningiomas. (asperbio.com)

Main outcome measure Surgery (resection or decompression) or radiotherapy for one or more intracranial meningiomas. (bmj.com)
Conclusions A strong dose-effect relation was observed between use of cyproterone acetate and risk of intracranial meningiomas. (bmj.com)
T he neurosurgical frequency of multiple intracranial meningiomas is low, although it has risen since the introduction of computerized tomography (CT). (thejns.org)
H istorically , primary intracranial meningiomas rarely metastasize extracranially. (thejns.org)
Meningiomas are the most common benign intracranial tumor . (aans.org)
Neurofibromatosis 1 is an autosomally dominated inherited genetic condition that predisposes those involved to the development of intracranial neoplasms. (medlink.com)
Neurofibromatosis 1 has protean manifestations, of which intracranial gliomas are one of the most common. (medlink.com)
The recurrence of intracranial meningiomas after surgical treatment. (scienceopen.com)
Management of benign and aggressive intracranial meningiomas. (medscape.com)
Epidemiology and etiology of intracranial meningiomas: a review. (medscape.com)
Meningiomas of the anterior skull base constitute 40% of all intracranial meningiomas. (medscape.com)
NF2 also predisposes patients to intracranial and spinal meningiomas. (unboundmedicine.com)
Intracranial meningiomas (including optic nerve meningiomas) and intraspinal meningiomas. (unboundmedicine.com)

Unlike most other autosomal dominant conditions, in which one altered copy of a gene in each cell is sufficient to cause the disorder, two copies of the NF2 gene must be altered to trigger tumor formation in neurofibromatosis type 2. (medlineplus.gov)
The symptoms of meningioma can be flared by water retention, engorgement of blood vessels , and the presence of sex hormone receptors on tumor cells. (wikidoc.org)
The characteristics of a meningioma can be determined based on histopathological variables like tumor gradient, histological subtype, proliferative index, and invasiveness of a tumor to the brain . (wikidoc.org)
In some cases of vestibular schwannoma, a sometimes-lethal tumor often associated with neurofibromatosis 2 (NF2), secretions from the tumor contain toxic molecules that damage the inner ear. (medworm.com)
Tumor diagnosis included low-grade glioma (n = 4, two with neurofibromatosis type 1), meningioma (n = 1), medulloblastoma (n = 1) and secondary high-grade glioma (n = 1). (rchsd.org)
In contrast to meningiomas arising in neurofibromatosis type 2 patients missing an NF2 gene coding for the Merlin tumor suppressor protein, the researchers found that more than one-third of the meningiomas fell into a Merlin-intact group with the most favorable clinical outcomes and vulnerability to cytotoxic therapy. (genomeweb.com)
This tumor is called Meningioma. (cowurine.com)
Growing emphasis on brain tumor research coupled with the advent of new genetic and molecular epidemiologic tools in genetic and molecular epidemiology promise hope for advancing knowledge about the causes of intra-cranial meningioma. (scienceopen.com)
Suitable for certain types of meningiomas located at the skull base, this technique ensures optimal tumor removal while preserving critical structures. (apollohospitals.com)

Predisposes to multiple meningiomas preferentially in the falx cerebri . (wikidoc.org)
Even now, however, cases reported as multiple meningiomas include a variety of conditions from which these lesions must be differentiated. (thejns.org)

1 Bilateral vestibular schwannomas are pathognomonic for NF2, and people with NF2 are also predisposed to schwannomas in other locations, and to other nervous system tumours such as meningiomas and ependymomas. (bmj.com)
In distinction, an individual is diagnosed to have neurofibromatosis 2 if the person has bilateral eighth nerve masses seen with appropriate imaging techniques or a first degree relative with neurofibromatosis 2 and either: (1) a unilateral eighth nerve mass, (2) Two or more of the following: neurofibroma, meningioma, glioma, schwannoma, or juvenile posterior subcapsular lenticular opacity. (medlink.com)
Coronal T1-weighted MRI with gadolinium enhancement of a sphenoid wing meningioma with some degree of encasement of bilateral cavernous sinuses. (medscape.com)
Intense enhancement of bilateral CPA schwannomas (s) and left sphenoid wing meningioma (m). (pacs.de)

The majority of meningiomas are benign (90%), about 6% are atypical, and 2% are malignant . (wikidoc.org)
Atypical meningiomas (WHO grade II, which account for 18% of meningioma cases) exhibit increased tissue and cell abnormalities. (aans.org)
Atypical meningiomas have a higher likelihood of recurrence than benign meningiomas (WHO grade I). (aans.org)
Malignant meningiomas (WHO grade III) show increased cellular abnormalities and grow at a faster rate than benign and atypical meningiomas. (aans.org)
He is involved in ongoing research reviewing patterns of distant recurrence in adult gliomas, diagnostic imaging features of primary central nervous system lymphomas, and effects of upfront radiation on outcomes in atypical meningiomas. (columbia.edu)
In contrast, meningiomas with mutant NF2 and/or chromosome 22 loss were more likely to be atypical, showing genomic instability, and localizing to the cerebral and cerebellar hemispheres. (scienceopen.com)
Medical treatment is reserved for the following: atypical and malignant meningiomas as an adjunct to surgery, partially resected benign meningiomas, and recurrence of meningiomas after a surgical resection. (medscape.com)

By understanding the relationship between overproduction of PrPC and Merlin deficiency in the development of schwannoma and meningioma, we have made a significant stride forward in the search for a drug treatment for NF2. (plymouth.ac.uk)

Schwannomas and meningiomas cause significant morbidities, and an FDA-approved medical therapy is currently not available. (medworm.com)
In addition to NF2 disease there could be potential benefit for other cancers with the same mutations, including mesothelioma (usually linked to exposure to asbestos), breast cancer, colorectal carcinoma, melanoma, glioblastoma and spontaneous schwannomas and meningiomas occurring independently of NF2. (plymouth.ac.uk)
Early phase clinical studies of AR-42, a histone deacetylase inhibitor, for neurofibromatosis type 2-associated vestibular schwannomas and meningiomas. (bvsalud.org)

There may be genetic mutations involved in the development of a meningioma, some of the genes involved includes NF2 , MEG3 , NDRG2 , and SMARCE1 . (wikidoc.org)
The Washington University Neurofibromatosis Center , in collaboration with Barnes-Jewish Hospital and the Alvin J. Siteman Cancer Center, have opened the only program in the Midwest specializing in the clinical care of patients with neurofibromatosis type 2 (NF2)-a genetic disorder affecting one in 40,000 people worldwide. (wustl.edu)
Tia Leigh, 14, from Cwmbran, Wales, was diagnosed with the genetic disorder neurofibromatosis type 1 as a baby. (medworm.com)
A genetic study of type 2 neurofibromatosis in the United Kingdom. (bmj.com)
Integrating genetic, epigenetic, transcriptomic, biochemical, proteomic, and single-cell approaches, we find meningiomas are composed of three DNA methylation groups with distinct clinical outcomes and biological drivers. (genomeweb.com)
Neurofibromatosis refers to several related disorders that have overlapping clinical manifestations but that are now understood to have distinct genetic causes. (msdmanuals.com)
Neurofibromatosis 1 is an autosomally dominant inherited genetic disorder that has variable clinical manifestations. (medlink.com)
Loss of DNA on chromosome 22 has been shown in 40% of meningiomas, and, in cytogenetic studies, genetic abnormalities at the level of chromosome 22 are seen frequently in meningiomas with loss of a copy of chromosome 22 as the most commonly reported abnormality. (medscape.com)

About 95% of vestibular schwannomas are sporadic and unilateral, and about 5% occur in the inherited disorder neurofibromatosis type 2 (NF2), an autosomal dominant disorder with an incidence of approximately 1 in 40 000 live births. (bmj.com)

Objective To assess the risk of meningioma associated with use of high dose cyproterone acetate, a progestogen indicated for clinical hyperandrogenism. (bmj.com)
After discontinuation of cyproterone acetate for one year, the risk of meningioma in the exposed group was 1.8-fold higher (1.0 to 3.2) than in the control group. (bmj.com)
Changes in the 5′ half of the gene have a higher risk of meningioma than do changes in the 3′ half. (unboundmedicine.com)

The majority of meningiomas are benign . (wikidoc.org)

NDRG2 (N-Myc downstream-regulated gene 2): Down regulation of this gene expression at the mRNA level is associated with the malignant progression and predisposition to recurrence of meningiomas. (wikidoc.org)

The signs and symptoms of neurofibromatosis type 2 usually appear during adolescence or in a person's early twenties, although they can begin at any age. (medlineplus.gov)
Neurofibromatosis type 2 has an estimated incidence of 1 in 33,000 people worldwide. (medlineplus.gov)
Variants (also known as mutations) in the NF2 gene cause neurofibromatosis type 2. (medlineplus.gov)
Neurofibromatosis type 2 is considered to have an autosomal dominant pattern of inheritance. (medlineplus.gov)
Subjects with Neurofibromatosis Type 2 (NF2) and progressive vestibular schwannoma (VS) will be treated with crizotinib administered orally. (uclahealth.org)
The WHO classification scheme recognizes 15 variations of meningiomas according to their cell type as seen under a microscope. (aans.org)
Schwannomas are benign tumours of the nervous system that are usually sporadic but also occur in the inherited disorder neurofibromatosis type 2 (NF2). (bmj.com)
Three tumours had gain on 17q, which has also been reported in malignant peripheral nerve sheath tumours that are associated with neurofibromatosis type 1. (bmj.com)
Neurofibromatosis type 1 (NF1, or von Recklinghausen disease) is most prevalent, occurring in 1 of 2500 to 3000 people. (msdmanuals.com)
Neurofibromatosis type 2 (NF2) accounts for 10% of cases, occurring in about 1 of 35,000 people. (msdmanuals.com)
Schwannomatosis, a rare disorder, is classified as a 3rd type of neurofibromatosis. (msdmanuals.com)
Neurofibromatosis Type 1 (NF1) occurs in about 1 in 3,000 to 4,000 babies in the U.S. NF1 is an autosomal dominant disorder. (uhhospitals.org)
Neurofibromatosis Type 2 (NF2) is less common, is also an autosomal dominant disorder. (uhhospitals.org)
This is the more common type of neurofibromatosis. (uhhospitals.org)
Abnormalities of chromosome 22 have been associated with type II neurofibromatosis. (medscape.com)
Neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2) are neurocutaneous syndromes (phakomatoses). (unboundmedicine.com)
Medicine Central , im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688570/1.1/Neurofibromatosis_Type_2. (unboundmedicine.com)
The TAND checklist: a useful screening tool in children with tuberous sclerosis and neurofibromatosis type 1. (asperbio.com)
Neurofibromatosis type 1 revisited. (asperbio.com)
The patient also had multiple schwannomas.The diagnosis was a meningioma in a patient with neurofibromatosis Type 2. (pacs.de)
Combined translabyrinthine, anterior petrosal approach for resection of collision vestibular schwannoma and petrous apex meningioma in neurofibromatosis type 2, with auditory brainstem implant placement. (ucsd.edu)
Comparative clinical and genomic analysis of neurofibromatosis type 2-associated cranial and spinal meningiomas. (cdc.gov)

Intradural extramedullary meningioma in the lower thoracic region. (medscape.com)

Monosomy of chromosome 22 has been reported to occur in 70-80% of meningiomas. (medscape.com)

In this review, we highlight current knowledge about meningioma epidemiology and etiology and suggest future research directions. (scienceopen.com)

Seen in meningiomas undergoing malignant histological progression. (wikidoc.org)

Characteristics of radiation-induced meningiomas include an average latent period of 36-38 years for patients who were exposed to low-dose radiation to the head, whereas patients who develop meningiomas after exposure to high-dose radiation may show signs as early as 5 years postradiation. (medscape.com)

Participants must meet the following criteria on screening examination to be eligible to participate in the study: Patients must have a confirmed diagnosis of neurofibromatosis 2 by fulfilling National Institute of Health (NIH) criteria or Manchester criteria, or by detection of a causative mutation in the NF2 gene. (uclahealth.org)

U]sing bioinformatic pipelines that account for the impact of CNVs on beta methylation detection, we identify three robust DNA methylation groups of meningiomas with distinct clinical outcomes, biological drivers, and therapeutic vulnerabilities," Raleigh wrote, adding that "ours is the first meningioma epigenetic study to validate all the bioinformatic groups we report using mechanistic and functional approaches across meningioma cells, organoids, xenografts, and patients. (genomeweb.com)
These findings underscore the importance of DNA methylation profiling for meningioma patients, which (we anticipate) will ultimately allow for selection or enrollment of clinical trials of cell cycle inhibitors or other molecular therapies," Raleigh explained. (genomeweb.com)
Large and giant medial sphenoid wing meningiomas involving vascular structures: clinical features and management experience in 53 patients. (medscape.com)

The extent to which immunologic factors influence meningioma etiology has been largely unexplored. (scienceopen.com)
A common etiology for meningiomas is radiation exposure in the range of 132-315 roentgens, which is equivalent to rad dose of 1-3 Gy. (medscape.com)
The progesterone receptor is the best candidate as an etiology for meningiomas. (medscape.com)

18 Despite this early recognition, these meningiomas are frequently grouped with suprasellar meningiomas or with meningiomas of the sphenoid ridge, 6, 11, 23, 24, 41, 51 masking their ominous course. (thejns.org)
These meningiomas may be associated with hyperostosis of the sphenoid ridge and may be very invasive, spreading to the dura of the frontal, temporal, orbital, and sphenoidal regions. (medscape.com)

E ditor -Neurofibromatosis 2 (NF2) is a rare autosomal dominant disease that is characterised by benign nervous system tumours, skin lesions, and ocular abnormalities. (bmj.com)

DNA diagnosis of neurofibromatosis 2. (nih.gov)

Patients with neurofibromatosis 1 have been reported to be at an increased risk for several malignancies. (medworm.com)
Due to the potential increased susceptibility of patients with neurofibromatosis 1 to the deleterious side effects of radiation therapy, such as mutagenesis and vasculopathy, alternative treatments are required for patients with progressive disease. (medlink.com)

Genomic analysis of non-NF2 meningiomas reveals mutations in TRAF7, KLF4, AKT1, and SMO. (scienceopen.com)

Analysis of the neurofibromatosis 2 gene in human ependymomas and astrocytomas. (nih.gov)
This condition is characterised by the development of multiple tumours of the nervous system such as schwannomas, meningiomas and ependymomas, each associated with mutations in a gene coding for a tumour suppressor called Merlin. (plymouth.ac.uk)

Some meningiomas may be positive for progesterone receptors on histological examination. (wikidoc.org)
Meningiomas may possess receptors for platelet derived growth factor, vascular endothelial growth factor (VEGF), glucocorticoid , and epidermal growth factor . (wikidoc.org)
Blankenstein MA, Blaauw G, Mulder E. Search for estrogen receptors in human meningioma tissue sections with a monoclonal antibody against the human estrogen receptor. (medscape.com)
Initially, interest was focused on estrogen because it had been found in one series that 30% of meningiomas have estrogen receptors. (medscape.com)
Progesterone receptors have been shown to be expressed in 81% of women and in 40% of men with meningiomas. (medscape.com)

Meningiomas located in the anterior skull base and middle skull base, particularly the medial third of the middle skull base, involving the spheno-orbital region, appeared to be specific to cyproterone acetate. (bmj.com)
These non-NF2 meningiomas were clinically distinctive-nearly always benign, with chromosomal stability, and originating from the medial skull base. (scienceopen.com)

Meningioma arises from the arachnoid "cap" cells, which are normally involved in the protection of the central nervous system by forming a thick envelope of meninges around the brain and spinal cord . (wikidoc.org)
Due to meningioma, there is pressure on the nerves of the brain, by which the person has to face many minor problems related to the nervous system. (cowurine.com)

Hormonal factors (eg, estrogen, progesterone) have been studied extensively as risk factors for meningiomas because of the striking predominance of meningiomas in women. (medscape.com)

Spinal angiography is performed for surgical planning if the meningioma is in the lower thoracic region, regardless of the side of the meningioma. (medscape.com)
They are second only to clival meningiomas in surgical mortality and morbidity rates, failure of total removal, and high rate of recurrence. (thejns.org)
Scarone P, Leclerq D, Heran F, Robert G. Long-term results with exophthalmos in a surgical series of 30 sphenoorbital meningiomas. (medscape.com)
Sphenoid Wing en plaque meningiomas: Surgical results and recurrence rates. (medscape.com)

Neurofibromatosis is a neurocutaneous syndrome (a syndrome with neurologic and cutaneous manifestations). (msdmanuals.com)

Some meningiomas are found along the dural lining in the venous sinuses of the brain and skull base - locations where arachnoid cap cells are most abundant. (aans.org)

These variations are called meningioma subtypes - the technical term for these cell variations is histological subtypes. (aans.org)
Collectively, these findings identify distinct meningioma subtypes, suggesting avenues for targeted therapeutics. (scienceopen.com)

Participants had at least one reimbursement for high dose cyproterone acetate and no history of meningioma or benign brain tumour, or long term disease status. (bmj.com)

The author reviews the role of chemotherapy for gliomas associated with neurofibromatosis 1 and its efficacy on disease control and visual outcome. (medlink.com)
Gliomas in children with neurofibromatosis 1, if requiring treatment, may be chemotherapy-sensitive, and radiotherapy should be used as a last resort, given potential long-term sequelae. (medlink.com)
Bernstein M, Villamil A, Davidson G. Necrosis in a meningioma following systemic chemotherapy. (medscape.com)

Inherited susceptibility to meningioma is suggested both by family history and candidate gene studies in DNA repair genes. (scienceopen.com)

The data presented here demonstrate a need for DNA methylation profiling to stratify meningioma patients for molecular therapy," co-senior and co-corresponding author David Raleigh, a radiation oncology and neurological surgery researcher at UCSF, and his colleagues wrote. (genomeweb.com)

On microscopic pathology, some of the characteristic findings of a meningioma include mitotic figures, necrosis , interdigitating processes, and brain invasion. (wikidoc.org)

New research published today, 10 July 2017, online in the journal Oncogene could offer hope to the thousands of, mainly young, people affected by the hereditary condition Neurofibromatosis 2 (NF2). (plymouth.ac.uk)

High dose ionizing radiation exposure is an established risk factor for meningioma, and lower doses may also increase risk, but which types and doses are controversial or understudied. (scienceopen.com)
Radiation therapy in the treatment of partially resected meningiomas. (medscape.com)

S pinal meningiomas are encountered mostly in the thoracic region in middle-aged women, 1, 11 but occur elsewhere along the spinal canal. (thejns.org)

In rare cases, spinal meningiomas may have an extradural component, which can cause enlargement of the neuroforamen. (medscape.com)
3 however, extradural meningiomas have been described, 4, 5 and all reported cases were reviewed recently. (thejns.org)

Anatomy10

Diseases47

Chemicals and Drugs2

Analytical, Diagnostic and Therapeutic Techniques and Equipment7

Psychiatry and Psychology1

Phenomena and Processes7

Information Science1

Health Care1

Tumors33

Intracranial13

Tumor9

Multiple meningiomas2

Bilateral4

Atypical7

Schwannoma and Meningioma1

Schwannomas and meningiomas3

Genetic8

Vestibular schwannomas1

Risk of meningioma3

Majority of meningiomas1

Recurrence of meningiomas1

Type22

Intradural extramedullary1

Chromosome 221

Epidemiology1

Malignant1

Develop meningiomas1

Mutation1

Clinical3

Etiology3

Sphenoid ridge2

Abnormalities1

Diagnosis of neurofibromatosis1

Patients with neurofibromatosis2

Genomic analysis1

Ependymomas2

Receptors5

Medial2

Nervous2

Progesterone1

Surgical4

Neurocutaneous1

Skull base1

Subtypes2

Benign brain1

Chemotherapy3

Susceptibility1

Oncology1

Necrosis1

Hereditary1

Radiation2

Occur1

Extradural2