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    A role for the p53 pathway in the pathology of meningiomas withNF2lossZeNan Chang

    Introduction

    Arising from the arachnoid coverings of the brain and spinal cord, meningiomas account for1520% of primary neoplasms of the central nervous system, and have an estimated annual

    incidence of about 6 per 100,000 individuals [1].

    Meningiomas have long been known to exhibit receptors to sexual steroids [44], suggesting

    that hormonal influences may explain the sexually dimorphic characteristics of this disease.

    However, discrepancies on the proliferative effects of sexual hormones on meningiomas and

    failure of variations in sexual receptor expression to explain the increased prevalence of

    meningiomas in women suggests that more complex factors are at play [45].

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    Sukri Rahman

    Meningioma merupakan tumor jinak yang biasanya ditemukan pada meningen otak dan medula spinalis, sangat

    jarang ditemukan di ekstrakranial.

    Meningioma adalah tumor jinak, dengan pertumbuhan tumor yang lambat dan biasanya terjadi di daerah

    intrakranial.1,3,5

    Menurut Longstreth Jr dkk2 angka kejadian meningioma 6 per 1000 populasi atau sekitar 13-26% dari keseluruhan

    tumor intrakranial. Meningioma yang terjadi di ekstrakranial merupakan kasus yang sangat jarang, diperkirakan

    antara 1-2% dari keseluruhan kejadian meningioma.

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    Meningiomas: causes and risk factorsJILL S. BARNHOLTZ-SLOAN, PH.D.,1 AND CAROL KRUCHKO, B.A.2

    MENINGIOMAS account for 30% of all primary brain

    tumor diagnoses in adults in the United States.9

    The overall age-adjusted incidence rate is 4.52 per

    100,000.9 Although age-adjusted incidence rates are reportedly

    similar across racial groups, the incidence in women is

    approximately twice that in men (Table 1).9 The incidence

    increases with increasing age, peaking in the seventh and

    eighth decades of life; these tumors are very rare in children(Fig. 1).9 It is currently estimated that 83% of all meningiomas

    are microscopically confirmed.36 The incidence

    of both diagnostically and nondiagnostically confirmed

    meningiomas increased between 1985 and 1999;36 on average

    the incidence of nondiagnostically confirmed meningiomasincreased significantly at 4.1% per year (95% CI

    2.55.6) potentially reflecting both the increased use of

    improved imaging techniques such as MR imaging and increasednumbers of meningiomas treated with observation

    or primary radiotherapy rather than through surgical intervention.

    The vast majority of meningiomas are considered histologically

    benign (92.8%); only 2.2% are defined as uncertain

    or atypical, and 5% as malignant.9 Five-year survival

    rates are high for this tumor type (reported to be anywherefrom 70 to 95%)60,77,94 and therefore the estimated population

    prevalence (number of individuals living with this

    tumor) is relatively high, 50.4 per 100,000.15 Long survival

    times coupled with potentially significant neurocognitive

    and physical deficits could lead to significant medical costs

    over time. The estimated average years of potential life lost in persons with meningiomas is 13 years,providing further

    evidence of the long-term burden of this disease.110

    In addition to increasing age, the most consistent factorassociated with risk of meningioma is exposure to ionizing

    radiation; many other environmental, lifestyle and genetic

    risk factors have been studied with inconclusive results.12Some of the factors that have been studied are endogenous

    and exogenous hormone use,11,13,33,41,49,51,97,113 cell phone use,10,28,

    30,31,40,42,46,55,65,100 and genetic variants or polymorphisms.

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    DIAGNOSIS AND TREATMENT OF ATYPICAL ANDANAPLASTIC MENINGIOMAS: A REVIEWAshok Modha, M.D.

    Between 13 and 26% of all intracranial

    tumors are meningiomas (9, 12, 24, 29, 48).Meningiomas occur mostly in middle-aged

    or elderly patients, but they can also occurin younger patients with dysgenetic syndromessuch as neurofibromatosis Type 2

    (NF2). The annual incidence rate is approximately6 per 100,000 (19) but some tumorsare only discovered during autopsy. Often,

    they are diagnosed incidentally on brain imagingfor unrelated complaints. A minorityof these tumors demonstrate histopathologicaland clinical features suggesting an aggressive

    potential. These are the atypicaland anaplastic meningiomas. The formerconstitute between 4.7 and 7.2% of meningiomas,

    whereas the latter account for 1.0 to2.8% (9, 12, 24, 48). Some series have shownthat up to 2% of all benign meningiomastransform into malignant forms (2, 51),whereas up to 28.5% of all recurrent benign

    meningiomas will be found to be atypical oranaplastic (2, 18, 19). Hug et al. (17) reportedthat the annual incidence of these tumors inthe United States is approximately 150 to

    225. There is a wide range in the prevalence

    data for these malignant forms because variable

    pathological criteria exist for their classification.Benign meningiomas are more

    prevalent in women, but atypical and anaplasticforms seem to be more common in men (29). The atypicaland anaplastic forms are also more

    common in the cerebral convexities (29).Atypical meningiomas have been reported to occur aftercranial irradiation for other tumors or conditions. These are

    usually found in younger patients (3). This complicationwasfirst reported in 1953, in a child receiving radiation therapyfor

    an optic glioma (17). Subsequently, children undergoingcranialradiation for medulloblastomas, astrocytomas, leukemia,

    and lymphoma have all been reported to developmeningiomas(17). These tumors have also been noted in patients whoreceived low doses of irradiation fortinea capitis or afterexperimental

    radiation treatments during World War II (17).Dental x-rays have also been implicated (17). Often,multiplemeningiomas are found in patients with these risk factors

    (3).

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    Risk of meningioma amongusers of high doses of cyproterone acetate as comparedwith the general population: evidence from a population-based cohortstudyMiguel Gil,1

    Meningiomas are mostly benign, slow growing tumoursoriginating from the arachnoid cap cells. Meningiomas

    have an annual incidence ranging from 3 to 8 per 100 000person-years in the different series, occur more frequentlyin women and its incidence increases with age [1, 2].