A role for the p53 pathway in the pathology of meningiomas with NF2 loss

ZeNan Chang

Introduction
Arising from the arachnoid coverings of the brain and spinal cord, meningiomas account for 1520% 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].

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.

Meningiomas: causes and risk factors

JILL S. BARNHOLTZ-SLOAN, PH.D.,1 AND CAROL KRUCHKO, B.A.2
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 meningiomas increased 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 increased numbers of meningiomas treated with observation or primary radiotherapy rather than through surgical intervention.
MENINGIOMAS

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 anywhere from 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 factor associated with risk of meningioma is exposure to ionizing radiation; many other environmental, lifestyle and genetic risk factors have been studied with inconclusive results.12 Some 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.

DIAGNOSIS AND TREATMENT OF ATYPICAL AND ANAPLASTIC MENINGIOMAS: A REVIEW

Ashok 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 occur in younger patients with dysgenetic syndromes such as neurofibromatosis Type 2 (NF2). The annual incidence rate is approximately 6 per 100,000 (19) but some tumors are only discovered during autopsy. Often, they are diagnosed incidentally on brain imaging for unrelated complaints. A minority of these tumors demonstrate histopathological and clinical features suggesting an aggressive potential. These are the atypical and anaplastic meningiomas. The former constitute between 4.7 and 7.2% of meningiomas, whereas the latter account for 1.0 to 2.8% (9, 12, 24, 48). Some series have shown that up to 2% of all benign meningiomas transform into malignant forms (2, 51), whereas up to 28.5% of all recurrent benign meningiomas will be found to be atypical or anaplastic (2, 18, 19). Hug et al. (17) reported that the annual incidence of these tumors in the 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 anaplastic forms seem to be more common in men (29). The atypical and anaplastic forms are also more common in the cerebral convexities (29). Atypical meningiomas have been reported to occur after cranial irradiation for other tumors or conditions. These are usually found in younger patients (3). This complication was first reported in 1953, in a child receiving radiation therapy for an optic glioma (17). Subsequently, children undergoing cranial radiation for medulloblastomas, astrocytomas, leukemia, and lymphoma have all been reported to develop meningiomas (17). These tumors have also been noted in patients who received low doses of irradiation for tinea capitis or after experimental radiation treatments during World War II (17). Dental x-rays have also been implicated (17). Often, multiple meningiomas are found in patients with these risk factors (3).

Risk of meningioma amongusers of high doses of cyproterone acetate as compared with the general population: evidence from a population-based cohort study Miguel Gil,1
Meningiomas are mostly benign, slow growing tumours originating from the arachnoid cap cells. Meningiomas have an annual incidence ranging from 3 to 8 per 100 000 person-years in the different series, occur more frequently in women and its incidence increases with age [1, 2].