Central nervous system tumors in Mexican children

Five hundred and seventy primary central nervous system (CNS) tumors from the Department of Pathology at the National Institute of Pediatrics in Mexico City, collected from 1970 to 1989, were histologically reclassified in order to find out their relative incidence as well as their outstanding features. With this, we could establish a frame of reference for our local population, contributing to the epidemiological analysis of these entities. All the tumors were examined independently by two pathologists (C.R. and M.R.), using the classification of Rorke et al. Histological type, patient age and sex, and tumor location were analyzed. CNS tumors were the secondmost frequently encountered solid tumors, after lymphomas, and were increasing in incidence at a rate of 2.2 annually. Children in the age group 0–9 years were most often affected, and there was a predominance of male patients. Astrocytoma and medulloblastoma were the most common tumor types. The infratentorial region was the most frequent tumor location in the 2-to 9-year age group. By contrast, in the under 2-year-olds a supratentorial location was more frequent, and the incidence of germ cell tumors was proportionally high. In general, some histological types seemed to be associated with particular age groups. Although we found primitive neuroectodermal tumors to be the fifth most common at all ages (except for medulloblastoma), many other authors do not report a similar finding.     

Pediatric brain tumors: Current treatment strategies and future therapeutic approaches

Pediatric CNS tumors are the most common solid tumors of childhood and the second most common cancer after hematological malignancies accounting for approximate 20 to 25% of all primary pediatric tumors. With over 3,000 new cases per year in the United States, childhood CNS tumors are the leading cause of death related to cancer in this population. The prognosis for these patients has improved over the last few decades, but current therapies continue to carry a high risk of significant side effects, especially for the very young. Currently a combination of surgery, radiation, and chemotherapy is often used in children greater than 3 years of age. This article will outline current and future therapeutic strategies for the most common pediatric CNS tumors, including primitive neuroectodermal tumors such as medulloblastoma, as well as astrocytomas and ependymomas.     

Trends in incidence of brain and central nervous system tumors in Norway, 1970-1999

The aim of this study was to investigate trends in the incidence of childhood and adult brain and central nervous system (CNS) tumors in Norway from 1970 through 1999. In this period, a total of 14,641 patients were diagnosed with a primary benign or malignant neoplasm of the brain and CNS. Age-adjusted incidence rates were reported together with results of loglinear regression and an age-period-cohort model based on the Poisson regression model. In children (<15 years), the proportion of brain and CNS tumors was 28.2% (1,042/3,697) of all new cancer cases compared with 2.8% in adults (13,599/492,237). The overall rate of brain and CNS tumors increased during the study period from 6.49 to 12.02 cases per 100,000 person-years. A trend of leveling off in incidence of most tumor categories during the study period was indicated with a possible continuing increase in the period 1995-1999, especially in the age group 0-4 years and in patients aged 60 years or more. Age and period together provided a satisfactory model in patients being <60 years of age and less completeness of diagnosis was found in males compared with females, possibly due to the distribution in males of more aggressive tumors.     

Epidemiology of Primary Brain and Central Nervous System Tumors in Korea

Objective

The aim of this report is to provide accurate nationwide epidemiologic data on primary central nervous system (CNS) tumors in Korea. Despite its importance, there are no accurate statistics on primary CNS tumors in Korea. We analyzed primary CNS tumors diagnosed in 2005 from the nationwide registry.

Methods

Data on primary CNS tumors diagnosed in 2005 were collected from the Korean Central Cancer Registry and the Korean Brain Tumor Society. Crude and age-standardized rates were calculated in terms of gender, age, and histological type. Tumors of uncertain histology were investigated individually at the corresponding hospitals and had their diagnoses confirmed.

Results

A total of 5,692 patients diagnosed with primary CNS tumors in 2005 were included in this study. CNS tumors occurred in females more often than in males (female to male, 1.43 : 1). The most common tumor was meningioma (31.2%). Glioblastoma accounted for 30.7% of all gliomas, and 19.3% of all malignant primary CNS tumors. In children under 19 years of age, both germ cell tumor and embryonal/primitive/medulloblastoma were the most common tumors.

Conclusion

This article is the first nationwide primary CNS tumor epidemiology report in Korea. Data from this study should provide valuable information regarding the understanding of primary CNS tumors epidemiology in Korea.     

Sunday July 2, 2006 9:30–11:30Hall 3A Discussion Group SessionLessons learned from local epidemiological studies

¹ A. Õun (   ¹ Department of Neurology and Neurosurgery, University of Tartu, Estonia )
Epidemiological data about epilepsy from central and eastern Europe is controversial. A modern study on prevalence and incidence of epilepsy in Estonia has been carried out on children–prevalence of active epilepsy was 3.6/1,000, and incidence 45/100,000 person-years being essentially similar to those in developed countries. In an adult populaton review of all databases and lists related to epilepsy in Tartu supplemented by re-examination of patients to identify all persons with active epilepsy aged ≥20 years on January 1, 1997. Special attention was paid to extensive adoption of definitions and criteria proposed by Guidelines for epidemiologic studies. The incidence rate was 35/100,000 person-years. The age-specific rates tended to increase with advancing age. The prevalence rate was 5.3/1,000. The largest syndromic categories were localization-related symptomatic and cryptogenic epilepsies, which are very likely due to the age-distribution of the study. The risk factors for epilepsy were identified in 39.6% cases of the prevalent cases. Of the subjects 19% did not take an entiepileptic drug on the prevalence day, 83% of them were taking a single drug. The most common agent was carbamazepine (68%), followed by barbiturates. Epidemiologic rates in the adult, as well as in the total population of Tartu was comparable to those reported from the developed countries. The large percentage of medication-free epileptics, notably small figures for polytherapy, and the associating low aggregate antiepileptic drug sales in Estonia, indicate a tendency for moderate medication in the epileptic population.     

Central nervous system (CNS) tumor trends in children in a western Canadian province: a population-based 22-year retrospective study

In Canada, CNS tumors accounted for nearly 22% of the new childhood cancer diagnoses during 1995-2000 in the ≤ 15 year age group. The study's objective was to describe children and youth (age <20 years) diagnosed with CNS tumors in Alberta, Canada during a 22-year period using population-based data. The Alberta Cancer registry was used to extract information, including sex, age and geography, on all CNS (ICCC-3 III) tumor diagnoses during April 1, 1982, and March 31, 2004. Analyses included population summaries and rates. During 22 fiscal years, 568 Alberta children were diagnosed with CNS tumors and nearly 82% of the cases were malignant (461). The majority of cases were male (322, 57%) and the median age at diagnosis was 8 years. The crude rate per 100,000 children increased over the study period from 2.1 in 1983/1984 to 4.2 in 2003/2004. Astrocytoma was the most common diagnosis (257, 45%), followed by medulloblastoma (12%), mixed and unspecified glioma (9%) and ependymoma (9%). There were 86 diagnoses of juvenile pilocytic astrocytoma (55% male) and the crude rates per 100,000 increased during the study (<0.5 in the early years to 1.15 in 2003/2004). Our data suggests an emerging trend with the latter few years having a seemingly higher standardized incidence rate than earlier years. Further study is required to determine if the trend persists.     

Incidence of Parkinson's disease in estonia

The incidence of Parkinson's disease (PD) was studied over a 9-year period (1990-1998) in Tartu county, Estonia, with a mean population of 156,417. Based on 264 patients, the average crude incidence rate of PD was 18.8 per 100,000 person-years. The age-adjusted incidence was 16.8/100,000; 18.3 for the urban group and 14.0 for the rural group; 16.6 for men and 17.1 for women. The age-specific incidence was 1.0/100,000 in the age range 30-39 years, reached a maximum of 117.2/100,000 in the age range 70-79 years and declined in the elderly. The incidence of PD was comparable with that found in developed countries, except for a slightly higher (not significant) incidence rate in the urban compared with the rural population in Estonia.     

Epidemiology of childhood brain tumors

The German Childhood Cancer Registry (GCCR) was established in 1980. From 1980 to 1997 4,447 CNS tumours in children below 15 years of age have been reported to the registry. From 1988 to 1997 the average annual incidence was 2.5/100,000 children. This corresponds to 19.2% of the registered diseases. Compared with incidence rates reported from other developed countries one can estimate that there is about 25% underreporting of CNS tumours in the GCCR. This is in contrast to the relatively complete ascertainment of other childhood malignancies (above 95%). Based on 3012 incident cases from 1988 to 1997 the Kaplan-Meier-estimate of 5-year-survival probability is 65% for all CNS tumours and 54% for PNETs and gliomas. An active long-term-follow-up shows that 30% of 142 children with secondary neoplasms following primary lymphoid leukaemias developed a CNS tumour. Following any primary childhood malignancies 45 CNS tumours were recorded, amongst these were one third leukaemias. The paper reports first results from a population based case control study which included 466 children with CNS tumours and 2,458 healthy controls. These indicate an increased risk for children with low birth weight and for children whose mothers smoked during pregnancy. Additional data can be found in the Internet (http:?info.imsd.uni-mainz.de/K_Krebsregister).     

Brain tumors in Yamaguchi Prefecture--incidence through 4 years

The incidence of brain tumors was studied in Yamaguchi prefecture of about 1,600,000 population. All of the brain tumor patients admitted to the neurosurgical hospitals in Yamaguchi prefecture were registered. Cases of osteoma, lipoma, scalp tumor and spinal tumor were excluded. From 1986 through 1989, 726 cases were registered. 135 recurrent cases were included. Therefore first-diagnosed primary brain tumors were selected to calculate the true incidence. The number of cases of primary brain tumor was 478 and showed female preponderance (male/female: 207/271). The incidence of primary brain tumor was 7.5/100,000/year (male/female: 6.8/8. 1). No difference was present between the incidence in cities and that in rural districts. Percentages of representative tumors were 28.2% for glioma, 32.8% for meningioma, 13.0% for pituitary adenoma and 10.7% for neurinoma. Age-adjusted incidence was 2.1/100,000/year for glioma and 2.1/100,000/year for meningioma. The incidence of glioma was lower and that of meningioma was higher in Yamaguchi prefecture than those in other reports. Compared with the Brain Tumor Registry of Japan (1969-1983), the percentage of meningioma cases was large in Yamaguchi prefecture. This difference owed partly to the increased number of population over age of 40's in Yamaguchi prefecture. The peak of age distribution was present in age of 50's in Yamaguchi prefecture and in age of 30's and 40's in Brain Tumor Registry of Japan. The peak of age distribution shifted to older ages in Yamaguchi and the difference was conspicuous in age of 60's. This peak consisted of mainly cases of meningioma and partly those of glioma.(ABSTRACT TRUNCATED AT 250 WORDS)     

Central nervous system involvement in Von Hippel-Lindau disease

Fifty individuals with Von Hippel-Lindau disease (VHL) were studied with gadolinium-enhanced magnetic resonance imaging (MRI) to determine the frequency and distribution of CNS lesions. The associated clinical features were also reviewed. Thirty-six (72%) of the 50 had 1 or more CNS tumors. The most frequently affected sites in the CNS excluding the retina were the cerebellum (52%), spinal cord (44%), and brainstem (18%). New regional predilections for the craniocervical junction and conus medullaris were demonstrated by this study. Forty-one percent of all VHL patients with CNS tumors were neurologically asymptomatic: cerebellar tumors (50%), spinal cord tumors (50%), and brainstem tumors (44%) were often without clinical signs or symptoms. Multiple lesions were common. The mean age of all VHL patients (34.5 years) was similar to the mean age of all CNS VHL patients (34.4 years), suggesting a lack of age association. CNS lesions commonly occurred in the 2nd decade of life. All patients at risk for VHL should be evaluated using gadolinium-enhanced MRI after 10 years of age, although ophthalmic examination should be initiated within the 1st 2 years of life. Enhanced MRI is particularly useful in the detection of CNS tumors in patients with the VHL gene.     

Average years of potential life lost in those who have died from brain and CNS tumors in the USA

The aim of this study was to quantify the impact of all primary malignant and nonmalignant brain and central nervous system (CNS) tumors on mortality in the USA in terms of years of life lost, an indicator of premature mortality in the population. In this study, US mortality data for the year 2001 from the National Center for Health Statistics and life tables from the US Census Bureau were used. Person-years of potential life lost (PYPLL) and average years of potential life lost (AYPLL) were calculated for all deaths due to brain and CNS tumors as an underlying cause. 16,819 deaths due to brain and CNS tumors occurred in 2001, leading to a total loss of 357,483 person-years of life and an average loss of 21.3 years (AYPLL). Hispanic men and women had an AYPLL of 31.7 and 33.9 years, respectively, a figure substantially higher than other race and ethnicity categories. Malignant tumors led to an AYPLL of 25 years, which was significantly higher than the AYPLL of 19 years due to nonmalignant tumors. The relative impact of tumors arising in the various anatomical sites in the brain and CNS among adults and among children was not similar. The use of population survival indicators, such as PYPLL and AYPLL, will help better understanding of the effect of racial, ethnic, gender and age differences on the mortality due to malignant and nonmalignant brain and CNS tumors. Information on years of life lost, as well as mortality data, will be useful in focusing research priorities and resources in this field.     

Incidence of central nervous system tumors in Gironde, France

The incidence of CNS tumors is subject to geographical and temporal variations which are poorly understood. The incidence of these tumors was studied in Gironde, a department of southwestern France with 1,058,911 inhabitants older than 16 years. We recorded any malignant or benign central nervous system (CNS) tumor diagnosed between May 1999 and April 2001 in adults living in Gironde. Three hundred and twenty-nine CNS tumors were diagnosed during the study period. The incidence of CNS tumors in adults was therefore 15.5 per 100,000. Overall, the incidence according to sex was 14.7 for males and 16.2 per 100,000 for females. The incidence rate increased according to age up to 80 years (3.7 per 100,000 for 20-29 years of age to 33.4 per 100,000 for 70-79 years of age) and reduced thereafter for gliomas. Our estimation of the incidence of CNS tumors ranks high among the earlier reports. Further analytic studies are ongoing.     

Pediatric central nervous system tumors: review of a single Portuguese institution

Introduction

Despite being the second most frequent tumor in children, pediatric central nervous system (CNS) tumors are rare, and the published European epidemiological data is limited. Our goal is to present the first surgical series of pediatric CNS tumors in Portugal and to review other similar worldwide series.

Methods

Retrospective review of all patients younger than 19 years old, operated to a CNS tumor in the Neurosurgery Department at Hospital de Santa Maria (Lisbon, Portugal) between January 2004 and December 2014. Demographic data, tumor location, clinical data, histopathology, and surgical treatment were analyzed and compared to surgical series of pediatric CNS tumors published in PubMed indexed journals over the last 20 years.

Results

We performed 253 surgeries in 215 patients, with a male:female ratio of 1.2:1 and a mean age of 9.2 years old. Primary brain tumors accounted for 95 % of all tumors and had more often a supratentorial location. Tumors of neuroepithelial tissue, particularly astrocytic tumors, embryonal tumors, neuronal and mixed neuronal-glial tumors, and oligodendrogliomas accounted for 81 % of cases. A gross-total resection was achieved in most cases. There was no mortality, and the overall morbidity was low.

Conclusions

The demography, topography, and clinical presentation of the tumors and the surgical results of this series are comparable to other European ones. We found a higher incidence of neuronal and mixed neuronal-glial tumors and oligodendrogliomas and a slight lower incidence of ependymomas. Our results should encourage further national multi-institutional studies to better characterize these tumors in the pediatric population.

     

Etiology of Seizures in the Elderly

Our study covered a 5-year period, and included all patients within a well-defined area who developed seizures after age 60 years. The dominant cause of seizures was a previous stroke, accounting for 32% of all cases. Tumors accounted for 14%, and the cause of seizures remained unknown in 25%. Seizures were recurrent in greater than 80% of patients with first seizure greater than 6 months after stroke. Fifteen of 21 patients with tumors had metastatic tumors. Of the six patients with primary brain tumors, five had malignant gliomas and one had a meningioma. We conclude that epilepsy with onset after age 60 years is more often symptomatic than is epilepsy in younger patients; since seizures were the first sign of a central nervous system (CNS) disease in half of the patients with brain tumors, careful investigation is necessary to reach a correct diagnosis.     

Epidemiology of central nervous system tumors in childhood and adolescence based on the new WHO classification

METHOD: The topography, sex distribution and histology of 340 primary CNS tumors in children up to 17 years of age were investigated and (re-)classified according to the latest WHO classification of nervous system tumors. RESULTS: Overall the prevalence for boys (60.9%) was higher and supratentorial locations predominated (53.3%); there was an even distribution of low-grade WHO I/II (51.5%) and high-grade WHO III/IV (48.5%) tumors. Boys were more commonly affected in all age groups throughout childhood and adolescence. Infratentorial location was more common between the ages of 3 and 11 years (57.5%). High-grade tumors were more frequently encountered up to the age of 5 years (53.2%). The main histological entities were pilocytic astrocytomas (23.5%), followed by medulloblastomas (16.3%), ependymomas (10.1%), anaplastic astrocytomas and glioblastomas (7.2% each), and craniopharyngiomas (5.6%); astrocytomas overall accounted for 47.3% of pediatric brain tumors. Rarer entities included germ cell tumors, gangliogliomas, and meningiomas (2.5% each), supratentorial PNET and pineal parenchymal tumors (1.9% each), atypical teratoid/rhabdoid tumors (1.3%), choroid plexus tumors (0.9%), and desmoplastic infantile astrocytomas and dysembryoplastic neuroepithelial tumors (0.6% each). A meta-analysis of 10,582 childhood brain tumors accumulated from 16 international surveys revealed a male-female ratio of 1.29 and a supra-/infratentorial ratio of 0.92. The most common histological diagnoses were astrocytomas (37.6%), medulloblastomas (17.7%), ependymomas (9.9%), craniopharyngiomas (7.3%), and germ cell tumors (4.4%). CONCLUSIONS: Pediatric brain tumors vary considerably in their histological, topographical and gender distribution throughout childhood and adolescence, reflecting different dynamics of individual tumor entities as well as a susceptibility to their occurrence during certain periods of a child's life. Although at times difficult to characterize, pediatric CNS tumors can be satisfactorily classified according to the latest WHO classification of nervous system tumors.     

Incidence and pattern of cerebrovascular diseases in Benghazi, Libya.

During a 12 month study period, a total of 329 incident cases of stroke occurred in Benghazi, Libya. This provided an unadjusted annual incidence rate for stroke of 63 per 100,000 population; the corresponding rates for the male and female were 69 and 58 per 100,000 respectively. The sex dependent difference in the incidence was not statistically significant. Cerebral infarction was the commonest type of stroke accounting for 80.9%. The incidence rates increased with age in all categories of stroke. Hypertension and ischaemic heart disease were common risk factors among the male patients, while diabetes and hypercholesterolaemia were more frequent in the female patients.     

Frequency of seizures in patients with newly diagnosed brain tumors: a retrospective review

Brain tumors may lead to symptomatic epilepsy. A retrospective analysis was undertaken to evaluate the frequency of seizure as the presenting symptom leading to brain tumor diagnosis in adults. One hundred and forty-seven consecutive patients with newly diagnosed brain tumors were analyzed regarding the frequency of seizures as the initial presenting symptoms and those subsequently developing seizures. One hundred twelve patients had primary central nervous system tumors (CNS) and 35 had metastatic lesions. Statistical evaluation was carried out using the Chi-square test with p values of <0.05 considered to be statistically significant. Astrocytomas and meningiomas were the most common primary CNS tumors in this study. Of these, oligodendrogliomas and grade 2 astrocytomas were significantly more likely to present with seizures (p<0.001). Seizures were a frequent presenting symptom, occurring in over 38% of those with primary brain neoplasms and 20% of those with cerebral metastases. Primary location of tumor also correlated amongst primary CNS tumors and was associated with a trend in seizure risk: parietal (80%); temporal (74%); frontal (62%); and occipital (0%) (p<0.5). The findings highlight the importance of obtaining appropriate evaluation for underlying malignancy in adults with new-onset seizures as well as provide more information to the patient for prognosis and counseling.     

Incidence of intracranial tumours in the Lothian region of Scotland, 1989-90.

OBJECTIVE--To determine the incidence of primary and secondary intracranial tumours in the Lothian region of south east Scotland. METHODS--A population based study was performed. Patients from Lothian with incident intracranial tumours diagnosed in 1989 and 1990 (by CT or histology) were identified retrospectively using multiple sources. Differences in incidence by tumour type, age, sex, and socioeconomic status were examined. RESULTS--Four hundred and forty two patients with incident intracranial tumours were identified (228 primary tumours and 214 secondary tumours). The crude yearly incidences of primary and secondary tumours were 15.3 and 14.3 per 100,000 respectively. The commonest primary tumours were neuroepithelial tumours (53.5%), meningeal tumours (19.5%), and sellar tumours (16.5%). About 50% of patients with secondary tumours had an underlying lung cancer. The incidence of primary and secondary tumours increased markedly with age. Meningeal tumours were more common in women, and neuroepithelial tumours were more common in those who lived in more affluent areas. CONCLUSIONS--The incidence rates of primary and secondary intracranial tumours in Lothian were more than twice those previously reported in the United Kingdom. Intracranial tumours are a significant cause of morbidity and mortality in the United Kingdom, and further research into their aetiology and treatment is urgently required.     

Pathological spectrum of neuronal/glioneuronal tumors from a tertiary referral neurological Institute

Neuronal/glioneuronal tumors are uncommon neoplasms of the CNS with frequent association with refractory epilepsy. Reports documenting the entire spectrum of neuronal/glioneuronal tumors are scarce in the literature. Zulch et al. from Germany in a large series reported that neuronal/glioneuronal tumors accounted for 0.4% (38/9000 cases) of all brain tumors, with similar incidence reported from Japan (0.4%), with higher incidence from Korea (2.1%). However, data from the Indian subcontinent are lacking. We reviewed 244 cases of neuronal/glioneuronal tumors of the CNS diagnosed over the last decade at our Institute and they constituted 0.86% of all CNS tumors (244/28061) received in that period. Mean age at presentation was 25.06 years (range: 1–75 years) with male preponderance (M : F = 1.54 : 1). The majority occurred in third decade (76 cases, 31.4%), with only few cases occurring beyond fifth decade (13 cases, 5.3%). Ganglioglioma/gangliocytoma (94 cases, 38.52%) was the most frequent followed by central neurocytoma (86 cases, 35.24%), paraganglioma (32 cases, 13.52%), dysembryoplastic neuroepithelial tumors (DNET) (21 cases, 8.6%), desmoplastic infantile astrocytoma/desmoplastic infantile ganglioglioma (DIA/DIG) (6 cases, 2.45%), papillary glioneuronal tumor (PGNT) (3 cases, 1.22%) and rosette‐forming glioneuronal tumor (RGNT) (1 case, 0.4%). Association with seizures was noted in 40.95% of cases. Glioneuronal tumors are an expanding group of tumors with varying spectra of morphologic patterns and biological behavior. An improved understanding has direct clinical implications for optimizing current treatments and developing novel therapeutic approaches. Although most glioneuronal tumors carry a favorable prognosis, other factors such as inaccessibility to surgical resection and rarely, malignant transformation, make it difficult to accurately predict the biological behavior based on histopathology alone. Reliable prognostic markers remain to be defined.     

Incidence of adult epilepsy in Estonia

PURPOSE: To estimate the main incidence-related characteristics of epilepsy in an adult population in Estonia. Epidemiologic data about epilepsy from central and eastern Europe is controversial. METHODS: All existing databases and lists in the area were reviewed to identify all persons with newly diagnosed epilepsy aged > or =20 years during 3 years. Special attention was paid to extensive adoption of definitions and criteria proposed by Guidelines for epidemiologic studies. RESULTS: Crude and age-adjusted incidence rates were 35 per 100,000 person-years. The age-specific rates tended to increase with advancing age. Of the seizure types, partial seizures had highest rates, 27 per 100,000. The largest syndromic categories were localization-related epilepsies. Risk factors for epilepsy were identified in 55.5% cases. CONCLUSIONS: Incidence-related characteristics in the adult population of Tartu was comparable with those reported from the developed countries. The predominance of localization-related syndromes and partial seizures is the result of age distribution of the study.