Registration, incidence patterns, and survival trends of central nervous system tumors among children in Germany 1980-2019: An analysis of 40 years based on data from the German Childhood Cancer Registry.

BACKGROUND: Tumors of the central nervous system (CNS) are the second most common type of pediatric cancer in Germany. We aimed to describe registration practice, incidence, and survival patterns for childhood CNS tumors in Germany for the past 40 years. PROCEDURE: Including all CNS tumor cases in children diagnosed at ages 0-14 years registered at the German Childhood Cancer Registry (GCCR) in 1980-2019 (for survival analysis 1980-2016), we calculated age-specific and age-standardized incidence rates (ASIR) over time, average annual percentage changes (AAPC), and 1- and 5-year overall survival. RESULTS: While we observed a pronounced increase in ASIR after the establishment of the GCCR during the 1980s, ASIR for all pediatric CNS tumors combined continued to increase markedly from 28.6 per million in 1990-1999 to 43.3 in 2010-2019 (AAPC = 2.7% in 1991-2010, AAPC = 0.3% in 2010-2019). The 5-year overall survival from CNS tumors improved from 63% in the 1980s, 70% in the 1990s to 79% in 2010-2016. These improvements have occurred across all age groups. Children diagnosed with ependymomas and choroid plexus tumors experienced the strongest increase (from 54% to 81%). CONCLUSIONS: Observed increases in incidence rates for pediatric CNS tumors are likely only partially caused by actual increasing case numbers. The majority is a function of improved registration and, to a minor extent, improvements in diagnostics. Survival from pediatric CNS tumors has, by and large, improved consistently, leading to a growing population of childhood cancer survivors with diverse health biographies and risk of lifelong adverse impact on health and wellbeing.

Clinical and histopathological spectrum of cranial small round cell tumors: An experience from a tertiary care center.

BACKGROUND: Small round cell tumors (SRCTs) are a group of malignant neoplasms with minimal or no differentiation, characterized by the presence of round cells with high nuclear-cytoplasmic ratio. Although SRCTs can occur in any part of the body, involvement of central nervous system (CNS) is uncommon. AIM: We aimed to study the clinicopathological spectrum of cranial SRCT diagnosed in our institute over a period of four years (2016-2019). MATERIAL AND METHODS: A retrospective review of medical records (2016-2019) with a morphological diagnosis of cranial SRCT was made. Both intra-axial and extra-axial tumors were included. A total of 60 cases were retrieved, and the clinical and histopathological features were studied. Special cytochemical staining and immunohistochemistry were performed, where needed. RESULTS: The mean age at presentation was 18.4 years (range, 1-60 years), with a male-to-female ratio of 2.5:1. The most common site was posterior fossa of brain (n = 28, 47%), followed by dorso-lumbar spine (n = 9, 15%). The most common type of tumor was medulloblastoma (n = 29, 48.3%), followed by Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) (n = 11, 18.3%), non-Hodgkin lymphoma (NHL) (n = 9, 15%), neuroblastoma (n = 3, 5%), and CNS embryonal tumor, NOS (n = 2, 3.3%). One case each of atypical teratoid rhabdoid tumor (ATRT), rhabdomyosarcoma, pineoblastoma, melanoma, rhabdomyosarcoma, and undifferentiated pleomorphic sarcoma was also documented. CONCLUSIONS: SRCTs have a variable age of presentation. Their incidence in CNS is low as compared to other organ systems. On light microscopy, the histopathology of these lesions is overlapping, posing a great diagnostic dilemma for the pathologist. The use of ancillary techniques like immunohistochemistry helps in arriving at the correct diagnosis. Treatment strategy and tumor prognosis also vary along the entire spectrum of SRCT, thus making exact characterization essential for proper management.

Risk of subsequent gliomas and meningiomas among 69,460 5-year survivors of childhood and adolescent cancer in Europe: the PanCareSurFup study.

BACKGROUND: Childhood cancer survivors are at risk of subsequent gliomas and meningiomas, but the risks beyond age 40 years are uncertain. We quantified these risks in the largest ever cohort. METHODS: Using data from 69,460 5-year childhood cancer survivors (diagnosed 1940-2008), across Europe, standardized incidence ratios (SIRs) and cumulative incidence were calculated. RESULTS: In total, 279 glioma and 761 meningioma were identified. CNS tumour (SIR: 16.2, 95% CI: 13.7, 19.2) and leukaemia (SIR: 11.2, 95% CI: 8.8, 14.2) survivors were at greatest risk of glioma. The SIR for CNS tumour survivors was still 4.3-fold after age 50 (95% CI: 1.9, 9.6), and for leukaemia survivors still 10.2-fold after age 40 (95% CI: 4.9, 21.4). Following cranial radiotherapy (CRT), the cumulative incidence of a glioma in CNS tumour survivors was 2.7%, 3.7% and 5.0% by ages 40, 50 and 60, respectively, whilst for leukaemia this was 1.2% and 1.7% by ages 40 and 50. The cumulative incidence of a meningioma after CRT in CNS tumour survivors doubled from 5.9% to 12.5% between ages 40 and 60, and in leukaemia survivors increased from 5.8% to 10.2% between ages 40 and 50. DISCUSSION: Clinicians following up survivors should be aware that the substantial risks of meningioma and glioma following CRT are sustained beyond age 40 and be vigilant for symptoms.

The impact of COVID-19 on 2020 monthly incidence trends of primary brain and other CNS tumors.

BACKGROUND: To mitigate disease spread, restrictions implemented in the United States surrounding the COVID-19 pandemic created an environment that led to delays in cancer diagnosis. The data needed to accurately analyze the impact of the pandemic on brain and CNS tumor incidence has not been available until now. Utilizing incidence data from the Central Brain Tumor Registry of the United States (CBTRUS) we analyzed the impact of the COVID-19 pandemic on primary brain and other CNS tumor incidence for the first year of the pandemic. METHODS: Monthly age-adjusted incidence rates and incidence trends for 2019 and 2020 were determined for age at diagnosis, sex, race, ethnicity, diagnostic confirmation, behavior, tumor histopathology, and county-level urbanization. Monthly incidence rate ratios comparing 2020 and 2019 were evaluated for the same factors. RESULTS: Overall, there was a notable decrease in incidence rates in March-May 2020 when compared to 2019. These decreases were driven by nonmalignant tumors, with a 50% incidence decrease between March 2020 and 2019. Individuals who were Black had a larger incidence decrease in early 2020 than individuals who were White. Radiographically confirmed tumors saw larger incidence decreases than histologically confirmed tumors. There were no changes in monthly incidence of glioblastoma in 2020 compared to 2019. CONCLUSIONS: These data provide evidence that disruptions in medical care, such as governmental and health care mandates, in response to the COVID-19 pandemic resulted in an overall decreased incidence of primary brain tumors in early 2020.

A system review of central nervous system tumors on children in China: epidemiology and clinical characteristics.

BACKGROUND: Central nervous system (CNS) tumors are the most common solid tumors in children and the leading cause of cancer-related death in the latter. Currently, the incidence rate exceeds that of leukemia and ranks first in the incidence of malignant tumors in children. METHODS: The epidemiological data on childhood CNS tumors were collected from the Chinese Cancer Registry Annual Report. The annual percent change (APC) of incidence and mortality-rate changes were estimated via Joinpoint regression. Due to a lack of pertinent data, we performed a system review on the clinical-pathological characteristics in Chinese publications. RESULTS: There was no significant increase in the incidence rate (APC: -0.1, 95% CI: -1.5 to 1.3), but there was a significant increase in the mortality rate (APC: 1.8, 95% CI: 0.3 to 3.4) for childhood CNS tumors. In the subgroup analysis, there were significant increases in both the incidence and mortality rates in rural areas (APC in the incidence: 6.2, 95% CI: 2.4 to 10.2; APC in mortality: 4.4, 95% CI: 0.4 to 8.4). The most common location and type of childhood CNS were, respectively, the cerebral hemisphere (25.5%, 95% CI: 21.7% to 29.4%) and astrocytomas (26.8%, 95% CI: 23.9% to 29.6%). CONCLUSIONS: The epidemiological trends, and the relevant prediction, highlighted the need to pay continual attention to childhood CNS tumors, and the clinicopathology evinced its own distinctive characteristics. Timely detection and effective treatment must be further promoted regarding childhood CNS tumors with a view to decreasing the disease burden, especially in rural areas.

Risk of central nervous system tumour incidence in a cohort of workers chronically exposed to ionising radiation.

The aim of the present study was to assess the risk of primary central nervous system (CNS) tumour incidence in a cohort of 22,377 Mayak Production Association workers chronically exposed to ionising radiation. There were 96 primary CNS tumours, including 42 cases of glioma and 44 cases of meningioma, registered during the whole follow-up period (1948-2018). The study demonstrated that the risk of primary CNS tumour incidence was associated with sex, attained age, calendar period, tall body height, age at the beginning of exposure, and facility type. There was no association found between risk of CNS tumour incidence and body mass index, smoking (males) and alcohol consumption status. The study did not find an effect of the total external gamma radiation dose absorbed in the brain on risk of CNS tumour incidence irrespective of whether an adjustment for the total external neutron dose absorbed in the brain was included or not. Excess relative risk per 1 Gy of external gamma brain dose was 0.05 (95% confidence interval (CI) -0.30; 0.70) for all CNS tumours, -0.18 (95% CI -; 0.44) for gliomas, and 0.38 (95% CI -0.32; 2.08) for meningiomas without adjustment for total neutron brain dose. There was no effect modification by sex, attained age, age at hire or facility.

The epidemiologic associations of food availability with national incidence and mortality rates of pediatric central nervous system tumors.

BACKGROUND: The epidemiology of central nervous system (CNS) tumors in pediatric patients worldwide continues to be defined. To date, there has been no evaluation of how national food availability may associate with the incidence and mortality of these tumors. Correspondingly, the aim of this study was to define if such associations exist. METHODS: The most updated incidence and mortality rates of CNS tumors in pediatric patients were abstracted by country from the Global Burden of Disease database. Data regarding food availability parameters were identified and abstracted from the Food Systems Dashboard database. Associations were tested using univariate and multivariate regression analyses. RESULTS: There were sufficient data in a total of 175 countries worldwide describing the required outcomes. Median incidence and mortality rates across these countries were 1.63 per 100,000 and 0.80 per 100,000, respectively. Higher incidence rates of pediatric CNS tumors were statistically associated with lower availability of fruit and vegetables (P = 0.02), higher average protein supply (P < 0.01), lower share of dietary energy from cereal and roots (P < 0.01), lower supply of meat (P < 0.01), lower supply of nuts and seeds (P < 0.01), lower supply of vegetable oils (P < 0.01), and higher supply of vegetables (P < 0.01). Higher mortality rates due to pediatric CNS tumors were statistically associated with lower availability of fruit and vegetables (P = 0.048), lower supply of fish (P = 0.046), and lower supply of nuts and seeds (P = 0.04). When categorizing countries based on income status, there was a decrease in significant associations found more pronounced in low-middle income countries. CONCLUSIONS: There are many novel associations between national food availability and the incidence and mortality rates of pediatric CNS tumors across the world, which may be more pronounced and divergent in low-middle income countries. A greater understanding is needed to identify what specific components of the significant parameters influence these trends and how public health efforts may best address these associations to improve overall outcomes.

Trends in pediatric CNS tumors in Armenia: a multicenter retrospective study.

PURPOSE: Central nervous system (CNS) tumors are the most common solid malignancies in children worldwide, including in Armenia. The current study aims to analyze epidemiological data, treatment, and outcomes of children and young adults (≤25 years) with CNS tumors in Armenia during the last 26 years. METHODS: We collected data from pediatric and young adult patients treated in selected sites in Armenia from 1st January 1995 to 31st December 2020. Incidence by sex, age at diagnosis, time from first complaints to diagnosis, histopathology results, treatment strategies, complications, and overall survival (OS) rates were calculated. RESULTS: The multicenter data analysis revealed 149 patients with diagnosed primary CNS tumors over 26 years. Among them, 84 (56.4%) were male. The median age at diagnosis was 7 years (range, 3 months to 25 years), and the median time from the first complaints to diagnosis was 2 months (range, 1 week to 70 months). Medulloblastomas and other embryonal tumors (47), low-grade gliomas (32), and high-grade gliomas (22) were the most commonly diagnosed malignancies. Ependymomas, craniopharyngiomas, germ cell tumors, and other malignancies were observed in 22 patients. For 26 patients, no histopathological or radiological diagnosis was available. Follow-up information was available for 98 (65.8%) patients. The 5-year OS rate for the whole study group was 67.7%. CONCLUSION: Consistent with international data, embryonal tumors, and gliomas were the most commonly diagnosed CNS malignancies in Armenia. Multimodal treatment was often not available in Armenia during the study period, especially for early cases.

Educational attainment in survivors of childhood cancer in Denmark, Finland, and Sweden.

BACKGROUND: Survivors of childhood cancer may face difficulties at school. We investigated whether childhood cancer affects attainment of upper secondary education, in a register-based cohort study from Denmark, Finland, and Sweden, where we limit bias from selection and participation. METHODS: From the national cancer registers, we identified all long-term survivors of childhood cancer diagnosed aged 0-14 years in 1971-2005 (n = 7629), compared them to matched population comparisons (n = 35,411) and siblings (n = 6114), using odds ratios (OR) and 95% confidence intervals (CI). RESULTS: Overall, 6127 survivors (80%) had attained upper secondary education by age 25, compared to 84% among comparison groups. Elevated OR for not attaining this level were mainly confined to survivors of central nervous system (CNS) tumours (ORSurv_PopComp2.05, 95%CI: 1.83-2.29). Other risk groups were survivors who had spent more time in hospital around cancer diagnosis and those who had hospital contacts in early adulthood, particularly psychiatric. Survivors of all cancer types were less likely to have attained upper secondary education without delay. CONCLUSIONS: Although survivors of childhood cancer experienced delays in their education, many had caught up by age 25. Except for survivors of CNS tumours, survivors attained upper secondary education to almost the same extent as their peers.

Menopausal hormone therapy and central nervous system tumors: Danish nested case-control study.

BACKGROUND: Use of estrogen-containing menopausal hormone therapy has been shown to influence the risk of central nervous system (CNS) tumors. However, it is unknown how the progestin-component affects the risk and whether continuous versus cyclic treatment regimens influence the risk differently. METHODS AND FINDINGS: Nested case-control studies within a nationwide cohort of Danish women followed for 19 years from 2000 to 2018. The cohort comprised 789,901 women aged 50 to 60 years during follow-up, without prior CNS tumor diagnosis, cancer, or contraindication for treatment with menopausal hormone therapy. Information on cumulative exposure to female hormonal drugs was based on filled prescriptions. Statistical analysis included educational level, use of antihistamines, and use of anti-asthma drugs as covariates. During follow-up, 1,595 women were diagnosed with meningioma and 1,167 with glioma. The median (first-third quartile) follow-up time of individuals in the full cohort was 10.8 years (5.0 years to 17.5 years). Compared to never-use, exposure to estrogen-progestin or progestin-only were both associated with increased risk of meningioma, hazard ratio (HR) 1.21; (95% confidence interval (CI) [1.06, 1.37] p = 0.005) and HR 1.28; (95% CI [1.05, 1.54] p = 0.012), respectively. Corresponding HRs for glioma were HR 1.00; (95% CI [0.86, 1.16] p = 0.982) and HR 1.20; (95% CI [0.95, 1.51] p = 0.117). Continuous estrogen-progestin exhibited higher HR of meningioma 1.34; (95% CI [1.08, 1.66] p = 0.008) than cyclic treatment 1.13; (95% CI [0.94, 1.34] p = 0.185). Previous use of estrogen-progestin 5 to 10 years prior to diagnosis yielded the strongest association with meningioma, HR 1.26; (95% CI [1.01, 1.57] p = 0.044), whereas current/recent use of progestin-only yielded the highest HRs for both meningioma 1.64; (95% CI [0.90, 2.98] p = 0.104) and glioma 1.83; (95% CI [0.98, 3.41] p = 0.057). Being an observational study, residual confounding could occur. CONCLUSIONS: Use of continuous, but not cyclic estrogen-progestin was associated with increased meningioma risk. There was no evidence of increased glioma risk with estrogen-progestin use. Use of progestin-only was associated with increased risk of meningioma and potentially glioma. Further studies are warranted to evaluate our findings and investigate the influence of long-term progestin-only regimens on CNS tumor risk.

Capturing evolving definitions of 12 select rare CNS tumors: a timely report from CBTRUS and NCI-CONNECT.

PURPOSE: Incidence, prevalence, and survival are population-based statistics describing cancer burden. The National Cancer Institute's (NCI) Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) specializes in tumor biology and outcomes for 12 rare CNS tumor types selected for their importance in adults, research interest, or potential for targeted treatment. The aim of this study was to update incidence, prevalence, and survival statistics for these tumors. METHODS: The Central Brain Tumor Registry of the United States (CBTRUS) database, a combined dataset of Centers for Disease Control and Prevention's (CDC) National Program of Cancer Registries (NPCR) and NCI's Surveillance, Epidemiology and End Results (SEER) data, was used to calculate average annual age-adjusted incidence rates (AAAIR) per 100,000 population overall and by sex, race-ethnicity, and age for diagnosis years 2008-2019. Incidence time trends were calculated for diagnosis years 2004-2019. NPCR data were used to calculate relative survival rates. Point prevalence on December 31, 2019 was estimated using annual age-specific incidence and survival. RESULTS: AAAIR was 1.47 per 100,000 for these tumors combined, with highest incidence in ependymomas (AAAIR = 0.41/100,000). Most tumor types were more common in males, adults (ages 40 + years) or children (ages < 15 years), and non-Hispanic White individuals. Ependymomas were the most prevalent tumor type (19,320 cases) followed by oligodendrogliomas (14,900 cases). Ependymomas had the highest five-year survival (90.6%) and primary CNS sarcomas the lowest (7.7%). CONCLUSIONS: These data provide means to measure the impact of clinical care and evaluate new therapies and the evolving histopathology definitions in rare CNS tumor types.

Central Nervous System Lymphoma.

Central nervous system lymphoma (CNSL) is a rare and aggressive malignancy that primarily affects the brain, spinal cord, and meninges. This article provides a comprehensive overview of the current understanding of CNSL encompassing its epidemiology, pathophysiology, clinical presentation, diagnosis, treatment modalities, and prognosis. Although the main focus is on primary CNS lymphoma (PCNSL), ocular lymphoma, primary leptomeningeal lymphoma, and secondary CNS lymphoma are also discussed. The pathobiology of CNSL involves the infiltration of malignant lymphocytes within the CNS parenchyma or leptomeninges. Various risk factors and immunological mechanisms contribute to its development, including immunodeficiency states, chronic inflammation, and genomic alterations. Accurate diagnosis is crucial for appropriate management, given the heterogeneous clinical presentation. The neuroimaging, systemic imaging, and other modalities for diagnosis and evaluation for extent of disease involvement will be discussed. Additionally, the importance of histopathological examination, cerebrospinal fluid (CSF) analysis, and molecular testing in confirming the diagnosis and guiding treatment decisions are highlighted. The treatment landscape for CNSL has evolved significantly. Therapeutic approaches encompass a multimodal strategy combining high-dose methotrexate-based chemotherapy, consolidation with whole-brain radiation therapy, and high-dose chemotherapy with stem cell rescue. Recent advancements in targeted therapies and immunomodulatory agents offer promising avenues for future treatment options. We review the clinical outcomes and prognostic factors influencing the survival of CNSL patients, including age, performance status, disease stage, and genetic abnormalities.

CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2016-2020.

The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control and Prevention and the National Cancer Institute, is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in the United States (US) and represents the entire US population. This report contains the most up-to-date population-based data on primary brain tumors available and supersedes all previous CBTRUS reports in terms of completeness and accuracy. All rates are age-adjusted using the 2000 US standard population and presented per 100,000 population. The average annual age-adjusted incidence rate (AAAIR) of all malignant and non-malignant brain and other CNS tumors was 24.83 per 100,000 population (malignant AAAIR=6.94 and non-malignant AAAIR=17.88). This overall rate was higher in females compared to males (27.85 versus 21.62 per 100,000) and non-Hispanic persons compared to Hispanic persons (25.24 versus 22.61 per 100,000). Gliomas accounted for 26.3% of all tumors. The most commonly occurring malignant brain and other CNS histopathology was glioblastoma (14.2% of all tumors and 50.9% of all malignant tumors), and the most common predominantly non-malignant histopathology was meningioma (40.8% of all tumors and 56.2% of all non-malignant tumors). Glioblastomas were more common in males, and meningiomas were more common in females. In children and adolescents (ages 0-19 years), the incidence rate of all primary brain and other CNS tumors was 6.13 per 100,000 population. There were 86,030 deaths attributed to malignant brain and other CNS tumors between 2016 and 2020. This represents an average annual mortality rate of 4.42 per 100,000 population and an average of 17,206 deaths per year. The five-year relative survival rate following diagnosis of a malignant brain and other CNS tumor was 35.7%, for a non-malignant brain and other CNS tumor the five-year relative survival rate was 91.8%.

Pesticide exposure and risk of Central Nervous System tumors in children: a systematic review with meta-analysis. / Exposição a agrotóxicos e o risco de tumores do Sistema Nervoso Central em crianças: revisão sistemática com metanálise.

Central Nervous System (CNS) tumors represent more than half of all childhood malignant neoplasms. The aim of this study was to determine the relationship between environmental exposure to pesticides and the development of CNS tumors in children. We conducted a systematic review of the literature in the PubMed/MEDILINE, Embase, Web of Science, Scopus, and CINAHL databases. The inclusion criteria were cohort and case-control studies investigating the association between exposure to pesticides and CNS tumors (all histological types included in group III of the WHO Classification of Childhood Cancer) in children aged 0-14 years. The meta-analysis was performed using a random effects model and the Mantel-Haenszel method. Strength of association was measured using odds ratios (OR). The review was registered in the International Prospective Register of Systematic Reviews (PROSPERO) under identification number CRD42021209354. The search identified 1,158 studies, 14 of which were included in the review. There was evidence of an association between the development of astrocytomas and exposure to all classes of pesticides (OR 1.50; 95%CI 1.15-1.96; p=0.03). The synthesis of the evidence pointed to a relationship between exposure to pesticides and some histological types of CNS tumors in childhood.

Os tumores do Sistema Nervoso Central (SNC) representam mais da metade das neoplasias infantis malignas que acometem crianças. Objetivou-se analisar o risco de exposição a agrotóxicos relacionado com o desenvolvimento de tumores do SNC em crianças. Realizou-se uma revisão sistemática da literatura nas bases de dados PubMed/MEDILINE, Embase, Web of Science, Scopus e CINAHL. Foram incluídos estudos de coorte e caso-controle sobre o desenvolvimento de tumores do SNC (todos os tipos histológicos do grupo III Classificação de Câncer Infantil) decorrentes da exposição a agrotóxicos em crianças de 0-14 anos. Na metanálise utilizou-se o modelo de efeito aleatório e o método estatístico de Mantel-Haenszel. A Razão de Chances (RC) ou Odds Ratio (OR) foi a medida de associação aplicada. A revisão foi registrada no International Prospective Register of Systematic Reviews (PROSPERO) sob o número CRD42021209354. A busca identificou 1.158 estudos, dos quais 14 compuseram a revisão. Verificou-se evidência de associação entre o desenvolvimento de astrocitomas e a exposição a todas as classes de pesticidas (OR 1,50; IC95% 1,15-1,96; p=0,03). A síntese dos resultados apontou para uma relação da exposição aos agrotóxicos com o desfecho de alguns tipos histológicos de tumores do SNC na infância.

Global, Regional, and National Incidence and Mortality Trends in Pediatric Central Nervous System Tumors over the Past 2 Decades.

BACKGROUND: Pediatric tumors of the brain and central nervous system (CNS) are a worldwide issue with variances in epidemiology. How exactly incidence and mortality rates have changed over time has not been summarized. Correspondingly, the aim of this study was to quantitively define the global, regional, and national epidemiological trends of these tumors. METHODS: A retrospective review of data from the Global Burden of Disease Study 2019 Database was performed incorporating data from 1999 to 2019. Global, regional, and national outcomes for pediatric CNS tumors were collected for incidence and mortality at a worldwide level, as well as across 7 continental regions, and then 204 countries and territories. RESULTS: Globally, the latest incidence of pediatric CNS tumors was 47,600 (uncertainty interval, 36,500-55,200) at a rate of 1.8 (1.4-2.2) per 100,000, with 23,500 (18,000-27,500) deaths due to these tumors at a rate of 0.9 (0.7-1.1) per 100,000 population. Both rates per 100,000 have decreased over the past 2 decades. With respect to regions, East Asia and Pacific had the highest incidence and mortality cases overall, but in terms of rate per 100,000, North America and Latin America and Caribbean had the highest values, respectively. There were 3/7 (43%) and 6/7 (86%) regions with decreasing incidence and mortality rates per 100,000 over the past 2 decades. China, India, and Pakistan were the 3 countries with both the highest incidence and mortality cases overall; however, San Marino, Denmark, and Norway had the highest incidence rates per 100,000, and Albania, Armenia, and Haiti had the highest mortality rates per 100,000. In the past 2 decades, 79/204 (39%) and 120/204 (59%) countries observed decreasing incidence and mortality rates per 100,000, respectively. CONCLUSIONS: Pediatric CNS tumors remains a worldwide issue, with there being multiple regions and countries worldwide that continue to experience uptrending incidence and mortality rates per 100,000. For both incidence and mortality, there is a clear discordance between regions and countries that report the highest number of cases versus the highest rate of cases per 100,000. Future research efforts are needed to identify positive intervention measures that respect the epidemiology of these tumors at global, regional, and national levels.

Central Nervous System Relapse in T and NK cell Lymphomas.

PURPOSE OF REVIEW: T and NK cell lymphomas are relatively rare and heterogeneous forms of non-Hodgkin lymphoma that are associated with high rates of mortality. Central nervous system relapse carries significant morbidity, though management is largely extrapolated from literature in B cell neoplasms. As such, outcomes for central nervous system involvement in T/NK cell lymphomas are dismal with no standard of care. In this review, we discuss the epidemiology of central nervous system relapse in T/NK cell lymphomas and critically analyze available literature regarding prophylaxis and treatment. RECENT FINDINGS: Retrospective studies of central nervous system involvement in T/NK cell lymphomas have been limited by small sample sizes and heterogeneity of subtypes, though sites of extranodal involvement and disease subtypes are consistently reported as risk factors. Compelling evidence for the use of central nervous system-directed prophylactic therapy has not yet been established, though recent reports of central nervous system activity with novel agents may suggest promising therapeutic options. The overall rarity of T and NK cell lymphomas has precluded adequate study of prophylaxis and treatment of central nervous system relapse. Collaborative efforts are needed to better define strategies to address CNS disease in T/NK cell lymphomas. These should involve the use of targeted agents, which may hold an advantage over traditional cytotoxic drugs.

Classification of Central Nervous System Tumors Histologically Diagnosed in a Single Center of China 2003-2019.

OBJECTIVE: The classification of central nervous system (CNS) tumors has changed greatly. The Central Brain Tumor Registry of the United States (CBTRUS) and other institutions have analyzed the incidence rate and characteristics of primary CNS tumors. However, there are limited studies analyzing the incidence rate and characteristics of CNS tumors in China. To better understand CNS tumors in China, we summarized all primary CNS tumors diagnosed pathologically in a single center from 2003 to 2019. METHODS: All patients with primary CNS tumors who underwent neurosurgery at our hospital from January 2003 to December 2019 were included in this study. The data were collected from the hospital information system, including diagnosis time, age, gender, anatomic sites, and pathologic results. RESULTS: A total of 17,226 cases of primary CNS tumors were retrospectively analyzed in this study. Among all cases, the major tumor types included meningiomas, tumors of neuroepithelial tissue, and pituitary adenomas. Most tumors of neuroepithelial tissue were glioblastoma and astrocytoma. Most tumors of neuroepithelial tissue were located in the frontal lobe. However, grade 4 tumors of neuroepithelial tissue were more common in the temporal lobe. The median age of all patients was 46 years. The incidence of CNS tumors was higher in women than in men. CONCLUSIONS: Based on this data set, we analyzed various parameters of CNS tumors and found that grade 4 tumors of neuroepithelial tissue were more common in the temporal lobe, which were rarely reported in previous articles.

[Secondary tumors following radiation therapy and chemotherapy : Incidence of cavernous hemangiomas]. / Zweittumoren nach Bestrahlung und Chemotherapie : Auftreten von Kavernomen.

PROBLEM: There is a risk of developing secondary central nervous system (CNS) tumors after cranial radiation therapy. Meningiomas and pituitary tumors are also increasingly treated with radiation therapy, which means that the risk of secondary tumors from radiation in children and adults must be communicated. METHODS: Studies in children show that radiation causes a 7- to 10-fold increase in subsequent CNS tumors with a cumulative incidence over 20 years ranging from 1.03 to 28.9. The latency period for the occurrence of secondary tumors ranged from 5.5 to 30 years, with gliomas developing after 5-10 years and meningiomas around 15 years after irradiation. The latency period for secondary CNS tumors in adults ranged from 5 to 34 years. CONCLUSION: After radiation treatment, tumors can rarely occur as secondary sequelae, mostly meningiomas and gliomas, but also cavernomas. The treatment and long-term results of radiation-induced CNS tumors showed no worse results than primary CNS tumors over the course of time.

Counts, incidence rates, and trends of pediatric cancer in the United States, 2003-2019.

BACKGROUND: Cancer is a leading cause of death by disease among children and adolescents in the United States. This study updates cancer incidence rates and trends using the most recent and comprehensive US cancer registry data available. METHODS: We used data from US Cancer Statistics to evaluate counts, age-adjusted incidence rates, and trends among children and adolescents younger than 20 years of age diagnosed with malignant tumors between 2003 and 2019. We calculated the average annual percent change (APC) and APC using joinpoint regression. Rates and trends were stratified by demographic and geographic characteristics and by cancer type. RESULTS: With 248 749 cases reported between 2003 and 2019, the overall cancer incidence rate was 178.3 per 1 million; incidence rates were highest for leukemia (46.6), central nervous system neoplasms (30.8), and lymphoma (27.3). Rates were highest for males, children 0 to 4 years of age, Non-Hispanic White children and adolescents, those in the Northeast census region, the top 25% of counties by economic status, and metropolitan counties with a population of 1 million people or more. Although the overall incidence rate of pediatric cancer increased 0.5% per year on average between 2003 and 2019, the rate increased between 2003 and 2016 (APC = 1.1%), and then decreased between 2016 and 2019 (APC = -2.1%). Between 2003 and 2019, rates of leukemia, lymphoma, hepatic tumors, bone tumors, and thyroid carcinomas increased, while melanoma rates decreased. Rates of central nervous system neoplasms increased until 2017, and then decreased. Rates of other cancer types remained stable. CONCLUSIONS: Incidence of pediatric cancer increased overall, although increases were limited to certain cancer types. These findings may guide future public health and research priorities.

Central nervous system tumours among users of vaginal oestradiol tablets: A nationwide population-based study.

BACKGROUND AND PURPOSE: It is currently unknown whether vaginal oestradiol is associated with development of meningioma and glioma. The aim of this study was to examine associations between cumulative use and treatment intensity of vaginally administered oestradiol tablets and incidence of meningioma and glioma in a nationwide, population-based study. METHODS: We conducted a nested case-control study within a nationwide cohort of Danish women followed from 2000 to 2018. The cohort consisted of 590,676 women aged 50-60 years at study start, without prior cancer diagnosis or use of systemic hormone therapy. Information on cumulative dose, duration, and intensity of vaginal oestradiol tablet use was assessed from filled prescriptions. Conditional logistic regression provided adjusted hazard ratios (HRs) for the association between vaginal oestradiol use and diagnosis of meningioma or glioma. RESULTS: We identified 1108 women with meningioma and 835 with glioma. Of these, 19.8% and 14.0% used vaginal oestradiol tablets, respectively. The HRs in those with ever-use of vaginal oestradiol tablets was 1.14 (95% confidence interval [CI] 0.97-1.34) for meningioma and 0.90 (95% CI 0.73-1.11) for glioma. The corresponding HRs for new users exclusively were 1.18 (95% CI 0.99-1.40) for meningioma and 0.89 (95% CI 0.71-1.13) for glioma. Intensity of vaginal oestradiol tablet use according to duration and user status yielded slightly elevated HRs for meningioma without an apparent dose-response pattern, while the HRs for glioma were generally below unity. Among new users, the HR with high intensity of current or recent vaginal oestradiol tablet use for 2+ years was 1.66 (95% CI 1.09-2.55) for meningioma and 0.77 (95% CI 0.41-1.44) for glioma. CONCLUSION: Use of vaginal oestradiol tablets was associated with a slightly increased incidence of meningioma but not of glioma. Owing to the observational nature of the study, residual bias cannot be ruled out.