Age–incidence patterns of primary CNS tumors in children, adolescents, and adults in England

Arora, Ramandeep Singh; Alston, Richard; Eden, Tim; Estlin, Edward J.; Moran, Anthony; Birch, Jillian M

doi:10.1215/15228517-2008-097

Cited by 138 publications

(90 citation statements)

References 33 publications

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“…Comparison of the incidence rates of the most frequent pediatric CNS tumors across Europe, England, and the United States shows highly similar rates in Austria, thereby confirming a high degree of case ascertainment and the validity of our findings. 1,39,42 Half of the atypical teratoid/rhabdoid tumors in our series were initially not recognized. Such misdiagnoses tended to be more common in the first years after the definition of atypical teratoid/rhabdoid tumor as an entity and before the introduction of the anti-SMARCB1 antibody as diagnostic tool.…”

Section: Discussionmentioning

confidence: 83%

Incidence of atypical teratoid/rhabdoid tumors in children

Wöehrer

Slavc

Waldhoer

et al. 2010

Cancer

126

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on behalf of the Austrian Brain Tumor Registry BACKGROUND: Atypical teratoid/rhabdoid tumors are highly malignant embryonal central nervous system (CNS) tumors that were defined as an entity in 1996. As compared with other malignant CNS tumors, their biological behavior is particularly aggressive, but patients may benefit from an intensified treatment. Atypical teratoid/rhabdoid tumors display a complex histomorphology, which renders them prone to misdiagnosis. They occur predominantly in young children, with an estimated prevalence of 1% to 2% among all pediatric CNS tumors. However, populationbased data on the incidence of these tumors are not yet available. METHODS: A nation-wide survey of malignant high-grade CNS tumors (World Health Organization grade III/IV), diagnosed in children (aged birth to 14 years) from 1996 to 2006 was conducted by the Austrian Brain Tumor Registry. A central histopathology review was performed including the assessment of SMARCB1 (INI1) protein status. RESULTS: A total of 311 newly diagnosed, malignant CNS tumors were included. Atypical teratoid/rhabdoid tumors constituted the sixth most common entity (6.1%), referring to an age-standardized incidence rate of 1.38 per 1,000,000 person-years in children. Peak incidence was found in the birth to 2 years age group, where they were as common as CNS primitive neuroectodermal tumors and medulloblastomas. A total of 47.4% of atypical teratoid/rhabdoid tumors were initially diagnosed, whereas 52.6% were retrospectively detected by the central review. The 5-year survival of atypical teratoid/rhabdoid tumor patients was 39.5%, with 66.7% in the correctly diagnosed group versus 15.0% in the not recognized group (P ¼ .0469). CONCLUSIONS: Clinicians and pathologists should be aware of the high incidence of atypical teratoid/rhabdoid tumors in young children to optimize diagnostic and therapeutic management of patients with these tumors. Cancer 2010;116:5725-32. V C 2010 American Cancer Society.KEYWORDS: atypical teratoid/rhabdoid tumor, central nervous system neoplasm, childhood, epidemiology, population-based incidence, survival.Central nervous system (CNS) tumors represent >20% of all childhood malignancies (0-14 years) in developed countries 1,2 and constitute the most common cause of cancer-related death in this age group.

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Section: Discussionmentioning

confidence: 83%

Incidence of atypical teratoid/rhabdoid tumors in children

Wöehrer

Slavc

Waldhoer

et al. 2010

Cancer

126

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“…The average annual age-adjusted IR of GBM is variable, ranging from 0.59 per 100,000 persons to 3.69 per 100,000 persons (11,(13)(14)(15)(16)(17), and is the highest among malignant primary brain tumors (Table 1).…”

Section: Incidence Of Glioblastomamentioning

confidence: 99%

Epidemiology and Outcome of Glioblastoma

2017

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Glioblastoma (GBM) is the most aggressive malignant primary brain tumor. With an incidence rate of 3.19 per 100,000 persons in the United States and a median age of 64 years, it is uncommon in children. The incidence is 1.6 times higher in males compared to females and 2.0 times higher in Caucasians compared to Africans and Afro-Americans, with lower incidence in Asians and American Indians. GBM is commonly located in the supratentorial region (frontal, temporal, parietal, and occipital lobes) and is rarely located in cerebellum. Genetic and environmental factors have been investigated in GBM. Risk factors include prior radiotherapy, decreased susceptibility to allergy, immune factors and immune genes, as well as some single nucleotide polymorphisms detected by genomic analysis. Use of anti-inflammatory medication has been found to be protective against GBM. Survival from GBM is poor; only few patients survive 2.5 years and less than 5% of patients survive 5 years following diagnosis. Survival rates for patients with GBM have shown no notable improvement in population statistics in the last three decades. Molecular epidemiology integrates molecular technology into epidemiological studies and outcomes. The future of the epidemiology of Epidemiology of Glioblastoma 144GBM will depend on multicenter studies generating large clinical data sets of genomic data potentially leading to further understanding of the roles of genes and environment in the development of this devastating disease.

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“…In children astrocytic tumours were common followed by medulloblastoma and ependymoma similar to several other studies. 15,16 Metastatic tumours account for 9.2% of all cases being common in elderly individuals above 50 years of age. The most common metastatic tumour was from lung similar to a study conducted by Sloan et al 17 and Saha et al 18 followed by metastasis from breast.…”

Section: Cns Tumourmentioning

confidence: 99%