Familial risks and incidence rates for childhood nervous system tumors in Sweden

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11140%2F25%3A10502230" target="_blank" >RIV/00216208:11140/25:10502230 - isvavai.cz</a>

Výsledek na webu

<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=YlBzXXNOsV" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=YlBzXXNOsV</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1016/j.ejcped.2025.100310" target="_blank" >10.1016/j.ejcped.2025.100310</a>

Jazyk výsledku

angličtina

Název v původním jazyce

Familial risks and incidence rates for childhood nervous system tumors in Sweden

Popis výsledku v původním jazyce

Background: Childhood (&lt; 20 years) nervous system tumors manifest in some rare cancer syndromes but how commonly they present with familial clustering between various histological types is not well known at a nation-wide level. Our aim is to enhance understanding of familial risks in childhood nervous system cancers. Methods: We used the Swedish population and cancer data from years 1958-2021 to address familial risks among nervous system cancers when the case was diagnosed before age 20 years but the proband could be diagnosed at any age. Adjusted familial (relative) risks were expressed as standardized incidence ratios (SIRs). Results: Familial childhood cancer cases amounted to 123 in the brain, 15 in the spinal cord and 9 in peripheral nerves. Familial risk for concordant brain cancer was about 2.0 irrespective of proband. Concordant risk of spinal cord cancer was high when mothers (17.92) or siblings were probands (24.91). High familial risk of 34.53 was recorded for hemangioblastoma, and moderate high risks were observed also for schwannoma (4.07), ependymoblastoma (3.48) and female ganglioneuroma (7.72) whereas astrocytoma risks were at the level of common cancers at other sites (1.7-2.0). Hemangioblastoma families appeared not to be related to von Hippel-Lindau syndrome because of lack of pathognomonic signs other than hemangioblastoma in the families. Conclusion: In spite of the low number of childhood nervous system tumors, we observed high familial risks for, probably novel, syndromic hemangioblastoma, and schwannomas for which concordant clusters were found particularly in the spinal cord. Inquiring about a detailed family history at diagnosis of a nervous system cancer in childhood may reveal a syndromic disease due to a constitutional variant amenable to treatment.

Název v anglickém jazyce

Familial risks and incidence rates for childhood nervous system tumors in Sweden

Popis výsledku anglicky

Background: Childhood (&lt; 20 years) nervous system tumors manifest in some rare cancer syndromes but how commonly they present with familial clustering between various histological types is not well known at a nation-wide level. Our aim is to enhance understanding of familial risks in childhood nervous system cancers. Methods: We used the Swedish population and cancer data from years 1958-2021 to address familial risks among nervous system cancers when the case was diagnosed before age 20 years but the proband could be diagnosed at any age. Adjusted familial (relative) risks were expressed as standardized incidence ratios (SIRs). Results: Familial childhood cancer cases amounted to 123 in the brain, 15 in the spinal cord and 9 in peripheral nerves. Familial risk for concordant brain cancer was about 2.0 irrespective of proband. Concordant risk of spinal cord cancer was high when mothers (17.92) or siblings were probands (24.91). High familial risk of 34.53 was recorded for hemangioblastoma, and moderate high risks were observed also for schwannoma (4.07), ependymoblastoma (3.48) and female ganglioneuroma (7.72) whereas astrocytoma risks were at the level of common cancers at other sites (1.7-2.0). Hemangioblastoma families appeared not to be related to von Hippel-Lindau syndrome because of lack of pathognomonic signs other than hemangioblastoma in the families. Conclusion: In spite of the low number of childhood nervous system tumors, we observed high familial risks for, probably novel, syndromic hemangioblastoma, and schwannomas for which concordant clusters were found particularly in the spinal cord. Inquiring about a detailed family history at diagnosis of a nervous system cancer in childhood may reveal a syndromic disease due to a constitutional variant amenable to treatment.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30204 - Oncology

Projekt

<a href="/cs/project/EH22_008%2F0004644" target="_blank" >EH22_008/0004644: Záchrana životů prostřednictvím výzkumu v oblasti včasné detekce a prevence rakoviny: Molekulární, genomické a sociální faktory</a><br>

Návaznosti

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)<br>I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2025

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

EJC Paediatric Oncology

ISSN

2772-610X

e-ISSN

2772-610X

Svazek periodika

6

Číslo periodika v rámci svazku

December

Stát vydavatele periodika

GB - Spojené království Velké Británie a Severního Irska

Počet stran výsledku

6

Strana od-do

100310

Kód UT WoS článku

001591572900001

EID výsledku v databázi Scopus

2-s2.0-105010344776