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síndrome de Li-Fraumeni-
like
e câncer pediátrico. Todos
os artigos publicados entre 1990 e 2014 usando essas pa-
lavras-chave foram recuperados e revisados.
Conclusão:
apesar de LFS/LFL ser considerada uma doen-
ça rara, ela parece ser mais frequente em certas regiões.
Reconhecer os critérios e condutas para identificação de
pacientes em risco para LFS/LFL é fundamental para o
manejo adequado dos pacientes com câncer hereditários
e suas famílias. Devido à complexidade dessas síndromes,
a abordagem multidisciplinar deve ser realizada. Pedia-
tras e oncologistas pediátricos em áreas com alta preva-
lência de síndromes hereditárias de câncer têm um papel
central no reconhecimento e encaminhamento adequa-
do dos pacientes e famílias para programas de avaliação
do risco de câncer genético e de gestão.
Palavras-chave:
síndrome de Li-Fraumeni, neoplasias, ge-
nes p53, aconselhamento genético,
TP53
.
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