Article
[Infant rhabdoid tumors: a diagnostic emergency].
Archives de pediatrie : organe officiel de la Societe francaise de pediatrie - 1 Nov 2014
Marty L, Cuinet A, Roujeau T, Prodhomme O, Saumet L, Coupier I, Sirvent N
Abstract excerpt
Rhabdoid tumors are a heterogeneous family of aggressive tumors affecting young children. Their grouping within a single entity is recent, following the discovery of a bi-allelic inactivation of the hSNF5/INI1 tumor suppressor gene in tumoral cells. This bi-allelic inactivation of the hSNF5/INI1 gene found at the constitutional level in up to one-third of cases has led to the identification of a predisposal...
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