Marialetizia Motta, Sabina Barresi, Simone Pizzi, Delfina Bifano, Jennifer Lopez Marti, Marta Garrido-Pontnou, Elisabetta Flex, Alessandro Bruselles, Isabella Giovannoni, Giovannina Rotundo, Alessandra Fragale, Valentina Tirelli, Silvia Vallese, Andrea Ciolfi, Gianni Bisogno, Rita Alaggio, Marco Tartaglia
Infantile fibrosarcomas (IFS) and congenital mesoblastic nephroma (CMN) are rare myofibroblastic tumors of infancy and early childhood commonly harboring the ETV6::NTRK3 gene fusion. IFS/CMN are considered as tumors with an 'intermediate prognosis' as they are locally aggressive, but rarely metastasize, and generally have a favorable outcome. A fraction of IFS/CMN-related neoplasms are negative for the ETV6::NTRK3 gene rearrangement and are characterized by other chimeric proteins promoting MAPK signaling upregulation...
April 17, 2024: Journal of Pathology