%0 Journal Article
%T Imaging characterization of paediatric tumours with the neurotrophic tyrosine receptor kinase fusion transcript.
%A Hermann AL
%A Lemelle L
%A Pierron G
%A Gauthier A
%A Nicolas N
%A Cardoen L
%A Moalla S
%A Petit P
%A Morel B
%A Ducou Le Pointe H
%A Hassani A
%A Fréneaux P
%A Guillemot D
%A Carton M
%A Corradini N
%A Rome A
%A Castex MP
%A Defachelles AS
%A Schleiermacher G
%A Berlanga P
%A Delattre O
%A Orbach D
%A Brisse HJ
%J Br J Radiol
%V 97
%N 1156
%D 2024 Mar 28
%M 38327010
%F 3.629
%R 10.1093/bjr/tqae016
%X <B>OBJECTIVE: </B>The neurotrophic tyrosine receptor kinase (NTRK) fusion transcript (FT) is a major genetic landmark of infantile fibrosarcoma (IFS) and cellular congenital mesoblastic nephroma (cCMN) but is also described in other tumours. The recent availability of NTRK-targeted drugs enhances the need for better identification. We aimed to describe the anatomic locations and imaging features of tumours with NTRK-FT in children.<BR><B>METHODS: </B>Imaging characteristics of NTRK-FT tumours of 41 children (median age: 4 months; 63% <1 year old; range: 0-188) managed between 2001 and 2019 were retrospectively analysed. The tumours were located in the soft tissues (n = 24, including 19 IFS), kidneys (n = 9, including 8 cCMN), central nervous system (CNS) (n = 5), lung (n = 2), and bone (n = 1). The tumours were frequently deep-located (93%) and heterogeneous (71%) with necrotic (53%) or haemorrhagic components (29%). Although inconstant, enlarged intratumoural vessels were a recurrent finding (70%) with an irregular distribution (63%) in the most frequent anatomical locations.<BR><B>CONCLUSIONS: </B>Paediatric NTRK-FT tumours mainly occur in infants with very variable histotypes and locations. Rich and irregular intra-tumoural vascularization are recurrent findings.<BR><B>CONCLUSIONS: </B>Apart from IFS of soft tissues and cCMN of the kidneys, others NTRK-FT tumours locations have to be known, as CNS tumours. Better knowledge of the imaging characteristics may help guide the pathological and biological identification.