{Reference Type}: Journal Article
{Title}: Imaging characterization of paediatric tumours with the neurotrophic tyrosine receptor kinase fusion transcript.
{Author}: Hermann AL;Lemelle L;Pierron G;Gauthier A;Nicolas N;Cardoen L;Moalla S;Petit P;Morel B;Ducou Le Pointe H;Hassani A;Fréneaux P;Guillemot D;Carton M;Corradini N;Rome A;Castex MP;Defachelles AS;Schleiermacher G;Berlanga P;Delattre O;Orbach D;Brisse HJ;
{Journal}: Br J Radiol
{Volume}: 97
{Issue}: 1156
{Year}: 2024 Mar 28
{Factor}: 3.629
{DOI}: 10.1093/bjr/tqae016
{Abstract}: <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.