Imaging characterization of paediatric tumours with the neurotrophic tyrosine receptor kinase fusion transcript.
Br J Radiol
; 97(1156): 734-743, 2024 Mar 28.
Article
in En
| MEDLINE
| ID: mdl-38327010
ABSTRACT
OBJECTIVES:
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. CASE SERIES 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.CONCLUSION:
Paediatric NTRK-FT tumours mainly occur in infants with very variable histotypes and locations. Rich and irregular intra-tumoural vascularization are recurrent findings. ADVANCES IN KNOWLEDGE 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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Receptors, Amino Acid
/
Nephroma, Mesoblastic
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Fibrosarcoma
/
Kidney Neoplasms
Type of study:
Prognostic_studies
Limits:
Child
/
Humans
/
Infant
Language:
En
Journal:
Br J Radiol
Year:
2024
Document type:
Article
Affiliation country:
Francia
Country of publication:
Reino Unido