Next-generation sequencing reveals rare PRRX1-rearranged mesenchymal tumours, indicating distinct clinical behavior in soft tissue cancers.
Next-generation sequencing (NGS) is increasingly uncovering disease-defining molecular alterations that refine the classification of fibroblastic and myofibroblastic soft tissue tumours — a diverse and diagnostically challenging group. 1 Among these, paired related homeobox 1 (PRRX1) rearranged mesenchymal tumours represent an exceptionally rare and recently characterised subset, typically harbouring PRRX1::NCOA1 or PRRX1::NCOA2 fusions. 1,2 Although their biological behaviour remains incompletely understood, early reports suggest an indolent clinical course. 2
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Fraser et al. (2026) studied this question.
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