What question did this study set out to answer?

To explore the molecular alterations in PRRX1-rearranged mesenchymal tumours and their classification.

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February 22, 2026

The new kid on the block: a case of PRRX1-rearranged mesenchymal tumour

Key Points

To explore the molecular alterations in PRRX1-rearranged mesenchymal tumours and their classification.
Utilized next-generation sequencing to identify molecular alterations in soft tissue tumours.
Characterized PRRX1 rearrangements and relevant fusions.
Evaluated existing literature on biological behavior and clinical course.
Identified PRRX1::NCOA1 and PRRX1::NCOA2 fusions in mesenchymal tumours.
Classified these tumours as a rare subset with distinct characteristics.
Noted an indolent clinical course in early reports.

Abstract

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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