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Rare myoepithelial tumor with EWSR1-PBX3 fusion in rib and soft tissue

Microscopic view of tissue stained with purple and pink dyes showing elongated cells and nuclei in a fibrous or muscular tissue sample under high magnification.
Research area:MedicineBone Tumor Diagnosis and TreatmentsSoft tissue

What the study found

A rare myoepithelial tumor was found in the rib and the surrounding soft tissue, and testing showed an EWSR1-PBX3 fusion and rearrangement.

Why the authors say this matters

The authors say the presence of EWSR1-PBX3 fusion and rearrangement in myoepithelial tumors of bone and soft tissue is diagnostically significant. They conclude that molecular testing is important when distinguishing this tumor from synovial sarcoma and other closely related entities.

What the researchers tested

The researchers described a 52-year-old man with worsening low back pain. Imaging showed osteolytic destruction of the left twelfth rib with a soft-tissue mass, histopathology showed spindle and oval tumor cells with mild atypia, immunohistochemistry was positive for S-100, EMA, SMA, and calponin but negative for cytokeratin, and next-generation sequencing was used to test for gene fusion and rearrangement.

What worked and what didn't

Next-generation sequencing verified the presence of the EWSR1-PBX3 fusion and rearrangement. After surgical resection and postoperative radiotherapy, the patient remained disease-free for 2 years.

What to keep in mind

This is a single case report, so the abstract does not provide broader estimates or compare treatments across groups. The available summary does not describe additional limitations beyond the rarity of the case.

Key points

  • The case involved a myoepithelial tumor in the left twelfth rib and surrounding soft tissue.
  • Next-generation sequencing verified an EWSR1-PBX3 fusion and rearrangement.
  • The tumor was positive for S-100, EMA, SMA, and calponin, and negative for cytokeratin.
  • After surgery and postoperative radiotherapy, the patient was disease-free for 2 years.
  • The authors say molecular testing is important for distinguishing this tumor from synovial sarcoma and related entities.

Disclosure

Research title:
Rare myoepithelial tumor with EWSR1-PBX3 fusion in rib and soft tissue
Authors:
Junting Yang, Jie Liu, Tingting Zhao, Shuwei Yu, Boxiukang Zhang, Yangcan Li, Hongling Wang
Institutions:
Yuhuangding Hospital, Xizang Minzu University, The First Hospital of Changsha
Publication date:
2026-02-27
OpenAlex record:
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AI provenance: This post was generated by OpenAI. The original authors did not write or review this post.