CASE REPORT article
Front. Oncol.
Sec. Cancer Genetics
Myoepithelial Tumor with EWSR1-PBX3 Fusion and Rearrangement in Rib and Soft Tissue: A Rare Case Report
Provisionally accepted
- 1Department of Clinical Laboratory, Qingdao University Medical College Affiliated Yantai Yuhuangding Hospital, Yantai, China
- 2School of Medicine, Xizang Minzu Uinversity, Xianyang, China
- 3Department of Radiology, Qingdao University Medical College Affiliated Yantai Yuhuangding Hospital, Yantai, China
- 4Department of Pathology, The First Hospital of Changsha, Changsha, China
- 5Department of Material Supply, Qingdao University Medical College Affiliated Yantai Yuhuangding Hospital, Yantai, China
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Myoepithelial tumor (MET) is rare and heterogeneous. We present a case of a MET occurring in the rib and the soft tissue surrounding it. Further, the tumor has been reported to contain both EWSR1-PBX3 fusion and rearrangement. A 52-year-old male presented with worsening low back pain. Imaging showed osteolytic destruction of the left twelfth rib accompanied by a soft-tissue mass. Histopathology revealed spindle and oval tumor cells with mild atypia. The entity was positive for S-100, EMA, SMA and calponin by immunohistochemistry but negative for cytokeratin. Testing through next-generation sequencing (NGS) verified the presence of the EWSR1-PBX3 fusion and rearrangement. After surgical resection and post-operative radiotherapy, the patient remained disease-free for 2 years. The presence of EWSR1-PBX3 fusion and rearrangement in METs of bone and soft tissue is diagnostically significant. It is critical that molecular testing should be performed by clinicians, notably when distinguishing it from synovial sarcoma and other closely related entities.
Keywords: EWSR1-PBX3, Molecular diagnosis, Myoepithelial tumor, Rib tumor, Soft tissue tumor
Received: 28 Dec 2025; Accepted: 16 Feb 2026.
Copyright: © 2026 Yang, Liu, Zhao, Yu, Zhang, Li and Wang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Hongling Wang
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