Chapter 12
EWSR1-SMAD3-Positive Fibroblastic Tumor: A New Frontier in Tumor Pathology
- By Parth Goswami, Rushang Dave, Sonagara NikhilKumar Mavjibhai - 10 Jun 2026
- Emerging Trends in Medical Research and Practice, Volume: 1, Pages: 70 - 73
Abstract/Preface
The EWSR1-SMAD3-positive fibroblastic tumor is recently added to the fifth edition of the WHO Classification of Soft Tissue Tumors. This rare tumor is formed by t(22;15)(q12;q21) translocation. That leads to the formation of the EWSR1-SMAD3 fusion gene. TGF B/SMAD signaling pathway disrupts results in tumorigenesis. Only 15 cases have been reported till date. Tumors are most commonly located on soft tissue extremities. Tumors have low malignant potential and locally recur if not effectively removed. Diagnosis based on FISH, RT-PCR, and NGS. Future research should focus on unraveling the oncogenic mechanisms of the EWSR1-SMAD3 fusion and exploring targeted therapies. This review shows molecular pathology in diagnosing and managing this emerging tumor entity.