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EWSR1-SMAD3-Positive Fibroblastic Tumor

MedGen UID:
Concept ID:
Neoplastic Process
Synonyms: EWSR1-SMAD3-positive fibroblastic tumor; EWSR1-SMAD3-Rearranged Fibroblastic Tumor; EWSR1::SMAD3-Positive Fibroblastic Tumor
SNOMED CT: EWSR1-SMAD3-positive fibroblastic tumor (1292983002); EWSR1-SMAD3-positive fibroblastic tumor (1292984008)


A benign fibroblastic neoplasm characterized by a fusion of exon 7 of EWSR1 gene with exon 5 of SMAD3 gene. Patients usually present with a small painless superficial tumor in the hands and feet. Morphologically, there is zonation with acellular hyalinized center and peripheral proliferation of fibroblastic spindle cells without atypia. It may recur following incomplete excision. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEWSR1-SMAD3-Positive Fibroblastic Tumor

Recent clinical studies


Foot O, Hallin M, Jones RL, Sumathi VP, Thway K
Int J Surg Pathol 2021 Apr;29(2):179-181. Epub 2020 Jul 3 doi: 10.1177/1066896920938124. PMID: 32615834


Zhao L, Sun M, Lao IW, Yu L, Wang J
Exp Mol Pathol 2019 Oct;110:104291. Epub 2019 Jul 31 doi: 10.1016/j.yexmp.2019.104291. PMID: 31376366

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