Low-grade fibromyxoid sarcoma: a brief review

Arch Pathol Lab Med. 2006 Sep;130(9):1358-60. doi: 10.5858/2006-130-1358-LFSABR.


Low-grade fibromyxoid sarcomas are uncommon deep soft tissue neoplasms first described by Evans in 1987. They exhibit a deceptively benign appearance, with a whorled or linear arrangement of spindle-shaped cells showing few to absent mitoses. A characteristic, but not specific, feature is the presence of areas of myxoid stroma. Recurrences are common, and late metastases have been recorded. A closely related but morphologically distinct tumor, the so-called hyalinizing spindle cell tumor with giant rosettes, has also been described; both neoplasms share the same cytogenetic abnormality, a balanced translocation resulting in a FUS/CREB3L2 fusion gene. Because of similar clinical behavior and the common cytogenetic abnormality, some authors prefer to consider both lesions as a single entity within the spectrum of low-grade sarcomas.

Publication types

  • Review

MeSH terms

  • Cyclic AMP Response Element-Binding Protein / genetics
  • Fibroma / genetics
  • Fibroma / pathology*
  • Humans
  • Leucine Zippers
  • Membrane Fusion Proteins / genetics*
  • Nerve Tissue Proteins / genetics
  • Oncogene Fusion
  • RNA-Binding Protein FUS / genetics
  • Sarcoma / genetics
  • Sarcoma / pathology*
  • Soft Tissue Neoplasms / genetics
  • Soft Tissue Neoplasms / pathology*
  • Translocation, Genetic


  • Cyclic AMP Response Element-Binding Protein
  • Membrane Fusion Proteins
  • Nerve Tissue Proteins
  • RNA-Binding Protein FUS