Low-grade fibromyxoid sarcoma: a brief review.
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.
Vernon
Stephen E
SE
Department of Pathology, University of Miami/JMH, Miami, Fla. 33136, USA. Svernon@med.miami.edu
Bejarano
Pablo A
PA
D017362:Cyclic AMP Response Element-Binding Protein / Q000235:genetics; D005350:Fibroma / Q000235:genetics / Q000473:pathology*; D006801:Humans; D016350:Leucine Zippers; D050576:Membrane Fusion Proteins / Q000235:genetics*; D009419:Nerve Tissue Proteins / Q000235:genetics; D050596:Oncogene Fusion; D034702:RNA-Binding Protein FUS / Q000235:genetics; D012509:Sarcoma / Q000235:genetics / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000235:genetics / Q000473:pathology*; D014178:Translocation, Genetic
D017362:Cyclic AMP Response Element-Binding Protein; D050576:Membrane Fusion Proteins; D009419:Nerve Tissue Proteins; D034702:RNA-Binding Protein FUS