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Low Grade Fibromyxoid Sarcoma
Definition 1
An indolent, late-metastasizing variant of fibrosarcoma characterized by alternating fibrous and myxoid areas and a whorling growth pattern. The neoplastic cells have a spindle morphology, and lack hyperchromasia or significant nuclear atypia. Approximately 40% of cases show the focal presence of collagen rosettes. A consistent, balanced t(7;16)(q32-34;p11) translocation has been identified, associated with the presence of FUS-CREB3L2 fusion protein. Most recent evidence suggests that rare cases may express the FUS-CREB3L1 fusion protein. (NCI Thesaurus)
Definition 2
A distinctive variant of fibrosarcoma, characterized by an admixture of heavily collagenized and myxoid zones, deceptively bland spindled cells with a whorling growth pattern and arcades of curvilinear blood vessels. There is a recurrent cytogenetic aberration, t(7;16)(q34;p11). (NCI Thesaurus)
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