low grade fibromyxoid sarcoma
http://purl.obolibrary.org/obo/MONDO_0006272
A low grade, 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 t(7;16)(q33;p11) translocation has been identified in the majority of cases, associated with the presence of FUS-CREB3L2 fusion protein. Rare cases carry the t(11;16)(p11;p11) translocation which is associated with the presence of the FUS-CREB3L1 fusion protein. [ NCIT:C45202 ]
Term info
- NCIT:C45202 (MONDO:exact-label-match)
- EFO:1000328 (MONDO:equivalentTo)
- UMLS:C1275282 (NCIT:C45202)
- ONCOTREE:LGFMS (MONDO:equivalentTo)
- SCTID:404088004 (MONDO:equivalentTo)
- ICD9:171.9 (MONDO:relatedTo)
A low grade, 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 t(7;16)(q33;p11) translocation has been identified in the majority of cases, associated with the presence of FUS-CREB3L2 fusion protein. Rare cases carry the t(11;16)(p11;p11) translocation which is associated with the presence of the FUS-CREB3L1 fusion protein.
http://identifiers.org/snomedct/404088004, http://linkedlifedata.com/resource/umls/id/C1275282, http://purl.obolibrary.org/obo/NCIT_C45202
low grade fibromyxoid sarcoma
low-grade fibromyxoid sarcoma
MONDO:0006272