Definition
[Low grade fibromyxoid sarcoma is a low grade malignant fibroblastic neoplasm, with deceptively bland spindle cells in an alternating collagenous and myxoid matrix, characterized by FUS-CREB3L2 or FUS-CREB3L1 gene fusions, predominantly occurring in the deep soft tissues of the proximal extremities or trunk of young adults, with late metastases (Mohamed et al. 2017).]
Clinical Features
Incidence
[Low grade fibromyxoid sarcoma is very rare, with a reported incidence of 0.18 per million (Mohamed et al. 2017).]
Age
[The tumor can affect patients of all ages but has a peak incidence in young adults, with a median age of 33 years (Mohamed et al. 2017). The reported age range is from 10 to 69 years. 13–19% of cases occur in patients younger than 18 years of age while being extremely rare in children under 5 years of age (Mohamed et al. 2017).]
Sex
[There is a strong male predominance in infants and young adolescents (Mohamed et al. 2017). In adults the distribution between sexes...
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References
Doyle, L. A., Moller, E., Dal Cin, P., Fletcher, C. D., Mertens, F., & Hornick, J. L. (2011). MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. The American Journal of Surgical Pathology, 35(5), 733–741.
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Folpe, A. L., Lane, K. L., Paull, G., & Weiss, S. W. (2000). Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: A clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas. The American Journal of Surgical Pathology, 24(10), 1353–1360.
Guillou, L., Benhattar, J., Gengler, C., et al. (2007). Translocation-positive low-grade fibromyxoid sarcoma: Clinicopathologic and molecular analysis of a series expanding the morphologic spectrum and suggesting potential relationship to sclerosing epithelioid fibrosarcoma: A study from the French sarcoma group. The American Journal of Surgical Pathology, 31(9), 1387–1402.
Mohamed, M., Fisher, C., & Thway, K. (2017). Low-grade fibromyxoid sarcoma: Clinical, morphologic and genetic features. Annals of Diagnostic Pathology, 28, 60–67.
Prieto-Granada, C., Zhang, L., Chen, H. W., et al. (2015). A genetic dichotomy between pure sclerosing epithelioid fibrosarcoma (SEF) and hybrid SEF/low-grade fibromyxoid sarcoma: A pathologic and molecular study of 18 cases. Genes, Chromosomes & Cancer, 54(1), 28–38.
Reid, R., de Silva, M. V., Paterson, L., Ryan, E., & Fisher, C. (2003). Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes share a common t(7;16)(q34;p11) translocation. The American Journal of Surgical Pathology, 27(9), 1229–1236.
Thway, K., Ng, W., Benson, C., Chapman, J., & Fisher, C. (2015). DOG1 expression in low-grade Fibromyxoid sarcoma: A study of 11 cases, with molecular characterization. International Journal of Surgical Pathology, 23(6), 454–460.
Vallejo-Benitez, A., Rodriguez-Zarco, E., Carrasco, S. P., et al. (2017). Expression of dog1 in low-grade fibromyxoid sarcoma: A study of 19 cases and review of the literature. Annals of Diagnostic Pathology, 30, 8–11.
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Bovée, J.V.M.G. (2020). Low Grade Fibromyxoid Sarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5420-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5420-1
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