Low Grade Fibromyxoid Sarcoma
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[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).]
[Low grade fibromyxoid sarcoma is very rare, with a reported incidence of 0.18 per million (Mohamed et al. 2017).]
[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).]
[There is a strong male predominance in infants and young adolescents (Mohamed et al. 2017). In adults the distribution between sexes...
- 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.CrossRefGoogle Scholar
- 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.CrossRefGoogle Scholar