Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Low Grade Fibromyxoid Sarcoma

Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_5420-1


[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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Authors and Affiliations

  1. 1.Department of PathologyLeiden University Medical Center (LUMC)LeidenThe Netherlands