Encyclopedia of Pathology

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

Low Grade Fibromyxoid Sarcoma

  • Judith V. M. G. BovéeEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_5420-1

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

  1. 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.CrossRefGoogle Scholar
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  7. 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.CrossRefGoogle Scholar
  8. 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.CrossRefGoogle Scholar
  9. 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.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

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