Encyclopedia of Pathology

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

Low-Grade Myofibroblastic Sarcoma

  • Alessandro FranchiEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_5421-1
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Synonyms

Definition

Low-grade myofibroblastic sarcoma (LGMFS) is a malignant tumor showing myofibroblastic differentiation. Myofibroblasts are modified fibroblasts that acquire contractile cytoplasmic actin-containing microfilaments (stress fibers) and a specific cell-to-stroma attachment complex, the fibronexus or microtendon. Other ultrastructural features of myofibroblasts are a well-developed rough endoplasmic reticulum, Golgi complex, and collagen secretion granules in Golgi-derived vesicles. Myofibroblasts are the major constituent of granulation tissue, but they are also found in benign soft tissue lesions, like nodular fasciitis, as well as in low-grade and high-grade sarcomas as a minor component.

Clinical Features

  • Incidence

    LGMFS is a rare tumor.

  • Age

    It occurs over a wide age range, with a peak in the fourth and fifth decades (Mentzel et al. 1998; Montgomery et al. 2001; Fisher 2004; Qiu et al. 2008; Cai et al. 2013).

  • Sex

    It affects preferentially in...

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References and Further Reading

  1. Cai, C., Dehner, L. P., & El-Mofty, S. K. (2013). In myofibroblastic sarcomas of the head and neck, mitotic activity and necrosis define grade: A case study and literature review. Virchows Archiv, 463, 827–836.CrossRefGoogle Scholar
  2. Fisher, C. (2004). Myofibroblastic malignancies. Advances in Anatomic Pathology, 11, 190–201.CrossRefGoogle Scholar
  3. Mentzel, T., Dry, S., Katenkamp, D., & Fletcher, C. D. (1998). Low-grade myofibroblastic sarcoma: Analysis of 18 cases in the spectrum of myofibroblastic tumors. The American Journal of Surgical Pathology, 22, 1228–1238.CrossRefGoogle Scholar
  4. Montgomery, E., Goldblum, J. R., & Fisher, C. (2001). Myofibrosarcoma: A clinicopathologic study. The American Journal of Surgical Pathology, 25, 219–228.CrossRefGoogle Scholar
  5. Qiu, X., Montgomery, E., & Sun, B. (2008). Inflammatory myofibroblastic tumor and low-grade myofibroblastic sarcoma: A comparative study of clinicopathologic features and further observations on the immunohistochemical profile of myofibroblasts. Human Pathology, 39, 846–856.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Translational Research and of New Technologies in Medicine and SurgeryUniversity of PisaPisaItaly