Definition
Myxoinflammatory fibroblastic sarcoma is a locally aggressive fibroblastic neoplasm composed of cytologically atypical cells with macronucleoli deposited in a variably myxoid and hyalinized stroma with a prominent inflammatory infiltrate.
Clinical Features
Incidence
Myxoinflammatory fibroblastic sarcoma is a rare mesenchymal neoplasm.
Age
These tumors occur of a broad age range but are most common in adulthood (median 40 years) (Laskin et al. 2014).
Sex
Males and females are affected equally (Laskin et al. 2014).
Site
The overwhelming majority of myxoinflammatory fibroblastic sarcomas present as a slow-growing painless mass in the distal extremity. The upper extremity is affected more often than the lower extremity, and the finger/hand is the most common site overall (Montgomery et al. 1998; Meis-Kindblom and Kindblom 1998). These tumors are typically centered in subcutaneous soft tissue.
Treatment
Wide local excision with negative margins is the treatment of choice.
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References and Further Reading
Kao, Y. C., Ranucci, V., Zhang, L., et al. (2017). Recurrent BRAF Gene Rearrangements in Myxoinflammatory Fibroblastic Sarcomas, but Not Hemosiderotic Fibrolipomatous Tumors. The American Journal of Surgical Pathology, 41, 1456–1465.
Kovarik, C. L., Barrett, T., Auerbach, A., & Cassarino, D. S. (2008). Acral myxoinflammatory fibroblastic sarcoma: case series and immunohistochemical analysis. Journal of Cutaneous Pathology, 35, 192–196.
Lambert, I., Debiec-Rychter, M., Guelinckx, P., Hagemeijer, A., & Sciot, R. (2001). Acral myxoinflammatory fibroblastic sarcoma with unique clonal chromosomal changes. Virchows Archiv, 438, 509–512.
Laskin, W. B., Fetsch, J. F., & Miettinen, M. (2014). Myxoinflammatory fibroblastic sarcoma: a clinicopathologic analysis of 104 cases, with emphasis on predictors of outcome. The American Journal of Surgical Pathology, 38, 1–12.
Meis-Kindblom, J. M., & Kindblom, L. G. (1998). Acral myxoinflammatory fibroblastic sarcoma: a low-grade tumor of the hands and feet. The American Journal of Surgical Pathology, 22, 911–924.
Montgomery, E. A., Devaney, K. O., Giordano, T. J., & Weiss, S. W. (1998). Inflammatory myxohyaline tumor of distal extremities with virocyte or Reed-Sternberg-like cells: a distinctive lesion with features simulating inflammatory conditions, Hodgkin’s disease and various sarcomas. Modern Pathology, 11, 384–391.
Zreik, R. T., Carter, J. M., Sukov, W. R., et al. (2016). TGFBR3 and MGEA5 rearrangements are much more common in “hybrid” hemosiderotic fibrolipomatous tumor-myxoinflammatory fibroblastic sarcomas than in classical myxoinflammatory fibroblastic sarcomas: a morphological and fluorescence in situ hybridization study. Human Pathology, 53, 14–24.
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Fritchie, K. (2020). Myxoinflammatory Fibroblastic Sarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5432-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5432-1
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