Synonyms
Fibromyxosarcoma; Myxoid fibrosarcoma; Myxoid malignant fibrous histiocytoma
Definition
Myxofibrosarcoma (MFS) is a malignant fibroblastic neoplasm mainly involving the superficial soft tissues of elderly patients, with variably prominent myxoid stroma and cellular pleomorphism.
Clinical Features
Incidence
MFS is a relatively common sarcoma type.
Age
It is the most common sarcoma of adult and elderly patients.
Sex
It shows a slight male predominance.
Site
MFS typically arises in the lower limbs and limb girdles, followed by the upper extremities. It is usually a superficial tumor, involving the subcutis and the dermis, whereas subfascial sites are involved in approximately one third of the patients. True genitourinary MFS have been observed in the penis (Mentzel et al. 1996), whereas cases reported in the kidney, retroperitoneum, spermatic cord, and prostate most likely represent examples of dedifferentiated liposarcoma (Resorlu et al. 2018; Ozkan et al. 2006; Tearada et al. 2012...
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References and Further Reading
Mentzel, T., Calonje, E., Wadden, C., Camplejohn, R. S., Beham, A., Smith, M. A., & Fletcher, C. D. (1996). Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. The American Journal of Surgical Pathology, 20, 391–405.
Ozkan, B., Ozgüroğlu, M., Ozkara, H., Durak, H., & Talat, Z. (2006). Adult paratesticular myxofibrosarcoma: Report of a rare entity and review of the literature. International Urology and Nephrology, 38(1), 5–7.
Resorlu, B., Onem, K., & Germiyanoglu, R. C. (2018). Primary myxofibrosarcoma of the kidney. International Urology and Nephrology, 50(1), 79–80.
Tearada, H., Nagata, M., Mugiya, S., & Ozono, S. (2012). High-grade myxofibrosarcoma presenting at the spermatic cord after radiotherapy for prostate cancer. BMJ Case Report, 2012. pii:bcr0320126082.
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Franchi, A. (2020). Myxofibrosarcoma, Genitourinary Tract. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4856
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DOI: https://doi.org/10.1007/978-3-030-41894-6_4856
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