Definition
Benign soft tissue tumor composed of benign-appearing spindle cells set in an abundant myxoid hypovascular stroma.
Clinical Features
Incidence
It is a rare tumor. It may occur sporadically or in the context of Mazabraud syndrome (single or multiple intramuscular myxomas associated with monostotic or polyostotic fibrous dysplasia) (Nielsen and Hogendoorn 2013).
Age
It generally affects adult patients, with a peak in the fourth to seventh decades of life.
Sex
Females are more frequently affected than males.
Site
It generally arises in the extremities, especially in the thigh.
Treatment
Simple surgical excision is curative.
Outcome
It is a benign lesion, with a low risk of local recurrence after incomplete surgery. No malignant transformation has been reported (Nielsen and Hogendoorn 2013).
Macroscopy
Size usually ranges between 5 and 10 cm in greatest diameter. The tumor appears well-circumscribed and diffusely gelatinous on cut surface, sometimes featuring fluid-filled cystic...
References
Enzinger, F. M. (1965). Intramuscular myxoma: A review and follow-up study of 34 cases. American Journal of Clinical Pathology, 43, 104–113.
Miettinen, M., Höckerstedt, K., Reitamo, J., & Tötterman, S. (1985). Intramuscular myxoma – A clinicopathological study of twenty-three cases. American Journal of Clinical Pathology, 84(3), 265–272.
Nielsen, G. P., & Hogendoorn, P. C. W. (2013). Intramuscular myxoma. In C. D. M. Fletcher, J. A. Bridge, P. C. W. Hogendoorn, & F. Mertens (Eds.), World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone (pp. 195–196). Lyon: IARC Press.
Okamoto, S., Hisaoka, M., Ushijima, M., Nakahara, S., Toyoshima, S., & Hashimoto, H. (2000). Activating Gs(alpha) mutation in intramuscular myxomas with and without fibrous dysplasia of bone. Virchows Archiv, 437(2), 133–137.
van Roggen, J. F., McMenamin, M. E., & Fletcher, C. D. (2001). Cellular myxoma of soft tissue: A clinicopathological study of 38 cases confirming indolent clinical behaviour. Histopathology, 39(3), 287–297.
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Gambarotti, M. (2020). Intramuscular Myxoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5407-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5407-1
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