Abstract
Epithelioid sarcomas tend to occur in young adults (Fig. 14.1) either in distal locations (classic form) or in the perineum/groin area (so-called the proximal type). The classic form typically arises in the feet, lower extremity, digits, or forearms of younger men, and can be sometimes difficult to distinguish from reactive processes, such a granuloma annulare or an ulcer base. In contrast, the proximal-type epithelioid sarcoma exhibits a high degree of cytologic atypia, sometimes displaying rhabdoid morphology microscopically (Fig. 14.2) [1]. The classic form of epithelioid sarcoma is usually a slowly growing lesion, that metastasizes relatively early to lymph nodes, which will be positive in 1/3 to 1/2 of cases (Fig. 14.3) [2, 3]. The proximal type is often associated with a more aggressive clinical behavior, with local, regional, and metastatic disease developing over several years.
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Brennan, M.F., Antonescu, C.R., Alektiar, K.M., Maki, R.G. (2016). Epithelioid Sarcoma. In: Management of Soft Tissue Sarcoma. Springer, Cham. https://doi.org/10.1007/978-3-319-41906-0_14
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DOI: https://doi.org/10.1007/978-3-319-41906-0_14
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