Definition
Epithelioid sarcoma (ES) is a malignant tumor.
Clinical Features
Incidence
ES of the genitourinary tract is rare, and literature consists mainly of single-case reports.
Age
ES is a tumor of young adults, but the proximal type occurs mainly in older adults (median age around 40 years).
Sex
There is a male gender predilection.
Site
ES typically originates in the distal extremities, but the proximal variant involves also the perineum and the genital tract, mainly localizing in the penis and the scrotum.
Treatment
Wide surgical excision is the mainstay of treatment, but more conservative surgery followed by radiotherapy has also been used.
Outcome
ES is an aggressive tumor often with a protracted clinical course, characterized by repeated local recurrences. Metastases may develop after several years and involve primarily the lungs, but lymph nodes may be involved as well.
Macroscopy
ES presents as a hard nodule or multiple nodules with ill-defined borders and central areas of...
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References and Further Reading
Armah, H. B., Palekar, A., Rao, U. N., & Parwani, A. V. (2008). Proximal-type epithelioid sarcoma of the scrotum: A difficult diagnosis in an unusual location and review of the literature. Pathology, 40, 326–330.
Onol, F. F., Tanidir, Y., Kotiloğlu, E., Bayramiçli, M., Turhal, S., & Türkeri, L. N. (2006). Proximal type epithelioid sarcoma of the scrotum: A source of diagnostic confusion that needs immediate attention. European Urology, 49, 406–407.
Usta, M. F., Adams, D. M., Zhang, J. W., Davis, R., & Hellstrom, W. J. (2003). Penile epithelioid sarcoma and the case for a histopathological diagnosis in Peyronie’s disease. BJU International, 91, 519–521.
Yaman, O., Bozlu, M., Heper, A. O., Tulunay, O., Anafarta, K., & Göğüş, O. (1999). Penile epithelioid sarcoma. Urologia Internationalis, 62, 252–254.
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Franchi, A. (2020). Epithelioid Sarcoma. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_3743
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DOI: https://doi.org/10.1007/978-3-030-41894-6_3743
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