Definition
Ewing sarcoma (ES) is a high-grade round cell translocation-associated sarcoma, showing varying degrees of neuroectodermal differentiation.
Clinical Features
Incidence
ES only rarely involves the genitourinary tract.
Age
Genitourinary ES arises in adolescents and young adults (Celli and Cai 2016; Teegavarapu et al. 2017; Thyavihally et al. 2008).
Sex
There is a predilection for the male gender (Celli and Cai 2016; Teegavarapu et al. 2017; Thyavihally et al. 2008).
Site
Genitourinary ES arises more frequently in the kidney, but some examples have also been reported in the prostate, the urinary bladder, the scrotum, and the penis (Colecchia et al. 2003; Funahashi et al. 2009; Peyromaure et al. 2003; Okada et al. 2011; Grimsby and Harrison 2014; Kilicaslan et al. 2008).
Treatment
Treatment is usually based on current regimens used in ES of the bone. Surgical resection is the preferred local treatment (Celli and Cai 2016;...
References and Further Reading
Celli, R., & Cai, G. (2016). Ewing sarcoma/primitive neuroectodermal tumor of the kidney: A rare and lethal entity. Archives of Pathology & Laboratory Medicine, 140, 281–285.
Colecchia, M., Dagrada, G., Poliani, P. L., Messina, A., & Pilotti, S. (2003). Primary primitive peripheral neuroectodermal tumor of the prostate. Immunophenotypic and molecular study of a case. Archives of Pathology & Laboratory Medicine, 127, e190–e193.
Funahashi, Y., Yoshino, Y., & Hattori, R. (2009). Ewing’s sarcoma/primitive neuroectodermal tumor of the prostate. International Journal of Urology, 16, 769.
Grimsby, G. M., & Harrison, C. B. (2014). Ewing sarcoma of the scrotum. Urology, 83, 1407–1408.
Kilicaslan, I., Karayigit, E., Bulut, F., Basaran, M., Dizdar, Y., Aslay, I., & Uysal, V. (2008). Ewing’s sarcoma/primitive neuroectodermal tumor (ES/PNET) of the penis. International Urology and Nephrology, 40, 113–115.
Okada, Y., Kamata, S., Akashi, T., Kurata, M., Nakamura, T., & Kihara, K. (2011). Primitive neuroectodermal tumor/Ewing’s sarcoma of the urinary bladder: A case report and its molecular diagnosis. International Journal of Clinical Oncology, 16, 435–438.
Peyromaure, M., Vieillefond, A., Boucher, E., et al. (2003). Primitive neuroectodermal tumor of the prostate. The Journal of Urology, 170, 182–183.
Risi, E., Iacovelli, R., Altavilla, A., et al. (2013). Clinical and pathological features of primary neuroectodermal tumor/Ewing sarcoma of the kidney. Urology, 82, 382–386.
Teegavarapu, P. S., Rao, P., Matrana, M. R., Cauley, D. H., Wood, C. G., Patel, S., & Tannir, N. M. (2017). Outcomes of adults with Ewing sarcoma family of tumors (ESFT) of the kidney: A single-institution experience. American Journal of Clinical Oncology, 40, 189–193.
Thyavihally, Y. B., Tongaonkar, H. B., Gupta, S., et al. (2008). Primitive neuroectodermal tumor of the kidney: A single institute series of 16 patients. Urology, 71, 292–296.
Yoshida, A., Sekine, S., Tsuta, K., Fukayama, M., Furuta, K., & Tsuda, H. (2012). NKX2.2 is a useful immunohistochemical marker for Ewing sarcoma. The American Journal of Surgical Pathology, 36, 993–999.
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Franchi, A. (2019). Ewing Sarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5000-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5000-1
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