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Epithelioid Sarcoma

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 114 Accesses

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.

    Article  Google Scholar 

  • Onol, F. F., Tanidir, Y., KotiloÄŸlu, 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.

    Article  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • Yaman, O., Bozlu, M., Heper, A. O., Tulunay, O., Anafarta, K., & Göğüş, O. (1999). Penile epithelioid sarcoma. Urologia Internationalis, 62, 252–254.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Translational Research and of New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy

    Alessandro Franchi

Authors
  1. Alessandro Franchi
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Corresponding author

Correspondence to Alessandro Franchi .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Franchi, A. (2019). Epithelioid Sarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3743-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_3743-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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