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Undifferentiated Pleomorphic Sarcoma

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

Synonyms

Malignant fibrous histiocytoma

Definition

Undifferentiated pleomorphic sarcoma (UPS) is a malignant mesenchymal tumor with no identifiable line of differentiation. It is a therefore diagnosis of exclusion.

Clinical Features

  • Incidence

    UPS originating in the genitourinary tract is rare, and literature consists mainly of single case reports.

  • Age

    UPS is a tumor of adults, arising mainly in patients between the fifth and the eight decades.

  • Sex

    It occurs predominantly in males.

  • Site

    Genitourinary UPS arises more frequently in the urinary bladder, but few examples have also been reported in the kidney, the prostate, and the spermatic cord (Kunze et al. 1994; Kulmala et al. 1994; Ptochos et al. 1999; Demir et al. 2012).

  • Treatment

    Treatment is surgical, combined in some cases with chemotherapy and radiation.

  • Outcome

    UPS is an aggressive tumor with frequent local and distant relapse and a poor prognosis.

Macroscopy

UPS usually appears as a large multilobulated gray-white, fleshy mass....

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References and Further Reading

  • de Peralta-Venturina, M., Moch, H., Amin, M., Tamboli, P., Hailemariam, S., Mihatsch, M., Javidan, J., Stricker, H., Ro, J. Y., & Amin, M. B. (2001). Sarcomatoid differentiation in renal cell carcinoma: A study of 101 cases. The American Journal of Surgical Pathology, 25, 275–284.

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  • Demir, L., Can, A., Dirican, A., Bayoğlu, V., Rezanko, T. A., Akyol, M., Kucukzeybek, Y., Erten, C., & Tarhan, M. O. (2012). Malignant fibrous histiocytoma of the spermatic cord in a patient with polycystic kidney disease; review of the literature. Clinical Genitourinary Cancer, 10, 280–283.

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  • Kulmala, R. V., Seppanen, J. H., Vaajalahti, P. J., et al. (1994). Malignant fibrous histiocytoma of the prostate. Case report. Scandinavian Journal of Urology and Nephrology, 28, 429–431.

    Article  CAS  Google Scholar 

  • Kunze, E., Theuring, F., & Kruger, G. (1994). Primary mesenchymal tumors of the urinary bladder. A histological and immunohistochemical study of 30 cases. Pathology, Research and Practice, 190, 311–332.

    Article  CAS  Google Scholar 

  • Ptochos, A., Karydas, G., Iosifidis, N., Tyrothoulakis, E., Papazafiriou, G., & Kehagia-Koutoufari, T. (1999). Primary renal malignant fibrous histiocytoma. A case report and review of the literature. Urologia Internationalis, 63, 261–264.

    Article  CAS  Google Scholar 

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Correspondence to Alessandro Franchi .

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Franchi, A. (2019). Undifferentiated Pleomorphic Sarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4964-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_4964-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

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