Synonyms
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....
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.
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.
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.
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.
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.
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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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