2020 Edition
| Editors: Maria Rosaria Raspollini, Antonio Lopez-Beltran


  • Alessandro FranchiEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_4931


Myosarcoma; Rhabdopoietic sarcoma; Rhabdosarcoma


Rhabdomyosarcoma (RMS) is a malignant tumor with skeletal muscle differentiation. Four subtypes are currently recognized: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing. The embryonal and the spindle cell/sclerosing subtypes are by far the most common in the urogenital tract.

Clinical Features

  • Incidence

    RMS is the most common pediatric soft tissue sarcoma, and approximately 10–15% of all RMSs originate in the bladder and prostate, making the genitourinary tract the second most common primary site.

  • Age

    RMS is a tumor of childhood and adolescence. Only rare cases have been reported in adults.

  • Sex

    Urinary bladder RMS shows a slight male predominance.

  • Site

    Embryonal RMS occurs in the soft tissue of the paratesticular region, bladder, and prostate. Spindle cell RMS involves the paratesticular region, while alveolar and pleomorphic RMS are exceedingly rare in the urogenital tract.

  • Treatment

    RMS is treated...

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References and Further Reading

  1. Ferrari, A., Bisogno, G., Casanova, M., Meazza, C., Piva, L., Cecchetto, G., Zanetti, I., Pilz, T., Mattke, A., Treuner, J., & Carli, M. (2002). Paratesticular rhabdomyosarcoma: Report from the Italian and German Cooperative Group. Journal of Clinical Oncology, 20, 449–455.PubMedCrossRefGoogle Scholar
  2. Fletcher, C. D., Bridge, J. A., Hogendoorn, P. C. W., & Mertens, F. (Eds.). (2013). WHO classification of tumours of soft tissue and bone. Lyon: IARC Press.Google Scholar
  3. Rodeberg, D. A., Anderson, J. R., Arndt, C. A., Ferrer, F. A., Raney, R. B., Jenney, M. E., Brecht, I. B., Koscielniak, E., Carli, M., Bisogno, G., Oberlin, O., Rey, A., Ullrich, F., Stevens, M. C., & Meyer, W. H. (2011). Comparison of outcomes based on treatment algorithms for rhabdomyosarcoma of the bladder/prostate: Combined results from the Children’s Oncology Group, German Cooperative Soft Tissue Sarcoma Study, Italian Cooperative Group, and International Society of Pediatric Oncology Malignant Mesenchymal Tumors Committee. International Journal of Cancer, 128, 1232–1239.PubMedCrossRefGoogle Scholar
  4. Rodríguez, D., Barrisford, G. W., Sanchez, A., Preston, M. A., Kreydin, E. I., & Olumi, A. F. (2014). Primary spermatic cord tumors: Disease characteristics, prognostic factors, and treatment outcomes. Urologic Oncology, 32, 52.e19–52.e25.CrossRefGoogle Scholar
  5. Seitz, G., Dantonello, T. M., Int-Veen, C., Blumenstock, G., Godzinski, J., Klingebiel, T., Schuck, A., Leuschner, I., Koscielniak, E., Fuchs, J., & CWS-96 Study Group. (2011). Treatment efficiency, outcome and surgical treatment problems in patients suffering from localized embryonal bladder/prostate rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma trial CWS-96. Pediatric Blood & Cancer, 56, 718–724.CrossRefGoogle Scholar
  6. Tavora, F., Kryvenko, O. N., & Epstein, J. I. (2013). Mesenchymal tumours of the bladder and prostate: An update. Pathology, 45, 104–115.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Translational Research and of New Technologies in Medicine and SurgeryUniversity of PisaPisaItaly