Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Spermatic Cord Desmoplastic Small Round Cell Tumor

  • Alessandro FranchiEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4944-1

Synonyms

Definition

Desmoplastic small round cell tumor (DSRCT) is a high-grade aggressive round cell tumor of uncertain histogenesis, associated with prominent stromal desmoplasia and polyphenotypic differentiation.

Clinical Features

  • Incidence

    Paratesticular DSRCT is very rare with approximately 20 such cases reported in the literature.

  • Age

    Like DSRCT at other sites, it is seen in older adolescents and young adults.

  • Treatment

    Surgical resection, often with orchiectomy, is the preferred local treatment. Radiotherapy may also be used. Chemotherapy is part of the standard therapy, both in the neoadjuvant and adjuvant setting.

  • Outcome

    The overall survival is poor, ranging between 15% and 30% at 5 years. However, tumors in the paratesticular location have a better survival, possibly because they are detected earlier and they are more amenable to complete surgical resection.

Macroscopy

DSRCT...

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References and Further Reading

  1. Cummings, O. W., Ulbright, T. M., Young, R. H., Dei Tos, A. P., Fletcher, C. D., & Hull, M. T. (1997). Desmoplastic small round cell tumors of the paratesticular region. A report of six cases. The American Journal of Surgical Pathology, 21, 219–225.CrossRefGoogle Scholar
  2. Sedig, L., Geiger, J., Mody, R., & Jasty-Rao, R. (2017). Paratesticular desmoplastic small round cell tumors: A case report and review of the literature. Pediatric Blood & Cancer, 64(12).  https://doi.org/10.1002/pbc.26631.CrossRefGoogle Scholar
  3. Thuret, R., Renaudin, K., Leclere, J., Battisti, S., Bouchot, O., & Theodore, C. (2005). Uncommon malignancies: Case 3. Paratesticular desmoplastic small round-cell tumor. Journal of Clinical Oncology, 23, 6253–6255.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

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