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
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.
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.
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.
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Franchi, A. (2019). Spermatic Cord Desmoplastic Small Round Cell Tumor. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4944-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4944-1
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