Definition
Clear cell sarcoma of the kidney is a rare malignant mesenchymal tumor occurring in children.
Clinical Features
Incidence
Clear cell sarcoma comprises 4% of pediatric renal tumors.
Age
It occurs usually at 2ā3 years of age and it is extremely rare before 6 months.
Sex
Male-to-female ratio is 1.6:1.
Site
The tumor usually shows the epicenter at the renal medulla.
Treatment
The treatment consists of surgical resection, when possible, followed by chemotherapy and radiotherapy.
Outcome
With current treatment (addition of doxorubicin to chemotherapeutic protocols), outcome has improved with a 5-year overall survival of 75ā90%. Bone metastases are common, and unusual sites, such as soft tissues of orbit, may occur. Brain metastases are more common, due to blood-brain barrier which impedes effective chemotherapy. True stage I disease has a favorable prognosis, and chemotherapy protocols are stage-dependent (Argani et al. 2000).
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References and Further Reading
Argani, P., Perlman, E. J., Breslow, N. E., et al. (2000). Clear cell sarcoma of the kidney: A review of 351 cases from the National Wilms Tumor Study Group Pathology Center. The American Journal of Surgical Pathology, 24, 4ā18.
Aw SJ, Chang KTE (2019). Clear cell sarcoma of the kidney. Archives of Pathology & Laboratory Medicine. [Epub ahead of print].
Gooskens, S. L., Furtwangler, R., Vujanic, G. M., et al. (2012). Clear cell sarcoma of the kidney: A review. European Journal of Cancer, 48, 2219ā2226.
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CaliĆ², A., Segala, D., Martignoni, G. (2019). Renal Clear Cell Sarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4922-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4922-1
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