Definition
Congenital mesoblastic nephroma is a low-grade fibroblastic neoplasm arising in the renal sinus of children.
Clinical Features
Incidence
It comprises 5% of pediatric renal neoplasms.
Age
It is the most common kidney tumor in the first 3 months of life and is uncommon after 6 months.
Sex
There is no gender predominance.
Site
They are located in the renal sinus.
Treatment
The treatment of choice is surgical excision.
Outcome
Mesoblastic nephroma is a low-grade neoplasm; it has infiltrative borders which must be studied carefully because the risk of recurrence appears to be dependent upon the completeness of the resection (Furtwaengler et al. 2006). Only rare cases with hematogeneous metastases have been reported, which are treated with chemotherapy (Loeb et al. 2002).
Macroscopy
Grossly this neoplasm is usually large, and its cut surface resembles that of a leiomyoma (firm, whorled or trabeculated, and light colored). The tumor is not encapsulated and typically interdigitated...
References and Further Reading
El Demellawy, D., Cundiff, C. A., Nasr, A., et al. (2016). Congenital mesoblastic nephroma: A study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement. Pathology, 48, 47ā50.
Furtwaengler, R., Reinhard, H., Leuschner, I., et al. (2006). Mesoblastic nephroma ā A report from the GPOH. Cancer, 106, 2275ā2283.
Loeb, D. M., Hill, D. A., & Dome, J. S. (2002). Complete response to recurrent cellular congenital mesoblastic nephroma to chemotherapy. Journal of Pediatric Hematology/Oncology, 24, 478ā481.
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CaliĆ², A., Segala, D., Martignoni, G. (2019). Congenital Mesoblastic Nephroma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4795-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4795-1
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