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Congenital Mesoblastic Nephroma

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Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

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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...

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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, 47ā€“50.

    ArticleĀ  Google ScholarĀ 

  • Furtwaengler, R., Reinhard, H., Leuschner, I., et al. (2006). Mesoblastic nephroma ā€“ A report from the GPOH. Cancer, 106, 2275ā€“2283.

    ArticleĀ  Google ScholarĀ 

  • 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, 478ā€“481.

    ArticleĀ  Google ScholarĀ 

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Correspondence to Guido Martignoni .

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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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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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