Definition
A highly aggressive carcinoma centered on the renal medulla associated with sickle cell and related hemoglobinopathies (Davis et al. 1995).
Clinical Features
Incidence
Renal medullary carcinoma is a very uncommon tumor.
Age
There is a predilection for young adults.
Sex
There is a remarkable male predominance.
Site
By definition, it is centered on the renal medulla.
Treatment
Radical nephrectomy is the standard treatment.
Outcome
Renal medullary carcinoma is an aggressive tumor. All patients are symptomatic at diagnosis with flank or abdominal pain and gross hematuria, with metastatic disease at the time of diagnosis.
Macroscopy
These tumors are poorly circumscribed medulla-centered and variable in size.
Microscopy
Architecturally, the most common patterns are reticular and cribriform glands; however many other patterns have been seen. The stroma present in the neoplasia is fibrotic/desmoplastic with intratumoral neutrophil predominant inflammatory infiltrates....
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References and Further Reading
Amin, M. B., Smith, S. C., Agaimy, A., et al. (2014). Collecting duct carcinoma versus renal medullary carcinoma: An appeal for nosologic and biological clarity. The American Journal of Surgical Pathology, 38, 871–874.
Davis, C. J., Jr., Mostofi, F. K., & Sesterhenn, I. A. (1995). Renal medullary carcinoma. The seventh sickle cell nephropathy. The American Journal of Surgical Pathology, 19, 1–11.
Ohe, C., Smith, S. C., Sirohi, D., et al. (2018). Reappraisal of morphologic differences between renal medullary carcinoma, collecting duct carcinoma, and fumarate hydratase-deficient renal cell carcinoma. The American Journal of Surgical Pathology, 42, 279–292.
Sirohi, D., Smith, S. C., Ohe, C., et al. (2017). Renal cell carcinoma, unclassified with medullary phenotype: Poorly differentiated adenocarcinomas overlapping with renal medullary carcinoma. Human Pathology, 67, 134–145.
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Caliò, A., Segala, D., Martignoni, G. (2019). Renal Medullary Carcinoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4924-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4924-1
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