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Renal Medullary Carcinoma: a Report of the Current Literature

  • Kidney Diseases (G Ciancio, Section Editor)
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Abstract

Purpose of the Review

We present an updated report of renal medullary carcinoma (RMC), a rare and aggressive condition.

Recent Findings

There is a majority of male patients, of African descent, in the second or third decade of life. In differential diagnosis, other tumors, such as malignant rhabdoid tumor (MRT), vinculin-anaplastic lymphoma kinase (VCL-ALK) translocation renal cell carcinoma, and collecting duct carcinoma, may present difficulties. Abnormalities of tumor suppressor gene SMARCB1 have been found in RMC. Reported symptoms were hematuria, pain, weight loss, respiratory distress, palpable mass, cough, and fever. Most patients present with metastases at diagnosis. There is no definite recommended treatment, and protocols are extrapolated from other malignancies, with nephrectomy and systemic therapies being most frequently used. Response to treatment and prognosis remain very poor.

Summary

RMC is a rare and aggressive tumor. Definitive diagnosis requires histological assessment and the presence of sickle-cell hemoglobinopathies.

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Correspondence to Javier Roberti.

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Leandro Blas, Javier Roberti, Jorgelina Petroni, Liliana Reniero, and Federico Cicora each declare no potential conflicts of interest.

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Blas, L., Roberti, J., Petroni, J. et al. Renal Medullary Carcinoma: a Report of the Current Literature. Curr Urol Rep 20, 4 (2019). https://doi.org/10.1007/s11934-019-0865-9

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