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Published in: Current Urology Reports 1/2019

01-01-2019 | Nephrectomy | Kidney Diseases (G Ciancio, Section Editor)

Renal Medullary Carcinoma: a Report of the Current Literature

Authors: Leandro Blas, Javier Roberti, Jorgelina Petroni, Liliana Reniero, Federico Cicora

Published in: Current Urology Reports | Issue 1/2019

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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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Metadata
Title
Renal Medullary Carcinoma: a Report of the Current Literature
Authors
Leandro Blas
Javier Roberti
Jorgelina Petroni
Liliana Reniero
Federico Cicora
Publication date
01-01-2019
Publisher
Springer US
Published in
Current Urology Reports / Issue 1/2019
Print ISSN: 1527-2737
Electronic ISSN: 1534-6285
DOI
https://doi.org/10.1007/s11934-019-0865-9

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