Abstract

Renal medullary carcinoma (RMC) is a rare, highly aggressive tumor recognized as an independent pathological entity. African-descent adolescents and young adults with sickle cell hemoglobinopathy are the most affected groups. This rare subtype of renal cell carcinoma has its own morphogenetic and pathological characteristics. The major clinical manifestations include gross hematuria, abdominal or flank pain, and weight loss. The prognosis is very poor, with 95% of cases diagnosed at an advanced stage of the disease. In this review, we summarize the morphologic and dynamic characteristics of RMC under various imaging modalities such as ultrasound, computed tomography, and magnetic resonance. Differential diagnosis and management strategies are also discussed.

Highlights

  • Renal medullary carcinoma (RMC) is a rare, highly aggressive tumor considered an independent pathological entity [1, 2]

  • Some authors consider RMC to be a subtype of collecting duct carcinoma [4]

  • Hypoxia increases the expression of hypoxia-inducible factor (HIF)

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Summary

Introduction

Renal medullary carcinoma (RMC) is a rare, highly aggressive tumor considered an independent pathological entity [1, 2]. Some authors consider RMC to be a subtype of collecting duct carcinoma [4] Both tumors are derived from the renal medulla, biologically aggressive, and characterized by an infiltrative growth pattern [1, 2]. In other two studies, ultrasound failed to detect renal masses, and in one of these, the increased echogenicity of the medullary areas of an enlarged right kidney was misinterpreted as papillary necrosis. Ten of these lesions were confirmed on histology (six clear cell carcinomas, three papillary carcinomas, and one lesion showed spindle and epithelioid cells, which could not exclude an angiomyolipoma) It appears that ultrasound imaging is insufficient for the diagnosis of RMC or medullary renal lesions.

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