Renal Medullary Carcinoma; A Rare Entity

Iranian Journal of Medical Sciences

Mine Önenerk¹  Gülistan Gümrükçü¹  Ali Murat Gökçe²  Selahattin Çalışkan³ 

[1] Department of Pathology,Haydarpaşa Numune Training and Research Hospital, Ä°stanbul,Turkey;Department of Surgery, Haydarpaşa Numune Training and Research Hospital, Ä°stanbul ,Turkey;Department of Urology, Çorum Training and Research Hospital, Hitit University, Çorum,Turkey

关键词: Medullary carcinoma;    Kidney neoplasms;    Case reports;   

DOI  :  

学科分类：基础医学

来源: Shiraz University of Medical Sciences

【 摘 要 】

Abstract Renal medullary carcinoma (RMC) is an uncommon aggressive neoplasm of the kidney. RMC is biologically aggressive with a very poor prognosis, and metastasis is seen in up to 95% of the patients at diagnosis or shortly thereafter. The common sites of metastasis are respectively lymph nodes, lungs, livers, and adrenal glands in order of frequency. The presence of poorly differentiated eosinophilic cells in a characteristic fibro-inflammatory stroma is seen in histological examination. The origin and pathogenesis of RMC are unclear. The radiographical and pathological findings suggest that RMC probably originates in the calyceal epithelium in or near the renal papillae, which could be the result of chronic ischemic damage in the renal papillae epithelium by sickled erythrocytes. Positivity of VEGF and HIF-1α supports the chronic hypoxia that may be caused in the pathogenesis of RMC. Other factors such as genetic or environmental factors are important. Although hemoglobinopathy is very common, RMC is very rare. An understanding of the molecular and genetic factors of this rare disease is important for its prevention and treatment. We herein describe an adult Turkish patient, who presented with hematuria. The diagnosis was RMC after pathological examination.

【 授权许可】

CC BY