Greco Federico, Faiella Eliodoro, Santucci Domiziana, Mallio Carlo Augusto, Nezzo Marco, Quattrocchi Carlo Cosimo, Beomonte Zobel Bruno, Grasso Rosario Francesco
Unit of Diagnostic Imaging, Università Campus Bio-Medico di Roma, Rome, Italy.
J Kidney Cancer VHL. 2017 Mar 21;4(1):1-7. doi: 10.15586/jkcvhl.2017.62. eCollection 2017.
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.
肾髓质癌(RMC)是一种罕见的、侵袭性很强的肿瘤,被认为是一种独立的病理实体。患有镰状细胞血红蛋白病的非洲裔青少年和年轻人是受影响最严重的群体。这种罕见的肾细胞癌亚型有其自身的形态发生和病理特征。主要临床表现包括肉眼血尿、腹部或侧腹痛以及体重减轻。预后非常差,95%的病例在疾病晚期被诊断出来。在本综述中,我们总结了肾髓质癌在超声、计算机断层扫描和磁共振等各种成像模式下的形态和动态特征。还讨论了鉴别诊断和管理策略。