Chen Xiao, Zhu Qingqiang, Li Baoxin, Cui Wenjing, Zhou Hao, Duan Na, Liu Yongkang, Kundra Vikas, Wang Zhongqiu
Department of Radiology, Affiliated Hospital of Nanjing University of Chinese Medicine, 155 Hanzhong Road, Nanjing, 210029, China.
Department of Medical Imaging, Subei People's Hospital, Medical School of Yangzhou University, No. 98 West Nantong Road, Yangzhou, 225001, China.
Eur Radiol. 2017 Feb;27(2):543-552. doi: 10.1007/s00330-016-4421-4. Epub 2016 Jun 2.
To characterize imaging features of renal cell carcinoma (RCC) associated with Xp11.2 translocation/TFE gene fusion.
Twenty-one patients with Xp11.2/TFE RCC were retrospectively evaluated. Tumour location, size, density, cystic or solid appearance, calcification, capsule sign, enhancement pattern and metastases were assessed.
Fourteen women and seven men were identified with 12 being 25 years old or younger. Tumours were solitary and cystic-solid (76.2 %) masses with a capsule (76.2 %); 90.5 % were located in the medulla. Calcifications and lymph node metastases were each observed in 24 %. On unenhanced CT, tumour attenuation was greater than in normal renal parenchyma (85.7 %). Tumour enhancement was less than in normal renal cortex on all enhanced phases, greater than in normal renal medulla on cortical and medullary phases, but less than in normal renal medulla on delayed phase. On MR, the tumours were isointense on T1WI, heterogeneously hypointense on T2WI and slightly hyperintense on diffusion-weighted imaging.
Xp11.2/TFE RCC usually occurs in young women. It is a cystic-solid, hyperdense mass with a capsule. It arises from the renal medulla with enhancement less than in the cortex but greater than in the medulla in all phases except the delayed phase, when it is lower than in the medulla.
• Xp11.2/TFE RCC was more prevalent in young women. • On unenhanced CT, Xp11.2/TFE RCC attenuation was greater than in renal parenchyma. • Xp111/2TFE RCC arises primarily from the renal medulla. • Xp11.2/TFE RCC enhancement was less than in the cortex on all phases. • Enhancement was greater than in the medulla in arterial and corticomedullary phase.
描述与Xp11.2易位/TFE基因融合相关的肾细胞癌（RCC）的影像学特征。
对21例Xp11.2/TFE RCC患者进行回顾性评估。评估肿瘤的位置、大小、密度、囊实性表现、钙化、包膜征、强化方式及转移情况。
共纳入14例女性和7例男性，其中12例年龄在25岁及以下。肿瘤多为孤立性囊实性肿块（76.2%），有包膜（76.2%）；90.5%位于髓质。钙化和淋巴结转移各占24%。平扫CT上，肿瘤密度高于正常肾实质（85.7%）。各期增强扫描肿瘤强化程度均低于正常肾皮质，皮质期和髓质期高于正常肾髓质，但延迟期低于正常肾髓质。在磁共振成像上，肿瘤在T1加权像上呈等信号，在T2加权像上呈不均匀低信号，在扩散加权成像上呈稍高信号。
Xp11.2/TFE RCC通常发生于年轻女性。它是一种有包膜的囊实性高密度肿块。起源于肾髓质，除延迟期强化低于肾髓质外，各期强化均低于皮质但高于髓质。
• Xp11.2/TFE RCC在年轻女性中更常见。• 平扫CT上，Xp11.2/TFE RCC密度高于肾实质。• Xp11.2/TFE RCC主要起源于肾髓质。• 各期Xp11.2/TFE RCC强化均低于皮质。• 动脉期和皮质髓质期强化高于髓质。
