ALK易位性肾细胞癌
ALK-translocation RCC
夏成青 赵明 程亮
更新时间：2019-12-27 22:31:00
概述：
发病部位：肾脏
诊断要点：
1. 罕见，占所有成人肾细胞癌的比例＜1%；发病年龄谱系宽广，6-61岁，男性略好发，男/女约1.4比1；发生在儿童患者部分与镰刀细胞病有关；
2. 大体切面灰白，实性或囊性，直径3-7cm；
3. 镜下肿瘤具有多种组织学生长方式，VCL-ALK和TMP3-ALK融合的肿瘤具有相似的组织学特征常见弥漫的实性和局灶的微囊和小管状结构，间质血管较丰富，部分可见丰富的淋巴浆细胞浸润和间质促纤维增生性反应，类似于肾髓质癌；瘤细胞黏附性较差，呈多角形、梭形至立方状和低柱状，胞浆丰富嗜酸性常见胞浆内空泡形成；核圆形或卵圆形，局灶可见核沟，核仁较明显（ISUP核分级3级）；
4. 非VCL和TMP3易位的肿瘤显示更大的组织学异质性包括乳头状（假乳头状结构）、筛状结构、实性、梁状、管状或管囊状结构，局部可见丰富的黏液性间质以及胞浆内黏液；瘤细胞胞浆嗜酸性，可见印戒样细胞，横纹肌样细胞，胞浆内空泡以及多形性的多核巨细胞等，间质可见砂粒体钙化和泡沫细胞聚集，有时可见地图样坏死。
免疫组织化学染色：
分子标记：
鉴别诊断：
治疗：
参考文献：
1.Yu W, Wang Y, Jiang Y, Zhang W, Li Y. Genetic analysis and clinicopathological features of ALK-rearranged renal cell carcinoma in a large series of resected Chinese renal cell carcinoma patients and literature review. Histopathology 2017; 71;53–62.
2.Sukov WR, Hodge JC, Lohse CM et al. ALK alterations in adult renal cell carcinoma: frequency, clinicopathologic features and outcome in a large series of consecutively treated patients. Mod. Pathol. 2012; 25; 1516–1525.
3.Cajaiba MM, Dyer LM, Geller JI et al. The classification of pediatric and young adult renal cell carcinomas registered on the Children’s Oncology Group (COG) protocol AREN03B2 after focused genetic testing. Cancer 2018; 124; 3381–3389.
4.Smith NE, Deyrup AT, Marino-Enriquez A et al. VCL–ALK renal cell carcinoma in children with sickle-cell trait: the eighth sicklecell nephropathy? Am. J. Surg. Pathol. 2014; 38; 858–863.
5.Debelenko LV, Raimondi SC, Daw N et al. Renal cell carcinoma with novel VCL–ALK fusion: new representative of ALKassociated tumor spectrum. Mod. Pathol. 2011; 24; 430–442.
