新近出现的一组高级别肾细胞癌,特别是遗传性平滑肌瘤病和肾细胞癌综合征相关性肾细胞癌,显示延胡索酸水合酶(FH)基因突变和功能缺失。基于这些肿瘤与浸润性腺癌中伴低分化灶的管状囊状癌(TC-PD)之间具有相似的细胞形态学和临床病理特征,作者推测这些肿瘤实体之间具有某种关联。 A new group of high-grade renal cell carcinomas, particularly hereditary leiomyomata and renal cell carcinoma-associated renal cell carcinomas, have been shown to exhibit mutations and functional deletions in the fumarate hydratase (FH) gene. Based on their similar morphological and clinical pathological features between these tumors and tubular cystic carcinoma with low differentiation in invasive adenocarcinomas (TC-PD), the authors speculate that there is some correlation between these tumor entities.