Schaefer Beverly A, Johnson Theodore S, Hooper David K, Nathan Jaimie D, Geller James I
Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
Division of Pediatric Hematology/Oncology, Medical College of Georgia, Augusta University, Augusta, GA, USA.
Pediatr Transplant. 2017 Jun;21(4). doi: 10.1111/petr.12912. Epub 2017 Mar 21.
Pediatric RCC is a rare pediatric neoplasm and is distinctly different compared to adult RCC, often demonstrating translocation morphology evidenced by unique histopathological features and TFE3 or TFEB nuclear expression. We report three cases of pediatric TFE3 positive RCC (TFE3-RCC) occurring in the setting of chronic kidney disease and long-term pharmacological immunosuppression, including two cases that developed in the native kidney following kidney transplantation. Together, these cases suggest that the kidney microenvironment in combination with immune dysregulation is likely contributing factors in the pathogenesis of some pediatric RCC, warranting further study. Long-term post-transplant surveillance may warrant screening for RCC.
小儿肾细胞癌是一种罕见的小儿肿瘤，与成人肾细胞癌明显不同，常表现出易位形态，具有独特的组织病理学特征以及TFE3或TFEB核表达。我们报告了3例小儿TFE3阳性肾细胞癌（TFE3-RCC），这些病例发生于慢性肾病和长期药物免疫抑制背景下，其中2例在肾移植后的原肾中发生。总体而言，这些病例提示肾脏微环境与免疫失调相结合可能是某些小儿肾细胞癌发病机制中的促成因素，值得进一步研究。移植后长期监测可能需要对肾细胞癌进行筛查。
