Format

Send to

Choose Destination
Pediatr Transplant. 2017 Jun;21(4). doi: 10.1111/petr.12912. Epub 2017 Mar 21.

TFE3-positive renal cell carcinoma occurring in three children with dysfunctional kidneys on immunosuppression.

Author information

1
Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
2
Division of Pediatric Hematology/Oncology, Medical College of Georgia, Augusta University, Augusta, GA, USA.
3
Division of Pediatric Nephrology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
4
Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.

Abstract

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.

KEYWORDS:

TFE3-positive RCC; pediatric renal transplant; renal cell carcinoma

PMID:
28322484
DOI:
10.1111/petr.12912
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center