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

Beverly A. Schaefer, Theodore S. Johnson, David K. Hooper, Jaimie D. Nathan, James I. Geller

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Article numbere12912
JournalPediatric Transplantation
Volume21
Issue number4
DOIs
StatePublished - Jun 2017

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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