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TFE 3‐positive renal cell carcinoma occurring in three children with dysfunctional kidneys on immunosuppression
Author(s) -
Schaefer Beverly A.,
Johnson Theodore S.,
Hooper David K.,
Nathan Jaimie D.,
Geller James I.
Publication year - 2017
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.12912
Subject(s) - immunosuppression , medicine , kidney , renal cell carcinoma , kidney disease , transplantation , kidney transplantation , dysfunctional family , pathogenesis , pathology , immunology , clinical psychology
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 TFE 3 or TFEB nuclear expression. We report three cases of pediatric TFE 3 positive RCC ( TFE 3‐ 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 .