TFE3-positive renal cell carcinoma occurring in three children with dysfunctional kidneys on immunosuppression.
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.
Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
Beverly A
http://orcid.org/0000-0001-5427-9666
BA
Schaefer
Division of Pediatric Hematology/Oncology, Medical College of Georgia, Augusta University, Augusta, GA, USA.
Theodore S
TS
Johnson
Division of Pediatric Nephrology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
David K
DK
Hooper
Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
Jaimie D
JD
Nathan
Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
James I
JI
Geller
D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000378:metabolism*; D014408:Biomarkers, Tumor / Q000378:metabolism*; D002292:Carcinoma, Renal Cell / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D002648:Child; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects; D007223:Infant; D007676:Kidney Failure, Chronic / Q000601:surgery*; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D016030:Kidney Transplantation; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D059016:Tumor Microenvironment
D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; D014408:Biomarkers, Tumor; D007166:Immunosuppressive Agents; C069203:TFE3 protein, human
TFE3-positive RCC; pediatric renal transplant; renal cell carcinoma