PMID- 37528880
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230803
IS  - 2296-2360 (Print)
IS  - 2296-2360 (Electronic)
IS  - 2296-2360 (Linking)
VI  - 11
DP  - 2023
TI  - MiT family translocation renal cell carcinoma with retroperitoneal metastasis in 
      childhood: a case report.
PG  - 1141223
LID - 10.3389/fped.2023.1141223 [doi]
LID - 1141223
AB  - RCC accounts for only 0.1%-0.3% of all kidney tumors and 2%-6% of malignant 
      kidney tumors in children. Accounting for approximately one-third of the total 
      number of cases in children and adolescents with RCC, Xp11.2 tRCC is the most 
      common subtype of the MiT family translocation renal cell carcinoma, which is a 
      group of rare childhood and adult tumors, characterized by recurrent gene 
      rearrangements of TFE3. Here we report a rare case of a 6-year-old male patient 
      with MiT family translocation renal cell carcinoma (MiTF tRCC) where the patient 
      developed retroperitoneal metastasis. The patient underwent partial nephrectomy 
      (PN), radical nephrectomy (RN), abdominal lymph node resection, and intestinal 
      adhesion lysis. Microscopically, we detected focal and nest clump-shaped clusters 
      of tumor cells whose cytoplasm was bright and clear. Immunohistochemistry (IHC) 
      showed tumor cells diffusely expressed TFE3, and fluorescence in situ 
      hybridization (FISH) demonstrated disruption of the TFE3 locus, confirming the 
      diagnosis of Xp11.2 tRCC, the most common subtype of MiTF tRCC. Eventually, the 
      patient obtained a good therapeutic result. This case can provide a good 
      reference and guidance for pediatric urologists and oncologists to recognize and 
      diagnose rare renal cell carcinoma in children.
CI  - (c) 2023 Yang, Ma, Dai and Dong.
FAU - Yang, Kaihang
AU  - Yang K
AD  - Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, 
      Children's Hospital of Fudan University, Shanghai, China.
FAU - Ma, Yuhao
AU  - Ma Y
AD  - Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, 
      Children's Hospital of Fudan University, Shanghai, China.
FAU - Dai, Shuyang
AU  - Dai S
AD  - Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, 
      Children's Hospital of Fudan University, Shanghai, China.
FAU - Dong, Rui
AU  - Dong R
AD  - Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, 
      Children's Hospital of Fudan University, Shanghai, China.
LA  - eng
PT  - Case Reports
DEP - 20230717
PL  - Switzerland
TA  - Front Pediatr
JT  - Frontiers in pediatrics
JID - 101615492
PMC - PMC10388245
OTO - NOTNLM
OT  - MiT family translocation renal cell carcinoma
OT  - TFE3
OT  - Xp11.2 translocation
OT  - partial nephrectomy (PN)
OT  - retroperitoneal metastasis
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2023/08/02 06:42
MHDA- 2023/08/02 06:43
PMCR- 2023/07/17
CRDT- 2023/08/02 03:45
PHST- 2023/01/10 00:00 [received]
PHST- 2023/06/30 00:00 [accepted]
PHST- 2023/08/02 06:43 [medline]
PHST- 2023/08/02 06:42 [pubmed]
PHST- 2023/08/02 03:45 [entrez]
PHST- 2023/07/17 00:00 [pmc-release]
AID - 10.3389/fped.2023.1141223 [doi]
PST - epublish
SO  - Front Pediatr. 2023 Jul 17;11:1141223. doi: 10.3389/fped.2023.1141223. 
      eCollection 2023.