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BCOR-CCNB3 fusion-positive clear cell sarcoma of the kidney

Hyojeong Han1, Kelsey C Bertrand1, Kalyani R Patel2

  • 1Section of Hematology/Oncology, Department of Pediatrics, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.

Pediatric Blood & Cancer
|December 27, 2019
PubMed

Insights

Clear cell sarcoma of the kidney (CCSK) can feature a BCOR-CCNB3 gene fusion. This finding in pediatric renal tumors suggests a need for specific genetic testing in CCSK cases lacking other common alterations.

Area of Science:

  • Pediatric Oncology
  • Molecular Pathology
  • Genetics

Background:

  • Clear cell sarcoma of the kidney (CCSK) is a significant pediatric renal malignancy.
  • Common genetic alterations include BCOR-ITD and YWHAE-NUTM2B/E fusions.
  • Rare somatic alterations also occur in CCSK.
Keywords:
pediatric oncologyrenal tumorssolid tumors

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