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Case Report Open Access

Renal clear cell sarcoma in children: A case report

  • Authors:
    • Lingfei Li
    • Hui Yang
    • Fang Li
    • Ning Zhou
    • Ying Luo
    • Hailan Ma
    • Zhengfu Wang
    • Liren Jian
    • Ling Dang
    • Hongyan Xiao
  • View Affiliations / Copyright

    Affiliations: Department of Pathology, Peking University First Hospital Ningxia Women and Children's Hospital (Ningxia Hui Autonomous Region Maternal and Child Health Hospital), Yinchuan, Ningxia 75004, P.R. China, School of Nursing, Ningxia Medical University, Yinchuan, Ningxia 75004, P.R. China
    Copyright: © Li et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
  • Article Number: 86
    |
    Published online on: May 14, 2026
       https://doi.org/10.3892/br.2026.2159
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Abstract

Clear cell sarcoma of the kidney (CCSK), a rare renal interstitial tumor in children, poses a notable threat to the physical and mental health of children because of its highly aggressive nature, tendency to compress surrounding tissue and potential for metastasis. The present study reports two cases of pediatric CCSK treated at the Peking University First Hospital Ningxia Women and Children's Hospital (Ningxia Hui Autonomous Region Maternal and Child Health Hospital, Ningxia, China; July 2024‑2025.7). Both patients were female infants aged 1 year. Upon initial admission, they were misdiagnosed with nephroblastoma and received radical nephrectomy and vincristine chemotherapy. Pathological consultation and advanced molecular sequencing identified specific expression of the BCL6 corepressor gene in patient tumor tissue, leading to a definitive diagnosis of CCSK. Furthermore, the present study aimed to provide an overview of the aberrant genes associated with CCSK to provide a useful reference for future research on the pathogenesis of CCSK and improvement of molecular pathology.
View Figures

Figure 1

Clinical information of patient 1. (A)
Gray-scale ultrasound of the transverse section of the right kidney
showed a solid mass occupying the lower-middle part of the kidney,
protruding towards the abdominal cavity. (B) Color Doppler flow
Imaging shows abundant blood flow signals. Repeated exploration
suggested branches of the renal artery extending into CCSK. (C)
Patient 1 was diagnosed with classical CCSK. Tumor cells (arrows)
are separated by fibrovascular stroma distributed in a dendritic
pattern. (D) Tumor cells (arrows) are arranged in a vortex pattern.
(E) Patient was diagnosed with classical CCSK. Widened vascular
fibrous septa were observed. (F) BCL6 corepressor (positive), (G)
transducin-like enhancer of Split 1 (positive), (H) B-cell lymphoma
2 (positive), (I) Cyclin D1 (positive), (J) Wilms' Tumor 1
(negative) immunohistochemistry. Magnification, x20. CCSK, clear
cell sarcoma of the kidney.

Figure 2

Clinical information of Patient 2. (A)
Prone position transverse section imaging showed a solid mass
~9.7x8.5x8.0 cm in size at the middle and lower pole of the right
kidney. (B) Color Doppler flow imaging showed rich blood flow
signals at the margin. (C) Tumor cells had large nuclei with
transparent cytoplasm, and the blood vessels were irregularly
distributed. (D) Coarse fibrovascular tissue and sheet-like tumor
tissue were observed. (E) Epithelioid tumor cells were arranged in
cord-like and small nested patterns. (F) BCL6 corepressor
(positive), (G) transducin-Like Enhancer of Split 1 (positive), (H)
B-cell lymphoma 2 (positive) (I) Cyclin D1 (positive), (J) Wilms'
tumor 1 (negative) immunohistochemistry. Magnification, x20.

Figure 3

DNA sequencing. Sequencing map of (A)
BCOR and (B) TSHR genes in patient 1 tumor tissue. (C) Sequencing
map of FLT1 genes in patient 2 tumor tissue. (D) Mutated genes in
patient 2. BCOR, BCL6 Co-repressor; TSHR, Thyrotropin Receptor;
FLT1, Fms-like Tyrosine Kinase 1; ANKRD, ankyrin Repeat Domain;
ARID, AT-Rich Interaction Domain; BRD, Bromodomain-containing; MGA,
managing General Agent; MITF, Microphthalmia-Associated
Transcription Factor; MST, Mitogen-Activated Protein Kinase Kinase
Kinase; NOTCH, Notch Receptor; PRKDC, Protein Kinase,
DNA-Activated, Catalytic Polypeptide; SMAD3, SMAD Family Member 3;
TBX, T-Box Transcription Factor; TP53BP1, Tumor Protein P53 Binding
Protein 1.

Figure 4

Clinical information of patients 1
year after the surgery. (A) CDFI of patient 1 showed no remarkable
findings in the right renal region. (B) while the left kidney
demonstrated normal morphology. (C) CDFI of patient 2 showed no
localized masses or sonolucent areas in the right renal region, (D)
though splenomegaly was noted. CDFI, color Doppler Flow
Imaging).
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Copy and paste a formatted citation
Spandidos Publications style
Li L, Yang H, Li F, Zhou N, Luo Y, Ma H, Wang Z, Jian L, Dang L, Xiao H, Xiao H, et al: Renal clear cell sarcoma in children: A case report. Biomed Rep 25: 86, 2026.
APA
Li, L., Yang, H., Li, F., Zhou, N., Luo, Y., Ma, H. ... Xiao, H. (2026). Renal clear cell sarcoma in children: A case report. Biomedical Reports, 25, 86. https://doi.org/10.3892/br.2026.2159
MLA
Li, L., Yang, H., Li, F., Zhou, N., Luo, Y., Ma, H., Wang, Z., Jian, L., Dang, L., Xiao, H."Renal clear cell sarcoma in children: A case report". Biomedical Reports 25.1 (2026): 86.
Chicago
Li, L., Yang, H., Li, F., Zhou, N., Luo, Y., Ma, H., Wang, Z., Jian, L., Dang, L., Xiao, H."Renal clear cell sarcoma in children: A case report". Biomedical Reports 25, no. 1 (2026): 86. https://doi.org/10.3892/br.2026.2159
Copy and paste a formatted citation
x
Spandidos Publications style
Li L, Yang H, Li F, Zhou N, Luo Y, Ma H, Wang Z, Jian L, Dang L, Xiao H, Xiao H, et al: Renal clear cell sarcoma in children: A case report. Biomed Rep 25: 86, 2026.
APA
Li, L., Yang, H., Li, F., Zhou, N., Luo, Y., Ma, H. ... Xiao, H. (2026). Renal clear cell sarcoma in children: A case report. Biomedical Reports, 25, 86. https://doi.org/10.3892/br.2026.2159
MLA
Li, L., Yang, H., Li, F., Zhou, N., Luo, Y., Ma, H., Wang, Z., Jian, L., Dang, L., Xiao, H."Renal clear cell sarcoma in children: A case report". Biomedical Reports 25.1 (2026): 86.
Chicago
Li, L., Yang, H., Li, F., Zhou, N., Luo, Y., Ma, H., Wang, Z., Jian, L., Dang, L., Xiao, H."Renal clear cell sarcoma in children: A case report". Biomedical Reports 25, no. 1 (2026): 86. https://doi.org/10.3892/br.2026.2159
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