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Related Experiment Videos

[Clear cell sarcoma extending into the inferior vena cava]

N Ohtake1, A Shiono, K Okabe

  • 1Department of Urology, Gunma University, School of Medicine.

Nihon Hinyokika Gakkai Zasshi. the Japanese Journal of Urology
|July 1, 1995
PubMed
Summary

This case report details a rare pediatric kidney cancer, clear cell sarcoma of the kidney (CCSK), presenting with tumor thrombus. Despite aggressive treatment, the patient succumbed to metastatic disease.

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Area of Science:

  • Pediatric Oncology
  • Nephrology
  • Pathology

Background:

  • Wilms tumor is a common pediatric kidney cancer.
  • Clear cell sarcoma of the kidney (CCSK) is a rare subtype, comprising 4-5% of Wilms tumors.
  • Tumor thrombus extension into the inferior vena cava is a rare but serious complication.

Observation:

  • A 6-year-old boy presented with a right flank mass and hematuria.
  • MRI revealed Wilms tumor with inferior vena cava extension.
  • Pathology confirmed clear cell sarcoma of the kidney.

Findings:

  • The patient underwent radical nephrectomy, lymphadenectomy, and thrombectomy.
  • Post-operative treatment included chemotherapy (ACTD, VCR, DXR, CPM) and radiotherapy per NWTS-III protocol.

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  • The patient died due to bone metastases and pneumomycosis.

    • Implications:

    • This case highlights the aggressive nature of CCSK, even with multimodal therapy.
    • It underscores the importance of early diagnosis and management of rare kidney tumors in children.
    • This is the first reported case of CCSK with tumor thrombus in Japan, contributing valuable data to rare tumor research.