Renal Clear Cell Sarcoma - Anaplastic Variant: A Rare Entity

Area of Science:

• Pediatric Oncology
• Renal Pathology
• Cancer Diagnostics

Background:

• Clear Cell Sarcoma of Kidney (CCSK) is a rare renal tumor known for aggressive behavior and metastasis.
• Anaplastic CCSK is a morphologic subtype associated with a poorer prognosis.

Observation:

• A 5-year-old boy presented with abdominal mass and pain, initially suspected as Wilms tumor.
• CT scan revealed a large right kidney mass.
• Diagnosis of anaplastic CCSK was confirmed post-nephrectomy via Immunohistochemistry (IHC) after excluding differentials.

Findings:

• Morphologic diversity of CCSK presents diagnostic challenges.
• Immunohistochemistry (IHC) is essential for definitive diagnosis of anaplastic CCSK.
• Accurate diagnosis is critical to avoid treatment delays.

Implications:

• This case highlights the diagnostic difficulties in rare pediatric renal tumors.
• Emphasizes the indispensable role of IHC in confirming anaplastic CCSK.
• Timely and accurate diagnosis ensures appropriate management and improves patient outcomes.