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Clear Cell Sarcoma With Cutaneous Presentation in a 4-Year-Old Boy.
Clinton Westover1, Carlos Bacchi2, Alejandro A Gru1
1Department of Pathology, The University of Virginia, Charlottesville, VA; and.
The American Journal of Dermatopathology
|April 24, 2020
Summary
This study details the youngest known case of clear cell carcinoma involving the skin in a 4-year-old boy. Diagnostic analysis revealed specific cellular markers and EWSR1 gene rearrangement.
Area of Science:
- Oncology
- Dermatopathology
- Pediatric Oncology
Background:
- Clear cell carcinoma is a rare malignancy with diverse presentations.
- Cutaneous involvement of clear cell carcinoma is exceptionally uncommon, particularly in pediatric cases.
- Early diagnosis and characterization are crucial for effective management.
Observation:
- A 4-year-old boy presented with a painful, erythematous skin nodule on his left arm.
- Histopathological examination revealed bland spindle cells with a "floret-like" appearance.
- Immunohistochemistry showed positivity for S100 and SOX10, with negativity for other markers like CD34, desmin, SMA, HMB-45, CD1a, and CD163.
Findings:
- Fluorescence in situ hybridization confirmed EWSR1 gene rearrangement.
- The observed cellular morphology and marker profile were initially suggestive of giant cell fibroblastoma.
- The definitive diagnosis was cutaneous clear cell carcinoma, representing the youngest reported case.
Implications:
- This case expands the known spectrum of clear cell carcinoma presentation.
- Highlights the importance of comprehensive diagnostic workup in pediatric skin lesions.
- Contributes to understanding the molecular underpinnings (EWSR1 rearrangement) in rare pediatric malignancies.
