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Skin Cancer01:30

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Skin cancer is a type of cancer that occurs when there is an abnormal growth of skin cells, usually triggered by damage to the DNA within the skin cells. It is primarily caused by exposure to ultraviolet (UV) radiation from the sun or artificial sources like tanning beds. Skin cancer is the most common type of cancer worldwide, and its incidence continues to rise.
Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
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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.

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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.

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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.