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Myxoid clear cell sarcoma.

You Chan Kim1, Daniel P Vandersteen, Hong-Geun Jung

  • 1Department of Dermatology, Ajou University School of Medicine, Suwon, Korea. maychan@ajou.ac.kr

The American Journal of Dermatopathology
|January 29, 2005
PubMed
Summary
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This study describes a rare myxoid variant of clear cell sarcoma, a soft-tissue tumor, found in a young adult. The tumor exhibited characteristic genetic alterations and distinct microscopic features, including significant mucin deposition.

Area of Science:

  • Oncology
  • Pathology
  • Genetics

Background:

  • Clear cell sarcoma (CCS) is a rare soft-tissue neoplasm typically affecting young adults, often in the extremities.
  • While historically linked to malignant melanoma due to melanin presence, CCS is now recognized as distinct, characterized by the specific t(12;22) translocation.
  • Myxoid malignant melanoma is a known variant, but a myxoid clear cell sarcoma has not been well-documented.

Observation:

  • A case of myxoid clear cell sarcoma is presented in a 22-year-old male with a tumor on the heel.
  • Histopathological examination revealed nests and fascicles of cells with clear to pale eosinophilic cytoplasm, separated by fibrous septa.
  • Tumor cells showed reactivity for S-100 protein, HMB-45, and MART-1. Cystic structures with mucin deposition and Ewing sarcoma gene rearrangement were noted.

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Findings:

  • The described tumor represents a rare myxoid variant of clear cell sarcoma.
  • Immunohistochemistry confirmed markers typical for CCS, while genetic analysis identified the characteristic Ewing sarcoma gene rearrangement.
  • Prominent mucin deposition within the tumor stroma and cystic lumens was a notable feature.

Implications:

  • This case expands the known morphologic spectrum of clear cell sarcoma.
  • Recognition of this myxoid variant is crucial for accurate diagnosis and appropriate patient management.
  • Further research may elucidate the specific behavior and treatment strategies for this subtype.