Malignant TFE3-rearranged perivascular epithelioid cell neoplasm (PEComa) presenting as a subcutaneous mass.
case_report · Level V
Where this comes from
- Record sourced from PubMed, PMID 26385848.
- Also identified by DOI 10.1111/bjd.14173.
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Abstract
Perivascular epithelioid cell neoplasms (PEComas) are a group of mesenchymal tumours with concurrent melanocytic and myogenic differentiation. Although many cases are sporadic, PEComas can be associated with tuberous sclerosis. A distinct subset of deep-seated PEComas has been shown to carry TFE3 fusions. To our knowledge, this is the first reported case of primary subcutaneous malignant PEComa with molecular confirmation of TFE3 gene rearrangement.
Medical subject headings
- Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
- Muscle Neoplasms
- Perivascular Epithelioid Cell Neoplasms