Citations 1
MalignantTFE3-rearranged perivascular epithelioid cell neoplasm (PEComa) presenting as a subcutaneous mass
Shon, Wonwoo , Kim, J, Sukov, William R. , Reith, John D.
British Journal of Dermatology | September 19, 2015 | Volume: 174 | Issue: 3 | Pages: 617-620
SummaryPerivascular 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.