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Complexity of PEComas

Utpatel, K. ; Calvisi, D. F. ; Köhler, G. ; Kühnel, T. ; Niesel, A. ; Verloh, N. ; Vogelhuber, M. ; Neu, R. ; Hosten, N. ; Schildhaus, H.-U. ; Dietmaier, W. ; Evert, M.



Abstract

Perivascular epithelioid cell neoplasms (PEComas) are a family of mesenchymal neoplasms with features of both melanotic and smooth muscle differentiation. PEComa morphology is highly variable and encompasses epithelioid to spindle cells often with clear cytoplasm and prominent nucleoli. Molecularly, most PEComas are defined by a loss of function of the TSC1/TSC2 complex. Additionally, a distinct ...

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