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Erschienen in:
11.09.2018 | Case Report
Intra-articular fibroma-like perivascular epithelioid tumor (PEComa) mimicking tenosynovial giant cell tumor, diffuse type
Erschienen in: Skeletal Radiology | Ausgabe 6/2019
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Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms composed of perivascular epithelioid cells that are immunoreactive for both melanocytic and myogenic markers. Recently, a fibroma-like PEComa associated with tuberous sclerosis complex (TSC) has been identified. We describe the first intra-articular case of a fibroma-like PEComa in a 44-year-old man who presented with a hypointense intra-articular knee mass, which was mistaken for tenosynovial giant cell tumor, diffuse type. This case report demonstrates that fibroma-like PEComa should be included in the extended differential diagnosis of intra-articular soft tissue masses. In addition, given their strong association with TSC mutations, a diagnosis of fibroma-like PEComa should trigger an evaluation for TSC.