Published in:
01-06-2019 | Case Report
Intra-articular fibroma-like perivascular epithelioid tumor (PEComa) mimicking tenosynovial giant cell tumor, diffuse type
Authors:
Joel P. Harvey, David I. Suster, Kevin A. Raskin, G. Petur Nielsen, Miriam A. Bredella
Published in:
Skeletal Radiology
|
Issue 6/2019
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Abstract
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.