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- Title
Intra-articular fibroma-like perivascular epithelioid tumor (PEComa) mimicking tenosynovial giant cell tumor, diffuse type.
- Authors
Harvey, Joel P.; Suster, David I.; Raskin, Kevin A.; Nielsen, G. Petur; Bredella, Miriam A.
- 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.
- Subjects
TUBEROUS sclerosis; GIANT cell tumors; FIBROMAS; TUMORS; DIFFERENTIAL diagnosis; MAGNETIC resonance imaging
- Publication
Skeletal Radiology, 2019, Vol 48, Issue 6, p965
- ISSN
0364-2348
- Publication type
journal article
- DOI
10.1007/s00256-018-3065-7