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Intra-articular fibroma-like perivascular epithelioid tumor (PEComa) mimicking tenosynovial giant cell tumor, diffuse type

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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.

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Funding

NIH grant K24 DK-109940.

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Authors and Affiliations

  1. Division of Musculoskeletal Imaging and Intervention, Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Yawkey 6E, 55 Fruit Street, Boston, MA, 02114, USA

    Joel P. Harvey & Miriam A. Bredella

  2. Department of Pathology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA

    David I. Suster & G. Petur Nielsen

  3. Department of Orthopedics, Massachusetts General Hospital and Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA

    Kevin A. Raskin

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  1. Joel P. Harvey
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  2. David I. Suster
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  3. Kevin A. Raskin
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  4. G. Petur Nielsen
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  5. Miriam A. Bredella
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Correspondence to Miriam A. Bredella.

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The authors declare that they have no conflicts of interest.

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IRB approval was not required for this case report per guidelines of our institution’s IRB and no informed consent was required.

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Harvey, J.P., Suster, D.I., Raskin, K.A. et al. Intra-articular fibroma-like perivascular epithelioid tumor (PEComa) mimicking tenosynovial giant cell tumor, diffuse type. Skeletal Radiol 48, 965–969 (2019). https://doi.org/10.1007/s00256-018-3065-7

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  • DOI: https://doi.org/10.1007/s00256-018-3065-7

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