Primary malignant perivascular epithelioid cell neoplasm (PEComa) of the bone mimicking granular cell tumor in core biopsy: A case report and literature review

Sadigh,Sam; Shah,Preya; Weber,Kristy; Sebro,Ronnie; Zhang,Paul J.

doi:10.3892/ol.2017.7662

Primary malignant perivascular epithelioid cell neoplasm (PEComa) of the bone mimicking granular cell tumor in core biopsy: A case report and literature review

Authors:
- Sam Sadigh
- Preya Shah
- Kristy Weber
- Ronnie Sebro
- Paul J. Zhang
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Affiliations: Department of Pathology, Hospital of The University of Pennsylvania, Philadelphia, PA 19104, USA, Department of Radiology, Hospital of The University of Pennsylvania, Philadelphia, PA 19104, USA, Department of Orthopaedic Surgery, Hospital of The University of Pennsylvania, Philadelphia, PA 19104, USA
Published online on: December 20, 2017 https://doi.org/10.3892/ol.2017.7662
Pages: 2946-2952
Copyright: © Sadigh et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

The present study investigated the case of a 46‑year‑old female with primary malignant perivascular epithelioid cell neoplasm (PEComa) of the femur. The patient presented with a 5‑month history of right distal thigh pain following trauma. Radiographs of the right distal femur revealed a mixed lytic and sclerotic lesion with subtle areas of cortical destruction and soft tissue extension, consistent with an aggressive tumor. A core biopsy revealed an epithelioid tumor with granular cell features, but a definitive diagnosis could not be made. Due to the aggressive features on radiologic evaluation, the patient underwent a resection of the distal femur and reconstruction with a distal femoral megaprosthesis and hinged knee replacement. The post‑resection pathology led to a final diagnosis of primary bone PEComa, with histologic features including epithelioid, granular cell and spindled cell morphologies and biphasic immunoreactivity for melanocytic and smooth muscle markers. The large tumor size (>5 cm), rapid mitotic rate, infiltrative growth pattern, high nuclear grade and cellularity, and the presence of necrosis rendered this a malignant PEComa. The present study discussed the case, including radiographic (radiographs, magnetic resonance imaging and positron emission tomography scans) and histologic appearance and a literature review.

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