Malignant perivascular epithelioid cell tumour ("PEComa") of soft tissue: a unique case |
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Authors: | Harris Gavin C McCulloch Thomas A Perks Graeme Fisher Cyril |
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Institution: | Canterbury Health Laboratories, Christchurch, New Zealand. |
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Abstract: | Tumours of perivascular epithelioid cells (PEComas) are being increasingly reported at visceral and somatic sites. Both benign and malignant variants have been identified, although clinical follow-up is often limited, which prevents meaningful predictions of behavior. We report the case of a malignant soft tissue PEComa with histologically confirmed regional lymph node metastases and radiologically confirmed pulmonary metastases. The light microscopic appearance and immunohistochemical profile (HMB-45, smooth muscle actin positive) and electron microscopic appearance support perivascular epithelioid cell differentiation. |
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