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  • Title: Malignant perivascular epithelioid cell tumour ("PEComa") of soft tissue: a unique case.
    Author: Harris GC, McCulloch TA, Perks G, Fisher C.
    Journal: Am J Surg Pathol; 2004 Dec; 28(12):1655-8. PubMed ID: 15577688.
    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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