Malignant perivascular epithelioid cell tumour ("PEComa") of soft tissue: a unique case.
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.
Harris
Gavin C
GC
Canterbury Health Laboratories, Christchurch, New Zealand.
McCulloch
Thomas A
TA
Perks
Graeme
G
Fisher
Cyril
C
D000368:Aged; D002277:Carcinoma / Q000378:metabolism / Q000473:pathology / Q000556:secondary*; D015622:Epithelioid Cells / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000556:secondary; D008207:Lymphatic Metastasis / Q000473:pathology; D008297:Male; D046529:Microscopy, Electron, Transmission; D012983:Soft Tissue Neoplasms / Q000378:metabolism / Q000473:pathology*