Malignant perivascular epithelioid cell tumour of the fibula: a report and a short review of bone perivascular epithelioid cell tumour.
Perivascular epithelioid cell tumour (PEComa) is a term applied to a family of mesenchymal tumours composed of varying proportions of spindle and epithelioid cell components associated with HMB-45 expression. PEComa rarely arises in the soft tissue, visceral organs, skin and bone. This report details an instance when a purely epithelioid PEComa arose from the right fibula of a 52-year-old Chinese woman without features of tuberous sclerosis complex. The excision specimen disclosed an epithelioid tumour with a nested pattern associated with areas of nuclear pleomorphism, mitotic activity, necrosis and vascular invasion in addition to HMB-45 expression on immunohistochemistry. To the best of the authors' knowledge, this represents the first case of a histologically malignant PEComa of the bone. A short review of primary bone PEComas and potential problems in diagnosis is presented.
Lian
D W Q
DW
Department of Pathology, Tan Tock Seng Hospital, Singapore.
Chuah
K L
KL
Cheng
M H W
MH
Yap
W M
WM
D001859:Bone Neoplasms / Q000473:pathology* / Q000601:surgery; D002277:Carcinoma / Q000473:pathology* / Q000601:surgery; D015622:Epithelioid Cells / Q000473:pathology; D005260:Female; D005360:Fibula / Q000473:pathology*; D006801:Humans; D008854:Microscopy, Electron; D008875:Middle Aged