Malignant perivascular epithelioid cell tumour of the fibula: a report and a short review of bone perivascular epithelioid cell tumour

Abstract

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.

Perirenal perivascular epithelioid cell tumor (PEComa) coexisting with other malignancies: a case report

“…As these tumors are mesenchymal of uncertain lineage, in our study cytokeratin AE1/AE3, cytokeratin 20 and cytokeratin 7 were entirely negative, whereas we noted positivity for vimentin, and focally for desmin. Unusually, the presence of focal cytokeratin expression in malignant PEComa of the fibula was noted [8]. In the present case immunohistochemical staining confirmed the melanocytic and myoid differentiation of the tumor cells.…”

“…To date, the extremely rare perivascular epithelioid cell tumor has been described in various anatomic locations: in lungs [1], pancreas [2], uterus [6,7], bone [8], retroperitoneum [9], ovary [10], liver [11], cutis [12], transverse colon [4] and in intraorbital location [3]. As was mentioned above, close perirenal location concerns only three cases of PEComas [5,13,14].…”

Primary perivascular epithelioid cell tumor (PEComa) in bone: A review of the literature and a case arising in the humerus with multiple metastases

“…A total of sixteen studies with nineteen unique case reports with pathologically confirmed primary bone PEComas were available via PubMed, Embase and Web of Science ( Fig. 5 ) [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] . Taking current case into account, ten males and ten females with a median age of 34 years, and a range from 3 to 93 years, were identified.…”

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

“…The cells lie adjacent to thin-walled blood vessels. Other findings common in more aggressive cases are high mitotic activity, nuclear pleomorphism, multinucleated giant cells, stromal hyalinization and focal necrosis ( 7 , 8 , 10 , 11 , 13 , 14 ). PEComas typically exhibit reactivity to both melanocytic (e.g., HMB45, melan-A) and myogenic (e.g., smooth muscle actin, desmin) markers, but not to epithelial markers.…”