Girdled and Hemmed In -Perivascular Epithelioid Cell Tumour

Abstract

PREFACE Exceptional neoplasia with perivascular clear cell and epithelioid cell differentiation are diverse and constituted by angiomyolipoma, clear cell “sugar tumour” of pulmonary and extra pulmonary sites, clear cell myomelanocytic tumour offalciform ligament or ligamentum teres and lymphangioleiomyomatosis on account of distinctive histological manifestations. Variable genetic and malignant biological behaviour is exemplified within perivascular epithelioid cell tumours. Perivascular epithelioid cell tumour (PEComa) is a contemporary neoplasm of mesenchymal origin emerging within diverse sites while representing a classified group with distinctive histology and immune histochemical staining. The tumefaction is composed of epithelioid cells which demonstrate dual properties of smooth muscle and melanocytes with pertinent immune reactivity. Cellular component tends to aggregate around and circumscribe blood vessels, thus engendering the acronym “PEComa”. Perivascular epithelioid cell tumour was initially described by Bonetti et al in 1992 [1]. Zamboni et al in 1996 documented perivascular epithelioid cell tumour of the pancreas and denominated the neoplasm as “sugar tumour” on account of clear, glycogen rich, cytoplasm imbued within perivascular epithelioid cells. A normal counterpart of perivascular epithelioid cell tumour is absent [2].