Retroperitoneal Sclerosing Perivascular Epithelioid Cell Tumour
VYK To, JPK Tsang, TW Yeung, MK Yuen
Hong Kong J Radiol 2015;18:51-6
DOI: 10.12809/hkjr1414242
Perivascular epithelioid cell tumour is a relatively new entity with rising incidence. This is a rare mesenchymal neoplasm that can occur in various organs and is characterised by proliferation of perivascular cells and expression of myomelanocytic markers. Here, we present an asymptomatic 52-year-old female patient with an incidental radiological finding of a large retroperitoneal mass, histologically and immunohistochemically proven to be a sclerosing type of perivascular epithelioid cell tumour. The mass showed typical morphological and microscopic features consistent with those described in the current literature. However, it had computed tomography findings of neovascularisation and hyper-vascularity, not often documented in previous case reports of the sclerosing type of tumours. Literature review, using PubMed, of intraperitoneal / retroperitoneal type of tumours, and specifically the sclerosing type, was performed. To the best of our knowledge, less than 20 sclerosing perivascular epithelioid cell tumours have been reported and few describe the associated radiological features.
中文摘要
腹膜後硬化型血管周上皮樣細胞瘤
杜婉筠、曾佩琪、楊子慧、袁銘強
血管周上皮樣細胞瘤是一種較新的疾病，發病率呈上升趨勢。它是一種罕見的間葉細胞瘤，可以發生在各種器官內，組織學特徵是血管周圍細胞增生和細胞肌黑色素標記物的表達。本文報告一名52歲女性於影像檢查偶然發現一個腹膜後大腫塊，組織學上和免疫組織化學上均顯示為血管周圍上皮樣細胞瘤的一種硬化型。腫塊顯示典型的形態學和鏡下特徵，與以往文獻描述的一致。然而，電腦斷層掃描結果顯示有新生血管和富血管性，這種情況在該腫瘤硬化型的病例報告並不常見。我們利用PubMed進行腹膜內／腹膜後該腫瘤（尤其是硬化型）的文獻回顧。據我們所知，文獻中有關硬化性血管周上皮樣細胞瘤的病例不足二十個，且其中很少相關影像學特徵的描述。