Retroperitoneal Sclerosing Perivascular Epithelioid Cell Tumour

Abstract

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.

Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature

“…In all cases, PEC replaces the normal smooth muscle and collagen in the muscular wall of the vessel[17,38,41]. A sclerosing variant of PEComa with predominant sclerotic and hyalinized stroma has also been reported[17,25,27,28,40].…”

Recurrence of retroperitoneal localized perivascular epithelioid cell tumor two years after initial diagnosis: case report

“…To the best of our knowledge, only 20 cases of retroperitoneal PEComa have been reported in the literature so far, 1 , 6 , 7 , 8 , 9 , 10 some of them commented below. Our case is the only one in which there was local recurrence and pulmonary metastasis, two years after the initial diagnosis of benign PEComa.…”