Primary retroperitoneal perivascular epithelioid cell neoplasm: A case report

Abstract

Abstract. Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumor, and primary retroperitoneal PEComa is rarer still. The present report discusses the case of a 51-year-old woman who was admitted to hospital following stiffness and discomfort in the upper right abdomen for longer than one month. Computed tomography identified a soft tissue mass in the rear right kidney, accompanied by clear cystic degeneration and bleeding. The borderline between the mass and the right kidney was not distinct. An enhanced scan revealed that the solid section of the mass was progressively enhanced. Following excision of the mass and the affected right kidney, the patient was pathologically diagnosed with retroperitoneal PEComa involving the right kidney. Short-term reexamination did not detect any recurrence.

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

“…Usually, PEC surrounds the blood vessels, arranging radially around the lumen, forming bunch- and web-shaped structures, while in a minority of cases, it has a focal association with the blood vessel walls. 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].…”

“…Since retroperitoneal PEComas cannot easily be differentiated from sarcomas and because half of them may develop a potentially malignant biological behaviour, radical surgical resection (usually organ sparing) constitutes the treatment of choice for localized disease[3,15,18,23,38,56]. Chemo-, immune- and/or radiotherapy have little efficacy[3,12,18,56,57].…”