Perivascular Epithelioid Cell Tumor of the Kidney: A Rare Case Report

Abstract

IntroductionPerivascular epithelioid cell tumors (PEComas) of the kidney are a group of tumor family covering classical angiomyolipoma (AML), microscopic AML (microhamartoma), intraglomerular lesions, cystic AML, epithelioid AML, oncocytoma-like AML and lymphangiomyomatosis of the renal sinus. The World Health Organization (WHO) defines PEComa as a "mesenchymal tumor that contains perivascular epithelioid cells presenting histological and immunohistochemical differences". While classical AML is benign, epithelioid AMLs are defined as potentially malign mesenchymal lesions for their capacity to develop local recurrence and metastasis (1,2,3). Surgical treatment may be required in AML for symptoms such as hemorrhage and pain, or for the risk of malignancy in tumors that cannot be differentiated from AML in the case of PEComa and certain variants of AML (3,4,5).This case report aimed to present a patient who undergone retroperitoneoscopic partial nephrectomy due to a renal mass and diagnosed with PEComa after pathological examination. Case PresentationWe planned magnetic resonance imaging (MRI) in a 25-year-old male patient, who was admitted to our clinic due to left-sided flank pain, with no pathological finding during the examination and laboratory tests, however, with a suspicious mass lesion located at the inferior pole of the left kidney detected during ultrasonographic examination. It was decided to perform surgical excision after detection of a 4 cm tumoral lesion in the inferior pole of the left kidney on MRI (Figure 1). We performed left retroperitoneoscopic partial nephrectomy in our clinic. The total operative time was 90 minutes with the warm ischemia time Böbreğin perivasküler epiteloid hücre tümörleri nadir görülen histolojik ve immünohistokimyasal olarak farklı perivasküler epiteloid hücrelerden oluşan mezenkimal tümörlerdir. Çoğunluğu benign tümörlerdir; fakat malign olanları agresif seyredebilir ve metastaza yol açabilir. Standart tedavi yöntemi ise cerrahi eksizyondur. Olgumuzda 25 yaşında sol böbrek alt polünde 4 cm kitle saptanan ve cerrahi olarak eksize ettiğimiz hastayı sunmayı amaçladık. Anahtar Kelimeler: Böbrek tümörü, perivasküler epiteloid hücre tümörü, parsiyel nefrektomi Perivascular epithelioid cell tumors are rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. They are mostly benign, however, malignant tumors with aggressive behavior and distant metastasis can also occur. The standard treatment is surgical excision. Here, we report a case of a 25-year-old male with a 4 cm-mass located in the inferior pole of the left kidney, treated by surgical excision.