Renal primitive neuroectodermal tumor. The first case series from Syria.

Abstract

Primitive neuroectodermal tumor (PNET) mainly arises from soft tissues of the extremities such as humerus, femur, C tibia. It rarely arises from kidney; less than 200 cases have been reported in the literature. The clinical presentation and radiography findings are not specific. Here we first report two cases of renal primitive neuroectodermal tumor in Syria.the first patient was 26-year-old- female that presented to urology clinic complaining of right flank pain. Ultrasonography of the abdomen showed a large mixed heterogeneous mass in the right kidney with no hemorrhage or calcification and MSCT of abdomen and pelvis demonstrate a mixed well-demarcated heterogeneous mass measuring (74*117) mm in the right kidney right radical nephrectomy was performed.The second patient 19-year-old-male presented with left flank pain. Ultrasonography of the abdomen showed mixed large mass involving the left kidney, with unmarked border. The CT of the abdomen and pelvis demonstrating a (30*110*90) mm left renal mass and periaortic lymphadenopathy measuring (45*28) mm. The patient underwent Left radical nephrectomy with periaortic lymphadenectomy dissection. The final diagnosis for both cases was Renal PNET based on microscopic and immunohistochemistry examination.In patient with suspected renal mass in the radiographic images, the diagnosis of renal primitive neuroectodermal tumor should be kept in the mind despite its rarity. The final diagnosis is done by histopathological study in association with immunohistochemical examination.