Primitive Neuroectodermal Tumors of the Kidney

Abstract

Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) rarely occurs as a primary renal tumor. The disease affects young adults and children and has an aggressive course. The clinical presentation and imaging of these tumors are nonspecific, and they often present at an advanced stage. We present the clinical features, imaging, diagnosis, and treatment of 7 cases of renal PNET (4 men, 3 women; median age, 32 years). Common presenting symptoms were flank or abdominal pain and a mass in the abdomen. On imaging, a large heterogenous infiltrating renal mass with areas of calcification, hemorrhage, and necrosis and tumor thrombus can give a clue to the diagnosis of renal PNET. Immunohistochemistry and molecular studies are essential to confirm the diagnosis. The prognosis of renal ES/PNET is generally poor. Radical nephrectomy combined with chemotherapy and radiotherapy is the standard treatment for renal PNET. An early and accurate diagnosis is crucial for the proper management of these aggressive tumors.