Primitive neuroectodermal tumor of the kidney with vena caval tumor thrombus: diagnosis and management

Primitive neuroectodermal tumors (PNET) of the kidney are rare and highly aggressive malignancies. We report a case of 28-year-old male with PNET of the kidney with inferior vena caval thrombus. Immunohistochemistry revealed strong positivity for CD99 and weak positivity for vimentin. Neuron-specific enolase (NSE), chromogranin and cytokeratin were negative. Patient underwent nephrectomy and six cycles of polychemotherapy the patient was in partial remission. He underwent two further cycles of high dose chemotherapy and died 9 months after diagnosis due to liver metastases. The diagnosis of renal PNET must be considered in young patients with renal neoplasm, particularly those with advanced disease at presentation. Achieving exact diagnosis has important clinical consequences.