Six-year disease-free survival after chemotherapy treatment for metastatic renal peripheral neuroectodermal tumor

Peripheral neuroectodermal tumors (PNET) of the kidney are extremely rare. They are often diagnosed at a late stage due to their nonspecific clinical presentation. Treatment of patients with metastases is based on palliative chemotherapy. We here report a case of PNET of the kidney with sudden onset of metastases to the lymph nodes and to the skin. The patient showed good clinical and radiological response and experienced progression-free survival at 6 years after polychemotherapy with vincristine, doxorubicin and cyclophosphamide.