Peripheral neuroectodermal tumors (PNET), or Ewing sarcoma, are tumors that generally develop in bone; extraskeletal locations are rare. Renal PNETs are rare and are characterized by an aggressive clinical course and a poor prognosis. We report the case of a young patient who presented with abdominal and lumbar pain with a palpable abdominal mass. The imaging was in favor of a huge renal tumor, and the histological analysis allowed the diagnosis of a renal PNET. The therapeutic attitude was multimodal, including surgery and chemotherapy, allowing complete remission and a favorable outcome.

Ewing sarcoma

Renal PNET

Nephrectomy

Chemotherapy

© 2024 The Authors. Published by Elsevier Inc.