Primitive neuroectodermal tumor of kidney and its rare affection for inferior vena cava

Abstract

Primitive neuroectodermal tumors (PNETs) are a group of small round cell malignancies of neural crest origin. Renal PNET (rPNET) is a rare entity affecting young adults and children with a poor prognosis, and involvement of inferior vena cava (IVC) is extremely uncommon. Nonspecific clinical features often preclude preoperative diagnosis which ultimately depends on microscopy and immunohistochemistry. The preferred treatment for rPENT is surgical resection associated with chemotherapy and radiotherapy. We report a case of rPNET in a 16-year-old male which was preoperatively thought to be of renal cell carcinoma and underwent radical nephrectomy with IVC venotomy. The case on histopathology study revealed features of malignant small blue round cell neoplasm and further immunohistochemical examination diagnosed the case as rPNET.