Beyond Bones: Exploring Primitive Neuroectodermal Tumor of Kidney

A primitive neuroectodermal tumor (PNET), classified as a malignancy of the Ewing's sarcoma family of tumors, is typically observed in bones or soft tissue among adolescents and young adults. However, its occurrence outside the skeletal system (extra-osseous location), particularly within visceral organs, is infrequent. Renal PNET is exceptionally uncommon and exhibits an exceedingly aggressive biological behavior, leading to a dismal prognosis as compared to conventional renal cell carcinoma. In this report, we present the case of a 28-year-old adult male patient diagnosed with renal PNET on ultrasound-guided biopsy, who initially presented with left flank pain and recent onset of hematuria within a brief timeframe. Left radical nephrectomy followed by postoperative VDC-IE (combined vincristine, doxorubicin, and cyclophosphamide followed by another combination of ifosfamide and etoposide) chemotherapy was given to the patient. This case serves as a reminder to nephrologists, medical oncologists, and pathologists that in adolescents and young adults presenting with suspicious renal masses, a diagnosis of renal PNET should always be considered. Timely surgical intervention, coupled with chemotherapy, is essential for optimal therapeutic management and improved prognosis in cases of such rare and aggressive malignancies.