A Case of Malignant Peripheral Nerve Sheath Tumour of the Kidney

Introduction: Malignant peripheral nerve sheath tumor (MPNST) of the kidney is an uncommon neoplasm. We present a rare case MPNST successfully managed by surgical intervention. Case Presentation: A 23-year-old male patient presented with dull aching pain in right flank associated hematuria. On examination, abdominal mass firm consistency occupied lumbar quadrant fullness renal angle. Contrast-enhanced computed tomography (CECT) showed heterogeneously hypoenhancing lobulated (9.2 × 6.4 7.6 cm) arising exophytically from anterior cortex middle and lower poles necrosis. It was compressing pelvicalyceal system causing mild hydronephrosis. Kidney function tests revealed normal results. Urine cytology for malignant cells negative. The underwent open radical nephrectomy. Renal adhered to ascending colon duodenum abutting liver. histopathological report suggested tumors margins microscopically clear cells. S-100 Vimentin were stained positively. has been under regular follow-up (every three months) last year. Conclusions: If feasible, wide local excision considered preferred approach, as prognosis mainly depends on completeness resection.