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