Xanthogranulomatous pyelonephritis in an eight year old male child

INTRODUCTION AND IMPORTANCE: Xanthogranulomatous pyelonephritis is an extremely rare but known form of chronic pyelonephritis resulting from prolonged suppuration of the kidney. Pre-operatively, it may mimic renal tuberculosis or neoplastic lesions including renal cell carcinoma due to its vague clinical presentation, equivocal laboratory and radiological investigations. Due to its rarity and academic interest, herein we report such a rare case we recently encountered in our clinical practice. CASE PRESENTATION: An-eight-year old male child patient resented to our hospital with three months history of abdominal distension associated with progressive left flank pain. Preoperative investigations including CT-scan were suggestive of nephroblastoma with differential diagnosis of clear cell sarcoma. Radical nephrectomy was performed and histopathology of the specimen confirmed the diagnosis of Xanthogranulomatous pyelonephritis. The patient fared well postoperatively and he had no symptoms in the subsequent follow up visits. CLINICAL DISCUSSION: Xanthogranulomatous pyelonephritis is a rare, severe and atypical form of chronic pyelonephritis due to infection (E. coli, Proteus) or stones. The disease may resemble renal cell carcinoma preoperatively. Thus, high index of suspicion is necessary for preoperative diagnosis. CONCLUSION: Preoperative diagnosis of Xanthogranulomatous pyelonephritis may be a daunting task related to the rarity of its presentation. Thus, scrupulous histopathological evaluation is essential for the definitive diagnosis. Radical nephrectomy is the mainstay treatment of choice especially in diffuse cases.