Xanthogranulomatous pyelonephritis in childhood: a rare but important clinical entity.

PURPOSE: Xanthogranulomatous pyelonephritis (XGPN) is extremely rare in children. The authors review their experience of this condition.

METHODS

Medical records were investigated to conduct a retrospective study of 4 patients with XGPN (3 boys, 1 girl; age range, 2 months to 7 years) at the authors' institute over the past 14 years.

RESULTS

Three of the 4 patients presented with fever of unknown origin and 1 with general fatigue. An abdominal mass was palpable in two cases at initial presentation. Although all patients had pyuria or hematuria, preoperative urine culture was positive in only 2 cases. Preoperative radiologic studies showed that 1 kidney was affected completely in 2 cases and affected partially in 2 cases. Preoperatively, the provisional diagnosis was XGPN in 3 cases, and Wilms' tumor in 1 case. Total nephrectomy was performed in 3 cases and enucleation in 1 case. XGPN was confirmed in all cases by histopathologic studies, but the underlying disease could be identified only in 1 case (cystinuria). All patients did well postoperatively and have had no further health problems over a mean follow-up period of 4.8 years.

CONCLUSIONS

XGPN should be considered when there is a history of recurrent or therapy-resistant pyelonephritis. Preoperative radiologic investigation is paramount for diagnosis, and nephrectomy is the treatment of choice, although partial resection or enucleation are adequate for partially affected kidneys.