Cystic nephroma and localized renal cystic disease in children: diagnostic clues and management.

BACKGROUND AND PURPOSE

Cystic nephroma (CN) and localized renal cystic disease (LRCD) are rare benign renal entities that can occur in children. A retrospective evaluation of the experience with these conditions was performed to emphasize the importance of differential diagnosis, recognition, and selection of the proper mode of treatment.

MATERIALS AND METHODS

The medical records of patients treated for CN (n = 4) and LRCD (n = 1) between 1980 and 2007 were retrospectively evaluated. The age, sex, family history of renal cystic disease, presenting symptoms, physical examination findings, diagnostic radiologic imaging used, operative findings, details of the surgical procedure, histopathologic features, and outcomes were evaluated.

RESULTS

There were 4 cases of CN and 1 case of LRCD without a positive family history. The age and sex of patients with CN were two 1-year-old girls and two 1.5-year-old boys. The patient with LRCD was a 4.5-year-old girl. The presenting symptoms were palpable mass in patients with CN and urinary tract infection in the patient with LRCD without impairment of renal function. Intravenous pyelography (n = 2), abdominal ultrasonography (n = 4), and computed tomography (n = 4) were used. All patients underwent an operation. Nephrectomy was performed in all 4 CN cases, and total excision of the cyst was performed in the LRCD case.

CONCLUSION

Pediatric surgeons should be familiar with the clinical, radiologic, surgical, and histopathologic characteristics of CN and LRCD. The presumptive diagnosis of CN prompts surgical intervention. Enucleation or partial nephrectomy is the treatment of choice in suitable cases. Nephrectomy should be reserved for CN, which totally replaces the parenchyma. Conservative treatment with follow-up imaging may be considered in selected cases of LRCD that have characteristic computed tomographic criteria. Operative intervention is indicated in symptomatic cases and should be limited to simple excision of the cysts.