MPNSTs are rare in childhood and have an unfavorable prognosis. Whereas chemotherapy and/or radiotherapy had no promising effect on overall survival rates, complete resection seems to be the most effective treatment for MPNSTs. Further multicenter studies on the role of chemotherapy and/or radiotherapy for MPNSTs are warranted.

The purpose of this study was to identify the prognostic significance of pulmonary and liver involvements present at diagnosis in children with Wilms' tumor. From 1971 to 2002, 57 patients with stage IV Wilms' tumor were diagnosed, treated, and followed in the authors' institution. Metastases were detected by chest radiography, abdominal ultrasonography, and/or thoracoabdominal computed tomography. Nephrectomy with tumor resection was performed and appropriate chemotherapy was given to all the patients. The Kaplan-Meier survival method was used to calculate survival. The log-rank test was used to compare the groups with respect to survival. The age range was 1.25 to 15 years (median 4 years). Thirty-seven patients had only lung metastases, 12 had only liver metastases, 6 had both lung and liver metastases, 1 had adrenal gland metastases, and 1 had other site metastases. The overall survival rate in the whole group was 37.1%. The overall survival rates for lung and liver metastases were 50.2% and 16.6%, respectively. The overall survival rates were significantly different between the two groups (P = 0.005). Only one patient survived in the group with liver involvement. Liver involvement at diagnosis indicates a worse prognosis than lung involvement. Wilms' tumor patients with liver metastases should be treated with more intensive regimens than those with lung metastases.

The survival rates in children with local disease are comparable with the results of developed countries; however, the results in children with advanced disease are still not satisfactory. To improve the outcome, especially in children with advanced disease, more effective chemotherapy regimens and molecular therapies should be investigated. Sharing the knowledge and capacity building to improve the treatment results in NBL are also critical for developing countries.

Metanephric adenoma (MA) is a renal tumor that is rarely diagnosed in children. Although it is considered benign and to have a good prognosis, the diagnosis of MA is challenging because of histopathologic and radiologic similarities to Wilms tumor. In this case report, we present a 6-year-old girl, with a renal mass and right hemihypertrophy, who was previously diagnosed as Wilms tumor on a fine-needle biopsy and diagnosed as MA after nephroureterectomy. The differentiation between Wilms tumor and metanephric adenoma is also discussed.

Skin and/or pulmonary involvement occur frequently in Langerhans cell histiocytosis (LCH) whereas nail involvement is rare. Herein, we present a case of LCH with initial nail disease and subsequent lung involvement causing recurrent pneumothoraces. Systemic chemotherapy was applied and intrapleural bleomycine and thoracoscopic pleurodesis with talc were performed. Although prognosis is not satisfactory in LCH with recurrent pneumothorax and nail involvement, the patient is under follow-up with no evidence of skin lesions, nail involvement, and pneumothorax for the last 10 months.