Malignant peripheral nerve sheath tumors in childhood: 13 cases from a single center.

PURPOSE: We aimed to review clinical characteristics, treatment results, and outcome for pediatric patients with malignant peripheral nerve sheath tumors (MPNSTs). METHODS: Files for 13 children diagnosed with MPNSTs and treated at our hospital between 1988 and 2009 were reviewed for clinical characteristics, treatment results, and outcome. RESULTS: The median patient age was 11 years (range, 0.16 to 18 y; female/male: 6/7). The most common symptoms were palpable mass (7 of 13) and pain (3 of 13). Four patients had neurofibromatosis type 1. Physical findings at diagnosis were palpable mass (10 of 13), scoliosis (2 of 13), paraplegia/cranial nerve palsy (2 of 13), and stigmas of neurofibromatosis type 1. The primary sites were head and neck (5 of 13), abdomen (3 of 13), chest wall (2 of 13), abdominal wall (2 of 13), and extremities (1 of 13). According to the Intergroup Rhabdomyosarcoma Study system, 5 cases were classified as Intergroup Rhabdomyosarcoma Study group I, 6 were classified as group III, and 2 were classified as group IV. Five-year overall and event-free survival rates were 34.6% (±13.8) and 18.8% (±11.9), respectively. Overall survival rates did not differ upon addition of chemotherapy and/or radiotherapy to the surgery. Five-year survival rates were 42.9% (±18.9) in patients with total resection and 37.5% (±28.6) in patients with partial resection (P=0.08). Overall survival rates did not differ according to stages (P=0.8). CONCLUSIONS: 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.

Metanephric adenoma in a 6-year-old child with hemihypertrophy.

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.

Neuroblastoma in Turkish children: experience of a single center.

OBJECTIVE AND METHOD: The survival of the patients with neuroblastoma has improved in last few decades. But still it depends on various clinical and biological factors. To assess the clinical features and trends in survival, the data for 500 newly diagnosed patients between January 1972 and December 2004 from a single center were retrospectively analyzed. RESULTS: Histopathologic subtypes were neuroblastoma (NBL) in 462 patients (92.4%) and ganglioneuroblastoma in 38 patients (7.6%). The median age was 2.9 years and Male/Female ratio was 1.3/1. Primary tumor sites were abdomen, thorax, pelvis, neck, and others with the frequency of 72.2%, 14.9%, 3.8%, 3.2%, and 5.9%, respectively. There were 30, 49, 133, 257, 31 patients with stage 1, 2, 3, 4, 4S disease and their 10-year survival rates were 100%, 75.8%, 34.1%, 6.5%, and 59.4%, respectively. The outcome has significantly improved according to 10-year periods. The 5-year overall survival rates were 14%, 26.1%, 39.2%, and 52.4% for the years of 1970s, 1980s, 1990s, and after 2000. Surgical procedure involving total or near total tumor removal improved the survival (P=0.002). Both 5-year overall survival and event free survival rates were higher when partial resection was performed, especially in stage 3 disease (P=0.002 and P=0.02). In multivariate analysis, age above 18 months at diagnosis (P=0.01), stage 4 disease (P<0.001), abdominal primary tumor site (P<0.001), NBL subtype in histopathology (P=0.001), responsiveness to chemotherapy (P<0.001) positive or high Vanillyl mandelic acid levels (P=0.02) and male sex (P=0.008) were the determinants of poor prognosis. CONCLUSIONS: 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.

Langerhans cell histiocytosis with involvement of nails and lungs in an adolescent.

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.

Prognostic significance of metastatic site at diagnosis in Wilms' tumor: results from a single center.

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.