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BACKGROUND: Despite the development of new treatment options, the prognosis of high‐risk neuroblastoma patients is still poor; more than half of patients experience disease recurrence. High‐dose chemotherapy and haematopoietic stem cell rescue (i.e. myeloablative therapy) might improve survival. This review is the second update of a previously published Cochrane review. OBJECTIVES: Primary objective To compare the efficacy, that is event‐free and overall survival, of high‐dose chemotherapy and autologous bone marrow or stem cell rescue with conventional therapy in children with high‐risk neuroblastoma. Secondary objectives To determine adverse effects (e.g. veno‐occlusive disease of the liver) and late effects (e.g. endocrine disorders or secondary malignancies) related to the procedure and possible effects of these procedures on quality of life. SEARCH METHODS: We searched the electronic databases The Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2014, issue 11), MEDLINE/PubMed (1966 to December 2014) and EMBASE/Ovid (1980 to December 2014). In addition, we searched reference lists of relevant articles and the conference proceedings of the International Society for Paediatric Oncology (SIOP) (from 2002 to 2014), American Society for Pediatric Hematology and Oncology (ASPHO) (from 2002 to 2014), Advances in Neuroblastoma Research (ANR) (from 2002 to 2014) and American Society for Clinical Oncology (ASCO) (from 2008 to 2014). We searched for ongoing trials by scanning the ISRCTN register (www.isrct.com) and the National Institute of Health Register (www.clinicaltrials.gov). Both registers were screened in April 2015. SELECTION CRITERIA: Randomised controlled trials (RCTs) comparing the efficacy of myeloablative therapy with conventional therapy in high‐risk neuroblastoma patients. DATA COLLECTION AND ANALYSIS: Two authors independently performed study selection, data extraction and risk of bias assessment. If appropriate, we pooled studies. The risk ratio (RR) and 95% confidence interval (CI) was calculated for dichotomous outcomes. For the assessment of survival data, we calculated the hazard ratio (HR) and 95% CI. We used Parmar's method if hazard ratios were not reported in the study. We used a random‐effects model. MAIN RESULTS: We identified three RCTs including 739 children. They all used an age of one year as the cut‐off point for pre‐treatment risk stratification. The first updated search identified a manuscript reporting additional follow‐up data for one of these RCTs, while the second update identified an erratum of this study. There was a significant statistical difference in event‐free survival in favour of myeloablative therapy over conventional chemotherapy or no further treatment (three studies, 739 patients; HR 0.78, 95% CI 0.67 to 0.90). There was a significant statistical difference in overall survival in favour of myeloablative therapy over conventional chemotherapy or no further treatment (two studies, 360 patients; HR 0.74, 95% CI 0.57 to 0.98). However, when additional follow‐up data were included in the analyses the difference in event‐free survival remained statistically significant (three studies, 739 patients; HR 0.79, 95% CI 0.70 to 0.90), but the difference in overall survival was no longer statistically significant (two studies, 360 patients; HR 0.86, 95% CI 0.73 to 1.01). The meta‐analysis of secondary malignant disease and treatment‐related death did not show any significant statistical differences between the treatment groups. Data from one study (379 patients) showed a significantly higher incidence of renal effects, interstitial pneumonitis and veno‐occlusive disease in the myeloablative group compared to conventional chemotherapy, whereas for serious infections and sepsis no significant difference between the treatment groups was identified. No information on quality of life was reported. In the individual studies we evaluated different subgroups, but the results were not univocal in all studies. All studies had some methodological limitations. AUTHORS' CONCLUSIONS: Based on the currently available evidence, myeloablative therapy seems to work in terms of event‐free survival. For overall survival there is currently no evidence of effect when additional follow‐up data are included. No definitive conclusions can be made regarding adverse effects and quality of life, although possible higher levels of adverse effects should be kept in mind. A definitive conclusion regarding the effect of myeloablative therapy in different subgroups is not possible. This systematic review only allows a conclusion on the concept of myeloablative therapy; no conclusions can be made regarding the best treatment strategy. Future trials on the use of myeloablative therapy for high‐risk neuroblastoma should focus on identifying the most optimal induction and/or myeloablative regimen. The best study design to answer these questions is a RCT. These RCTs should be performed in homogeneous study populations (e.g. stage of disease and patient age) and have a long‐term follow‐up. Different risk groups, using the most recent definitions, should be taken into account. It should be kept in mind that recently the age cut‐off for high risk disease was changed from one year to 18 months. As a result it is possible that patients with what is now classified as intermediate‐risk disease have been included in the high‐risk groups. Consequently the relevance of the results of these studies to the current practice can be questioned. Survival rates may be overestimated due to the inclusion of patients with intermediate‐risk disease.

Treatment of renal cell carcinoma (RCC) in children is based on an extensive surgery. Radical nephrectomy with lymph node dissection is sufficient for most of the stage I-II tumors. However, in advanced disease some different management strategies have been defined including immunotherapy. We present a 9-year-old girl with advanced stage RCC, inoperable at the time of diagnosis and successfully treated with interferon-alpha after operation., Renal hücreli karsinomun çocukluk çağındaki tedavisi esas olarak geniş cerrahi rezeksiyon ile yapılmaktadır. Çoğu evre I-II vaka için radikal nefrektomi ve lenf nodu diseksiyonu yeterli olurken ileri evrelerde immünoterapinin de dahil olduğu farklı tedavi yöntemleri tanımlanmıştır. Burada tanı anında opere edilemeyen, sonrasında interferon alfa ile başarılı bir şekilde tedavi edilen ileri evre renal hücreli karsinomu olan 9 yaşında bir kız sunulmuştur.

By comparing our data for the period 1985-2008 with findings from a previous report covering the period 1975-1984, we aimed to share our experience with poisoning cases in order to contribute toward its prevention, diagnosis and treatment. The records of patients admitted to the Pediatric Intensive Care Unit with acute poisoning between November 1985 and October 2008 were evaluated retrospectively. The records of 2251 patients with acute poisoning could be retrieved. Poisoning mostly occurred in the home (92%), via the oral route (92.5%) and by a single intoxicant (81.3%). Two distinct peaks were observed: in boys between 1-5 years of age and in girls between 13-16 years of age. It was noted that 67.4% of poisoning cases were accidental, whereas 25.9% were suicidal and 6.7% were a result of a therapeutic error. Nearly two-thirds (64%) of cases were drug-related, while 36% were non-drug-related. Analgesics-antipyretics ranked first among the drug-related cases, whereas ingestion of a corrosive substance was most common among cases with non-drug poisoning. Colchicine was associated with the highest fatality, while among the causes of non-drug poisoning, carbon monoxide was the deadliest. The overall mortality rate in this study was 1.9%. Mortality from non-drug poisoning was higher than from drug-related causes (3.9% vs. 1.3%). Almost all cases of poisoning below the age of 6 years are potentially preventable. The results of this study highlight the need for reforms in industrial and health policies, with the aim of increasing awareness regarding potential toxins, appropriate storage of potential toxins, and general precautions to promote safety in the home.

Thoracic neuroblastic tumors (NBTs) are reported to have better prognosis. We aimed to review clinical characteristics, treatment results, and outcome of our patients with thoracic NBT.Files of 87 children treated at our hospital between 1973 and 2007 with the diagnoses of thoracic NBT were reviewed for clinical and pathological characteristics. Treatment results and outcomes of these cases were examined.All but one tumors were located in posterior mediastinum, one in the posterior chest wall. Median age of all was 2.1 years (range, 0.03-14; F/M: 1.42). Fifteen cases had ganglioneuromas (GN), 26 ganglioneuroblastomas (GNBL), and 46 neuroblastomas (NBLs). Stages were: I, 20.5%; II, 22.1%; III, 38.2%; IV, 14.7%; IVS, 4.5%. Stages III and IV were more common in cases over 1 year of age. In 20 patients diagnoses were incidental. Twenty-two of 87 (25.3%) had symptomatic spinal cord compression and 15 (17.3%) had Horner syndrome. Ten-year overall and event-free survival rates were 71.2% and 67.4%, respectively. Survival rates did not differ depending on the age being younger or older than 1 year. Ten-year survival rates were 88.8% in stages I, II, IVS; 65.3% in stage III and 27.8% in stage IV (P = 0.0002).Thoracic NBLs had a favorable prognosis. This might be a result of earlier diagnosis and some distinct biological characteristics. Favorable prognosis would suggest less aggressive treatment for such patients. Further studies on the biological characteristics of NBLs in the thoracic site and their association with outcome should be done.

Herpes zoster is a painful neurocutaneous disease caused by the reactivation of the varicella zoster virus, and it can develop any time after a primary infection, which usually occurs during childhood. A variety of immunocompromised patient populations are known to be at increased risk for herpes zoster. Postherpetic pruritus is a serious complication, which may last after the infection, and has the potential to cause injury and disability. The medical literature on postherpetic pruritus is very limited. This is a case report of a six-year-old child who developed postherpetic pruritus lasting three months, which responded to topical urea treatment.

The objectives of the study were to compare the results of surgical procedures, amputation and limb-sparing surgery, and to assess the influence of preoperative chemotherapeutic regimens on the survival of pediatric patients with osteosarcoma. We retrospectively analyzed 69 patients treated at our institution between January 1985 and April 2004. The primary treatment modalities were limb-sparing surgery or amputation with or without preoperative chemotherapy. The need for postoperative chemotherapy was determined by the histological response, the tumor margins, and the burdens created by the metastatic disease. The age range was 5.3 to 18.6 years (median, 13.3 years); with a male-female ratio of 0.9. The most common lesion site was the femur, found in 39 patients. Fourteen of the patients had metastases involving the lungs or other bones at the time of diagnosis. Preoperative chemotherapy was done in 45 patients. Most of the patients were treated with cisplatin + adriamycin (27/69, 39.1%). Forty-two patients were surgically treated by amputation and 19 with limb-sparing surgery. Four patients had surgical resection of masses located at sites other than the extremities, and in 4 patients, surgery was not possible. The overall survival rate for the whole group was 32.6%. The overall survival rates were 27.2% and 66.9% for the patients treated with amputation and limb-sparing surgery, respectively. Osteosarcoma has a poor prognosis. Based on our 20-year experience, limb-sparing surgery as surgical management and the cisplatin + adriamycin preoperative chemotherapy regimen seem to be a promising modality for the patients with osteosarcoma.

Background: PUVA has been suggested as an alternative treatment modality for resistant, diffuse alopecia areata (AA). However, there are conflicting reports on its efficacy. Objective: The aim of this study was to investigate the efficacy of PUVA treatment of AA in a Turkish population. Methods: Twenty-four patients (15 female and 9 male) suffering from extensive AA for more than 1 year were included in a retrospective study. PUVA was administered to the whole body 3 times a week. Only 75–100% hair regrowth was defined as cosmetically acceptable. Results: Excellent hair regrowth was achieved in 37.5% of the patients. The therapeutic efficiency was not related to age and sex of the patients, duration and extent of the disease (p Conclusion: PUVA can be an alternative therapy for patients with extensive AA independently of the duration of disease.

A 13-year-old girl was admitted to our hospital with a one-month history of bullous skin lesions. Physical examination revealed ulcerated and nonulcerated cutaneous plaques, bullae, enlarged cervical and supraclavicular lymph nodes and hepatomegaly. In another hospital, histopathological diagnosis of a skin biopsy was reported to be consistent with tuberculosis and she was treated with antimycobacterial drugs. Since no response was obtained, she was referred to our center after a new lymph node biopsy was obtained. At our center, histopathological diagnosis was anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma (ALCL). We started LMT chemotherapy regimen and initial response was complete. Eight months after initial admisision, she experienced cutaneous recurrence of disease while on maintenance protocol. Chemotherapy was changed to LSA4 regimen. She is still on chemotherapy and has been in complete remission for nine months. Clinicians should be aware of this uncommon presentation of ALCIL, which can be confused with other diseases clinically or histologically.

A one-week-old boy had undergone resection of a sacrococcygeal benign cystic teratoma. At the age of 12 months, he had a serum alpha-fetoprotein level of 139,000 IU/ml and a recurrent pelvic mass which was removed, and the microscopic examination revealed endodermal sinus tumor. Postoperatively, massively enlarged inguinal lymph nodes and abdominal distention developed. Computerized tomography displayed enlarged inguinal lymph nodes, metastatic lesions in the liver, and a pelvic recurrent mass. He received BEP (bleomycin, etoposide, cisplatin) chemotherapy regimen, and a complete remission was achieved with a normal serum alpha-fetoprotein. Close follow-up and serum alpha-fetoprotein monitoring are mandatory after the resection of a sacrococcygeal teratoma.

Ataxia telangiectasia (AT) homozygotes have an increased risk for development of Hodgkin's disease (HD). Parenchymal lung involvement is not uncommon in HD; however, cavitary pulmonary lesions are quite unusual. We report on 3 cases of AT with HD who had mediastinal disease and parenchymal pulmonary involvement with cavitation. Of 6 AT patients in our HD series, 3 developed pulmonary cavities. The patients displayed pulmonary infiltration, cavitation in the lung parenchyma, and mediastinal enlarged lymph nodes on both plain chest X-rays and thoracic computed tomographies. No infectious etiologies were established for the pulmonary findings. Histopathological examination of open lung and mediastinal biopsies revealed HD, and all patients received multiagent chemotherapies. The outcome was fatal in all 3 patients. Respiratory infections are the principle cause for morbidity and mortality in AT patients. Reports on cavitating pulmonary lesions in HD are quite rare. Furthermore, data regarding the patterns of pulmonary involvement in AT patients with or without HD are lacking. The increased incidence of malignancies in AT patients may relate to immunodeficiency and to the chromosomal alterations identified.