Clinical Features And Treatment Results Of Children With High-Risk Neuroblastoma Undergone To Autologous Stem Cell Transplantation

As autologous stem cell transplantation has a better survival rate than conventional chemotherapy, some prognostic factors are effective on survival and event in children with high risk NBL after autologous stem cell transplantation. This is a retrospective study. We aim to evaluate the effect of clinical and treatment features of 26 patients [Newly diagnosed / Relapsed 15 (57.7%) / 11 (42.3%)] with high-risk neuroblastoma (NBL) undergone to autologous stem cell transplantation with Busulphan-Melphalan or CarboplatinEtoposid- Melphalan. They were included in the study between 1998 and 2015. We aim to evaluate the effect of clinical and treatment features of them on outcome of autologous stem cell transplantation. EFS and OS were 21.4% and 61.8% at 3-year, but they were 14.2% and 53% at 5-year in patients with high-risk NBL. Post-transplant PFS and OS in patients with high risk were 31% and 51.7 at 3-year. Accordingly conditioning regimen, post-transplant PFS and OS rates were 51.9% and 25% in Bu-Mel, 45.5% and 44% in CEM group at 3-year (OS; p= 0.42 and PFS; p= 0.10). Unfavorable histology (p= 0.07, HR= 3.2, CI: 0.9-11.2) was effective factor on EFS, although it was not statistically significant. Remission status (without CR) at auto-SCT was effective factor on both PFS (p= 0.01, HR= 4.6, CI: 1.4-14.9) and OS (p= 0.04, HR= 5.1, CI: 1.1-24.2). Too deep and long-lasting hematologic toxicity was our experience in MIBG treatment before CEM conditioning regimen compared to Bu-Mel. Any conditioning transplant regimen is not superior to each other exactly. The major factors affecting the prognosis of children with neuroblastoma seem tumor load and unfavorable histology. Before autologous stem cell transplantation, complete remission status is essential for progression free and overall survival.WoSScopu

Coexistence Or A Related Condition: An Infant With Retinoblastoma And Gaucher Disease

Gaucher disease (GD) is the most prevalant lysosomal lipid storage disease that results from loss of function of acid beta-glucosidase due to mutations in the glucocerebrosidase gene. Common features of all types of GD include hepatosplenomegaly, cytopenia, and various patterns of bone and lung involvement. Retinoblastoma is a malignant tumor of the developing retina that occurs in children, typically before the age of five. Retinoblastoma develops from cells that have cancer-predisposing variants in both copies of RB1. The association between GD and retinoblastoma has not been reported until now. Here we report the case that was diagnosed with, retinoblastoma at the age of 2 months and then GD at the age of 11 months. Although there are controversies concerning the association between GD and cancer; malignancies should be kept in mind during GD patients follow up.WoSScopu

The Efficacy of Delayed Surgery in Children with High-Risk Neuroblastoma

Context: Surgery is an important part of treatment in children with neuroblastoma; however, exact timing is unclear. Both initial and delayed surgery was suggested as the best by numerous studies. Aims: Thus, we aimed to investigate the role of delayed surgery on 31 children with high-risk neuroblastoma. Materials and Methods: Thirty-one children with high-risk neuroblastoma were enrolled into the study. Statistical Analysis Used: Statistical analysis was performed using Statistical Package for the Social Sciences (SPSS) for windows 10.0. Results: There 'were 15 male and 16 female patients with a median age of 3.0 +/- 3.2 years. Primary tumor site was adrenal in 27, non-adrenal in two, pelvic in one, and mediastinal in one patient. MYCN gene was amplified in four and non-amplified in 11 children on totally 15 children with available data. Lactate dehydrogenase was elevated in 30 children. The tumor volumes at diagnosis and before surgery in the whole group were 154.3 and 12.5 mL, respectively. The decline in tumor volume was statistically significant (P < 0.0001). Initial surgery was performed in three and delayed in 20 children, and eight children were inoperable. Surgical complication rate was 66.6% (two out of three patients) in initial surgery group; however, the rate was 15% (3 out of 20 patients) in delayed surgery group. The 5-year event-free survival and overall survival rates in the whole group were 44.8% and 50.8%, respectively. Primary tumor area control rate was 95% Conclusions: In conclusion, the delayed surgery with intensive chemotherapy and radiotherapy has been successful for primary control in high-risk neuroblastoma patients.WoSScopu

Fibrous Hamartoma Of Infancy: A Case Report With Typical Ultrasonographic Findings

Fibrous hamartoma of infancy (FHI) is a rare, benign lesion which is commonly seen under two years of age. Clinical and radiological features of FHI can mimic malignant soft tissue sarcomas, it is important to make differential diagnosis. Ultrasound (US) examination reveals heterogeneous echogenicity that can be also suggestive for other soft tissue tumors but newly defined "serpentine pattern" of intervening hypoechoic portions in the hyperechoic mass with poorly defined margins and with poor vascularity is special for FHI. Here we report a 15-month-old-boy with FHI with serpentine pattern on US. He initially presented with a painless mass in his left axilla existing for approximately seven months. The mass was successfully excised and he has been followed for three years without any evidence of recurrence. Fibrous hamartoma of infancy should always be considered in differential diagnosis in children under two years of age with a firm and solitary mass in the axilla especially when US reveals serpentine pattern with poorly defined margins and with poor vascularity. If these clinical and ultrasonographic findings are seen in a child under two years old, surgery can be performed without any additional imaging modalities. Awareness and careful assessment are important in order not to misdiagnose this benign mass for which surgical excision is curative.WoSScopu

TREATMENT OF WILMS TUMOR: A Report from the Turkish Pediatric Oncology Group (TPOG)

WOS: 000278808800001PubMed ID: 20367260Aim: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. Methods and patients: Between 1998 and 2006, WT patients were registered from 19 centers. Patients 2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. Conclusions: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects

Outcome Of 102 Patients Under 5 Years Of Age With Hodgkin Lymphoma

Background. Hodgkin's lymphoma (HL) is one of the most curable pediatric cancers, however it is rare among children under five years of age and prognostic factors for survival rate are still unknown due to low frequency in this age group. Objectives. The aim of this study was to evaluate clinical characteristics, treatment regimens, and outcome of patients under five years of age with HL. Methods. Patients diagnosed with HL between 1972 and 2013 were retrospectively evaluated. All patients were treated with chemotherapy with or without radiotherapy. Results. There were 102 patients with a median age of 4 years (range: 2 to 4.9). The median follow-up time was 13 years. Twenty-three patients had B symptoms, 15 patients had `bulky disease' and the most common stages were stage I and II. Overall survival (OS) rates were significantly different according to the stage of the cancer (p = 0.008). Although there were no statistically significant differences; the positivity of `bulky disease' and B symptoms were associated with poor prognosis. Conclusion. Our single-center study included the largest number of patients under five years of age with HL. The stage was the main predictor for OS; on the other hand, the presence of B symptoms and bulky disease has also affected the prognosis.WoSScopu

An Eye Movement Desensitization and Reprocessing (EMDR) Group Intervention for Syrian Refugees With Post-traumatic Stress Symptoms: Results of a Randomized Controlled Trial

The number of refugees has increased significantly over the past few years. PTSD and depression are among the most common mental health problems among refugees. Eye Movement Desensitization and Reprocessing (EMDR), an effective treatment for PTSD, is usually administered individually. The availability of mental health resources would be greatly enhanced when EMDR can be delivered to groups. The EMDR G-TEP is a group protocol based on Early EMDR intervention protocols. There is clinical evidence and one field study published on the effect of EMDR G-TEP and there is only one RCT published on the treatment of PTSD and depression in a refugee camp. The aim of our study was to investigate the efficacy of EMDR G-TEP in treating post-trauma symptoms and depression and preventing the development of chronic PTSD among refugees living in a refugee camp. 47 adult participants with PTSD symptoms were randomly allocated to experimental (n = 18) and control (n = 29) groups. We measured Impact of Event Scale (IES-R), Beck Depression Inventory-II (BDI-II) and International Neuropsychiatric Interview (MINI) at pre-, post- and 4-week follow-up. Analysis of the results showed that the EMDR G-TEP group had significantly lower PTSD and depression symptoms after intervention. The percentage of PTSD diagnosis decreased from 100 to 38.9% in the EMDR G-TEP group and was unchanged in the control group. Following the EMDR G-TEP intervention 61.1% of the experimental group no longer had a PTSD diagnosis; this decrease was maintained at 4 weeks follow-up. In the control group the percentage of people who no longer met the diagnostic criteria for PTSD was 10.3% post-test and 6.9% at 4 weeks follow-up. A significant decrease in depression symptoms from pre-test levels was found in EMDR group but not in the control group follow up-test. This study indicated that EMDR G-TEP effectively reduced PTSD symptoms among refugees living in a camp, after two treatment sessions conducted over a period of 3 days. Further studies need to be performed using a larger number of participants, followed for a longer period of time and given more treatment sessions to strengthen our findings

The effect of CTLA-4 and CD28 gene variants and circulating protein levels in patients with gastric cancer

Objective: Gastric cancer is one of the most common malignancies worldwide. The risk factors for gastric cancer include environmental and genetic factors. Inflammation and the immune system are known to contribute to the development of the gastric cancer. We examined the influence of critical polymorphisms of CTLA-4 and CD28 genes and circulating protein levels on the etiology of gastric cancer