The association of miR-204 and mir-483 5p expression with clinicopathological features of Wilms tumor: Could this provide foresight?

BACKGROUND: Wilms tumor is the most common cancer of the kidney that occurs during childhood, and histologically, it mimics renal embryogenesis. With the development and improvement of up-to-date treatment protocols, the survival rates of Wilms tumor have increased. However, metastases or local relapses are still observed in 15% of patients. The search for reliable biomarkers to identify at-risk patients is ongoing to predict the variability in treatment success. Currently, the evaluation of clinical, histopathological and genetic features are common diagnostic methods; however, epigenetic features can be examined with microRNA expression analyses and might allow us to comment on the behavior of the tumor and treatment response. METHODS: In this study, we aimed to evaluate the relationship between microRNA-204 and microRNA-483-5p expression with clinicopathological data and the effect on Wilms tumor survival. For this purpose, the expression levels of RNU6B, microRNA-204 and microRNA-483-5p were evaluated in tumor and normal tissue by qreal time-polymerase chain reaction. We also investigated the relationship between microRNA expression levels with the clinicopathological and histological features of Wilms tumor. RESULTS AND CONCLUSION: The results of our study indicate that the relative expression levels of microRNA-204 in Wilms tumor tissues were significantly lower than that in adjacent normal tissues. By contrast, tumor tissue had a higher microRNA-483-5p expression than the corresponding normal tissues. A statistically significant difference between microRNA-204 expression level with age and the presence of anaplasia was observed. The upregulation of microRNA-483-5p was found to have a significant correlation with patients after preoperative chemotherapy and complete tumor necrosis. Taken together, our data suggest that microRNA-204 could play a critical role as a tumor suppressor, whereas microRNA-483-5p acts as an oncogene in Wilms tumor progression. More importantly, microRNA-204 might be a novel predictive biomarker for anaplastic histology and could be useful for developing therapeutic interventions targeting this marker.

MRI-based texture analysis for differentiating pediatric craniofacial rhabdomyosarcoma from infantile hemangioma.

OBJECTIVES: To evaluate the diagnostic performance of MRI texture analysis (TA) for differentiation of pediatric craniofacial rhabdomyosarcoma (RMS) from infantile hemangioma (IH). METHODS: This study included 15 patients with RMS and 42 patients with IH who underwent MRI before an invasive procedure. All patients had a solitary lesion. T2-weighted and fat-suppressed contrast-enhanced T1-weighted axial images were used for TA. Two readers delineated the tumor borders for TA independently and evaluated the qualitative MRI characteristics in consensus. The differences of the texture features' values between the groups were assessed and ROC curves were calculated. Logistic regression analysis was used to analyze the value of TA with and without the combination of the qualitative MRI characteristics. A p value < 0.05 was considered statistically significant. RESULTS: Thirty-eight texture features were calculated for each tumor. Eighteen features on T2-weighted images and 25 features on contrast-enhanced T1-weighted images were significantly different between the RMSs and IHs. On contrast-enhanced T1-weighted images, the short-zone emphasis (SZE), which was a gray-level zone length matrix (GLZLM) parameter, had the largest area under the curve: 0.899 (sensitivity 93%, specificity 87%). The independent predictor for the RMS among the qualitative MRI characteristics was heterogeneous contrast enhancement (p < 0.001). Using only a GLZLM_SZE value of lower than 0.72 was found to be the best diagnostic parameter in predicting RMS (p < 0.001; 95% CI, 8.770-992.4). CONCLUSION: MRI-based TA may contribute to differentiate RMS from IH without invasive procedures. KEY POINTS: • Texture analysis may help to distinguish between rhabdomyosarcoma and infantile hemangioma without invasive procedures. • The gray-level zone length matrix parameters, especially the short-zone emphasis, may be a potential predictor for rhabdomyosarcoma. • Using contrast-enhanced T1-weighted images may be superior to T2-weighted images to differentiate rhabdomyosarcoma from infantile hemangioma in texture analysis.

Cross-sectional study: long term follow-up care for pediatric cancer survivors in a developing country, Turkey: current status, challenges, and future perspectives

Aim: The main purpose of this study is to determine the current status of long-term follow-up (LTFU) for childhood cancer survivors and the challenges of LTFU for pediatric cancer survivors at pediatric oncology institutions in Turkey. Material and methods: A questionnaire was e-mailed to the directors of 33 pediatric oncology centers (POCs) registered in the Turkish Pediatric Oncology Group (TPOG). Of these 33 active TPOG institutions, 21 participated in the study and returned their completed questionnaires. Results: Only 1 of the 21 participating centers had a separate LTFU clinic. The remaining centers provided LTFU care for childhood cancer survivors at the pediatric oncology outpatient clinic. Of these centers, 17 (80.9%) reported difficulty in transition from the pediatric clinic to the adult clinic, 14 (66.6%) reported insufficient care providers, and 12 (57.1%) reported insufficient time and transportation problems. As neglected late effects, 16 (76.1%) centers reported psychosocial and getty job problems and 11 (52.3%) reported sexual and cognitive problems. None of the centers had their own LTFU guidelines for their daily LTFU practice Conclusion: This study was the first to gain an overview of the needs of POCs and the gaps in survivorship services in Turkey. The results from this study will help to develop a national health care system and national guidelines for pediatric cancer survivors.

The effect of the closed intravenous catheter system on first insertion success, indwelling time, and complications in pediatric hematology and oncology patients: A randomized controlled study.

PURPOSE: The primary purpose of this research is to evaluate the effect of two different catheter systems (closed IV catheter system: BD Nexiva™, peripheral open IV catheter: BD Insyte™ Autoguard™) on first insertion success, catheter indwelling time, and the catheter complications. METHOD: This randomized controlled study used a single-blind and parallel trial design guided by the CONSORT checklist. The "Peripheral Intravenous Catheter (PIVC) Bundle" was applied to all patients. A total of 214 catheters of 38 patients were included in the intervention (BD Nexiva™) (n = 107 catheter) and control (open IV catheter) groups (n = 107 catheter) of the study. The indwelling time and PIVC complications were followed. RESULTS: The mean age of the patients in the study group was 5.9 ± 2.2, and the mean age of the patients in the control group was 5.7 ± 1.9. The PIVC was successfully placed in 68.2% of the patients in the study group and in 65.4% of the patients in the control group at the first attempt. It was determined that the indwelling time was 4.9 ± 3.9 (max. 20.25 days) in the study group and 2.9 ± 2.8 (max. 11.25 days) days in the control group. The complication rates were found to be 86.8 for the study group and 166.9 for the control group in 1000 catheter days. In this study, no difference was found in terms of complication. CONCLUSIONS: The PIVC indwelling time is longer in patients with the closed IV catheter system. These new technology PIVCs can be used for this special patient population. GOV IDENTIFIER: NCT05769452.

The association between birth weight and the risk of neuroblastoma: a meta-analysis of observational studies involving 4,361,141 participants.

One of the most common extracranial solid tumors in childhood is neuroblastoma. In this study, it was aimed to perform a systematic review and meta-analysis to evaluate the risk of neuroblastoma in both high and low birth weights. The PRISMA and MOOSE guidelines were followed during the design, analysis, and reporting of this study. A comprehensive literature search was undertaken for the published papers in Embase, PubMed/Medline, Scopus, and the Web of Science (WoS) databases. The odds ratio (OR) of neuroblastoma in high and low birth weight groups, with 95% confidence intervals (CIs), were calculated using the random-effects and fixed-effects models. A total of 16 papers and 4,361,141 participants were included in this study. When the random-effects model and the fixed-effects model were used, high birth weight was associated with an increased risk of neuroblastoma (OR = 1.17; 95% CI: 1.06-1.29, P = 0.002; heterogeneity: Chi2 = 2.33, df = 15, I2 = 0%, P>0.05). Similarly, it was observed that individuals with low birth weights may also face an increased risk of developing neuroblastoma later in life (OR = 1.19; 95% CI: 1.03-1.37, P = 0.017; heterogeneity: Chi2 = 16.93, df = 15, I2 = 0%, P = 0.323). In conclusion, both high and low birth weight in individuals may be among the important risk factors for neuroblastoma development.

Identification of ALK Mutation in Neuroblastoma on the Point of Molecular Heterogeneity.

BACKGROUND AND AIM: In neuroblastoma, anaplastic lymphoma kinase mutations have recently received attention as molecular targets for the treatment of neuroblastoma, as 6% to 10% of patients with neuroblastoma have anaplastic lymphoma kinase mutations. There are little data from the cases in Turkey. We aimed to detect anaplastic lymphoma kinase mutations and molecular heterogeneity in neuroblastoma using next-generation sequencing. This study is the first one with this many cases in Turkey. METHODS: Next-generation sequencing analysis was performed using an Illumina MiniSeq custom gene panel. Clinically important mutations were selected for the analysis. We also gathered clinical data of the patients from Turkish Pediatric Oncology Group cohorts to associate them with anaplastic lymphoma kinase mutations. This study is a retrospective cross-sectional study. We followed STROBE guideline (https://www.equator-network.org/reporting-guidelines/strobe/) on this study. RESULTS: We analyzed anaplastic lymphoma kinase in 108 patients with neuroblastoma, with a mean age of 43.76 months. Pathogenic anaplastic lymphoma kinase mutations were detected in 13 patients (12.04%). We noted that anaplastic lymphoma kinase mutations were primarily observed in intermediate- and high-risk patients (P = .028). R1275Q and F1174-related mutations were predominant; I1171T, L1226F, S1189F, V1135A, and G1125S mutations were rare. Duplicate samples did not exhibit any heterogeneity. CONCLUSIONS: We found that F1174 and R1275Q-related anaplastic lymphoma kinase mutations are the most common pathogenic mutations in neuroblastoma. Anaplastic lymphoma kinase mutation status did not show any heterogeneity, and the mutations were correlated with intermediate- or high-risk groups.

A case of rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation, and neural crest tumor: ROHHADNET syndrome.

OBJECTIVE: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) is a rare disorder that mimics both common obesity and genetic obesity syndromes along with several endocrine disorders during early childhood. We aim to present the clinical features, laboratory and imaging results, and treatment outcomes of a patient with ROHHAD syndrome. METHODS: In this case report, we describe a 26-month-old boy who was admitted to our emergency department with dyspnea and cyanosis and was suspected to have ROHHAD syndrome due to his rapid-onset obesity and alveolar hypoventilation. RESULTS: A thoracal and abdominal magnetic resonance imaging was performed to demonstrate a possible accompanying neural crest tumor and it provided a yet asymptomatic retroperitoneal ganglioneuroblastoma. Based on these findings, the patient was diagnosed as ROHHADNET syndrome. CONCLUSION: Because of the high prevalence of cardiorespiratory arrest and probability of accompanying tumors, early recognition of ROHHAD syndrome is important. To prevent presumptive mortality and morbidity, ROHHAD syndrome should be considered in all cases of rapid and early-onset obesity associated with hypothalamic-pituitary endocrine dysfunctions.