BACKGROUND:This study aims to evaluate the prognostic factors and survival outcomes of pediatric patients with pineal region tumors (PRTs) using a large population-based registry. METHODS:We employed a retrospective cohort design, utilizing data from the Surveillance, Epidemiology, and End Results program, covering the period from 2000 to 2019. We included pediatric patients (ages 0-18) with histologically verified malignant PRTs. To determine prognostic indicators, we conducted survival analyses using Kaplan-Meier methods and Cox proportional hazards models. Furthermore, we constructed a predictive nomogram derived from the Cox regression outcomes. RESULTS:The study included 596 pediatric patients with PRTs. Germ cell tumors were the most common subtype (63.3%), followed by pineoblastomas (26.3%) and other subtypes (10.4%). The overall 5-year survival rate was 79.6%. Multivariate analysis revealed that age at diagnosis, Surveillance, Epidemiology, and End Results stage, histological subtypes, and radiation therapy were significant predictors of survival. Patients under 5 years of age and those diagnosed with pineoblastomas had worse survival outcomes. Radiation therapy was associated with improved survival. CONCLUSIONS:Early age at diagnosis and aggressive histological types such as pineoblastomas are associated with poorer outcomes. Future research should focus on refining therapeutic protocols that integrate the histological and molecular profiles of these tumors.

PURPOSE:This population-based study examined pediatric liposarcoma (LPS) prognosis and developed a nomogram to predict overall survival (OS). METHODS:Data from 145 pediatric LPS patients (2000-2019) were analyzed. Survival curves were compared using log-rank tests and generated via Kaplan-Meier estimation. Cox proportional-hazards regression identified OS-associated variables, and a nomogram was constructed. RESULTS:Myxoid LPS (72.5%) was the most common subtype, predominantly in extremities (66.8%). Most cases were localized (77.9%), with regional (17.2%) and distant (4.9%) stages less frequent. Surgery alone was the primary treatment (69.0%). OS rates were 90.5% (3-year) and 86.9% (5-year). Localized disease had significantly better survival than distant disease (96.2% vs. 14.3%, p < 0.001), and myxoid LPS showed superior OS (p < 0.001). Cox analysis identified primary site (HR 7.78, p = 0.004) and SEER stage (HR 30.44, p < 0.001) as independent prognostic factors. CONCLUSIONS:Pediatric LPS typically presents as localized, extremity-based myxoid tumors with favorable outcomes. High-grade or distant-stage disease, though rare, correlates with poorer survival. The nomogram provides individualized OS prediction to guide clinical management.

Neonatal necrotizing enterocolitis (NEC) is a common gastrointestinal emergency in neonates. MiRNA-192-5p was found associated with ulcerative colitis (UC) progression, also with aberrant expression in intestinal cancer tissue. However, the effects of miRNA-192-5p on NEC have not been reported. Based on the bioinformatics analysis of the GEO dataset, miR-192-5p was identified as the differentially expressed miRNA in NEC, and activated leukocyte cell adhesion molecule (ALCAM) was predicted as its target. After that, in vitro, rat intestinal epithelial cell-6 (IEC-6) were stimulated with LPS to construct a cell model of NEC. IEC-6 cells were transfected with miRNA-192-5p mimics, miRNA-192-5p inhibitors, or miRNA-192-5p inhibitors + sh-ALCAM, and relevant negative control. In vivo, SD rats were treated with artificial feeding, hypoxic reoxygenation, cold stimulation, and LPS gavage to induce NEC, followed by injection of agomiR-NC or agomiRNA-192-5p. Then effects of miRNA-192-5p on NEC model IEC-6 cell viability, apoptosis, ALCAM expression, Interleukin (IL)-1β and IL-6 levels, intestinal injury, intestinal permeability were detected. MiRNA-192-5p expression was downregulated in NEC IEC-6 cells, whose overexpression increased IEC-6 cell viability. MiRNA-192-5p inhibitors increased IL-1β, IL-6 levels and promoted IEC-6 cell apoptosis. MiRNA-192-5p targeting of ALCAM decreased ALCAM expression, IL-1β, and IL-6 levels. AgomiRNA-192-5p decreased ALCAM, IL-1β, and IL-6 levels in intestinal tissue and pathological damage and increased miRNA-192-5p levels. MiR-192-5p protects against intestinal injury by inhibiting ALCAM-mediated inflammation and intestinal epithelial cells, which would provide a new idea for NEC treatment.

Neural tube defects (NTDs) are severe congenital anomalies that significantly impact the central nervous system, arising from the neural tube's failure to close during early embryogenesis. In this study, we investigated NTDs and associated pathophysiological mechanisms in foetal rats following exposure to all-trans retinoic acid (atRA). Out of 168 embryos from 15 pregnant rats in the experimental group, 78% displayed NTDs with notable spinal deformities, primarily in the lumbar-sacral region, similar to human cases. Body weight and crown-rump length (CRL) measurements indicated significant growth impairment in the NTD group compared to controls, while the atRA-treated group without NTDs showed no notable differences in growth. Immunohistochemistry (IHC) results demonstrated decreased NeuN and PCNA expression in the NTD group's spinal cord. Oxidative stress markers showed markedly reduced superoxide dismutase (SOD) and glutathione peroxidase (GSH-px) activity, alongside increased malondialdehyde (MDA) levels in the NTD group, indicating heightened oxidative stress. Analysis of apoptosis-related proteins revealed elevated Bax and caspase-3 levels, reduced Bcl-2 and lower poly (ADP-ribose) polymerase (PARP) in the NTD group, suggesting a pronounced shift towards proapoptotic pathways, potentially contributing to NTD progression. Our findings indicate that oxidative stress and apoptosis play significant roles in the development of NTDs. Future investigations should aim to pinpoint critical regulatory genes or proteins that might be targeted for therapeutic interventions to alleviate oxidative stress and apoptosis in NTD development.

Adrenocortical carcinoma (ACC) is an uncommon adrenal gland endocrine tumor that has a poor prognosis in children. We aimed to conduct a population-based cohort study to predict overall survival (OS) in pediatric patients with ACC. We used the Surveillance, Epidemiology, and End Results (SEER) database to conduct a retrospective cohort research on pediatric patients diagnosed with ACC between 1975 and 2018. We examined demographic characteristics, tumor stage and size, treatment options, and survival results. Kaplane-Meier estimations were used to generate survival curves based on several parameters. To compare survival curves, the log-rank test was applied.Cox proportional-hazards regression was used to determine the variables related with OS. In addition, we created a nomogram to predict overall survival in pediatric ACC patients. A total of 143 pediatric ACC patients were identified. Females were the most impacted (60.8

Purpose Colorectal cancer (CRC) is rarely found in children and adolescents. The purpose of this study was to retrospectively conduct a population-based cohort of pediatric patients with CRC. Methods All pediatric patients with CRC diagnosed between 1975 and 2018 were identified using the Surveillance, Epidemiology, and End Results (SEER) database. The demographics and clinical variables of the patients were summarized, and treatment outcomes and prognostic factors were examined. The study was presented in accordance with the STROBE reporting checklist. Results A total of 284 CRC patients were identified. At 3- and 5-year follow-up, the overall survival rates were 63.1% and 52.6%, respectively. Patients with local disease had a significantly improved overall survival (OS) than patients with distant disease. At 3- and 5-year follow-up, the overall survival rates of adenocarcinoma (nos) and adenocarcinoma (polyp) were similar and significantly better than those of patients with mucinous adenocarcinoma and signet ring cell carcinoma ( P < 0.001). In terms of treatment, patients who underwent surgery outlived non-surgery patients (3-year OS, 70.4% versus 26.6%, P < 0.001). Multivariate analysis revealed that SEER stage and histologic type were important independent predictors of outcomes. Conclusions Children and adolescents with CRC are likely to be in an advanced stage, have a worse histologic subtype, and have a poorly differentiated grade. Although surgical resection considerably increases survival for the majority of patients, pediatric patients with CRC have a poor prognosis. Considerable efforts are required to improve their survival outcomes.

SALL4 is a zinc finger transcription factor that exerts its physiological role during embryo-fetal development. Analyses of SALL4 expression have shown its oncogenic role in precursor B-cell lymphoblastic lymphoma, acute and chronic myeloid leukemia, gastrointestinal, breast, and lung cancers. The aim of this study was to determine the immunohistochemical profile of SALL4 in pediatric yolk sac tumors (YSTs). Immunohistochemistry detection of SALL4 was performed in 22 cases of pediatric YSTs and 10 mature teratomas. The percentage of tumor cells stained was scored as 0, 1+ (1–30% cells), 2+ (31–60%), 3+ (61–90%), and 4+ (> 90%). To compare its sensitivity and specificity with Glypican-3 and α-fetoprotein (AFP), we also stained tumors from these cases for Glypican-3 and AFP. In contrast to AFP and glypican-3, SALL4 staining in more than 90% of the tumor cells was seen in all 22 pediatric YSTs (100% sensitivity) (P < 0.001 for both SALL4 vs. AFP and SALL4 vs. glypican-3). SALL4 is a sensitive marker for pediatric YSTs and it can be used to distinguish them from mature teratomas. SALL4 is likely to become a new and valuable biomarker for the diagnosis of pediatric YST.

PURPOSE:We conducted a systematic review and meta-analysis to compare the clinical outcomes between laparoscopic splenectomy and the traditional open splenectomy in children. METHODS:Literature searches were conducted to identify studies having compared the laparoscopic splenectomy (LS) and open splenectomy (OS) for children. Parameters such as operative time, blood loss, length of postoperative stay, the removal of accessory spleens and postoperative complications including postoperative high fever, acute chest syndrome (ACS), and ileus were pooled and compared by meta-analysis. RESULTS:Among the 922 pediatric participants included in the 10 studies, 508 had received LS and 414 OS. There were shorter length of hospital stays, less blood loss, and longer operative times with the LS approach compared with OS. However, no significant difference was found between LS and OS in the secondary outcome, such as the removal of accessory spleens or postoperative complications including postoperative high fever, ACS, and ileus. CONCLUSION:LS is a feasible, safe, and effective surgical procedure alternative to OS for pediatric patients. Compared with OS, LS has the advantage of shorter hospital stay and less blood loss. Besides, total postoperative complications may be slightly lower in LS. We conclude that LS should be considered an acceptable option for children.