Background: Pediatric posterior fossa tumors (PFT) as a significant cause of morbidity and mortality in children and adolescents. This study aimed to investigate the characteristics of the patient population and report the experience in managing and treating children with PFT, as well as their survival outcomes in Kazakhstan.Material and methods: This retrospective study analyzed data from the archives of the Pediatric Neurosurgery Department and included 214 pediatric patients with PFT in the period from January 2015 to December 2020.Results: The study included 214 patients with a mean age of 7.32±4.18 years, of whom 59.81% were males. The most common tumor pathology observed in this study was medulloblastoma (33.18%), followed by pilocytic astrocytoma (32.71%), and ependymoma (15.42%). The 5-year survival rate was significantly associated with age (p = 0.018), histology type (p = 0.000), tumor location (p = 0.000 and p = 0.032), and adjuvant therapy (p = 0.004), but not with sex or tumor volume.Conclusion: The study found similar death rates in children with PFT compared to other population studies, with lethality influenced by tumor location and histology type. The results provide valuable insights into clinical characteristics and outcomes in Kazakhstan, emphasizing the need for improved diagnosis and management of these tumors.
Background: Maffucci syndrome is a rare genetic disorder associated with the development of multiple enchondromas and soft tissue cavernous hemangiomas, as well as an increased risk of malignant tumors. Case Description: Here we report a case of Maffucci syndrome in a patient who presented with a giant left frontal lobe tumor. Molecular genetic analysis of the tumor revealed an isocitrate dehydrogenase (IDH) mutation p.R132H (c.395C>A) mutation in the IDH1 gene and a heterozygous duplication of the CDKN2A genes. Conclusions: The presence of an IDH1 mutation is notable because this mutation is frequently seen in glial tumors and other neoplasms, and its co-occurrence with Maffucci syndrome may represent a novel risk factor for the development of gliomas. This case underscores the importance of genetic testing in patients with Maffucci syndrome who present with central nervous system tumors, as well as the need for further research to understand the relationship between IDH1 mutations and the development of gliomas in this population.
Pineal region tumors are classically presented with vertical gaze palsy, hypothalamic symptoms, or, more often, hydrocephalus, by obstructing the aqueduct. This case report describes a patient with obstructive hydrocephalus and a hearing loss as an initial complaint of pineal region tumor.
The article describes a classic case of olfactory neuroblastoma in a 33-year-old female patient diagnosed with «nasal cavity neoplasm (polyp?)» who was referred to the National Centre for Neurosurgery JSC from the ENT Department of the City Hospital No. 1. The first symptoms of the tumor occurred during her pregnancy. The aggressive tumor has invaded the anterior cranial fossa and spread into the right half of the ethmoid sinuses and the orbit. The tumor diagnostics was complicated by the prevalence of symptoms of a nasal cavity neoplasm. The “olfactory neuroblastoma” diagnosis was verified by clinical observations, MRI, and pathomorphological studies. Histological examination of the tumor showed a typical structure of olfactory neuroblastoma. IHC examination showed an expression of neuronal differentiation markers. The degree of malignancy was determined by Hyams grading.
Background: Glioma patients with mutant isocitrate dehydrogenase have improved survival; this could be in part due to the suppressive effect of mutant IDH on the level of chronic inflammation. This study aimed to prospectively analyze the association of IDH1 mutation status with preoperative levels of blood inflammatory markers: neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), C-reactive protein (CRP), and red cell distribution width (RDW) in gliomas. Patients and methods: Receiver operating characteristic curves for cutoff value determination, various bivariate tests, and survival analyses (Kaplan-Meier curves and Cox regression) were performed. Results: Patients with mutant IDH1 had reduced levels of NLR (P<0.032) and CRP (P<0.008). Moreover, these patients showed better median overall survival compared to those without IDH1 mutation (P<0.000). In univariate analysis, IDH1 mutation status (P<0.000), NLR (P<0.000), PLR (P<0.008), and CRP (P<0.001) were among the factors associated with survival. By multivariate analysis, IDH1 mutation (P<0.044) and NLR<2.65 (P<0.022) remained independent factors associated with better survival; other independent variables were tumor grade (P<0.000) and location in noneloquent area (P<0.015). Conclusion: The obtained results show that IDH1 mutation is associated with lower levels of chronic inflammation that could account for an improved prognosis in this group of patients.
Histologic research is one of the most reliable methods of diagnosis of pathologies of organs and tissues. This method allows estimating both macroscopic and microscopic structural changes in organs and tissues of animals. Data obtained in the course of the pathomorphological research have fundamental value for studying toxic influence of preparations at the stage of preclinical research. Results of histologic structure of internals and tissues of laboratory Sprague Dawley rats are presented in this article. As a result of experiments it has been established that no side effects have been revealed at preparation administration toanimals. “Microfit” biological preparation exerted no negative impact on functional activity of internals of an organism of rats, caused no allergic reactions. The macroscopic research of internals of all experimental animals revealed no pathology after autopsy. Pathomorphological research of the main organs and tissues confirms safety of the structure of tissues. Following the research results, within one month (30 days) of administration of the biological preparation, it has been established that in case of course intragastric administration of “Microfit” preparation to white Sprague Dawley rats in a conditional-therapeutic dose (30 mg/kg) and a dose exceeding the conditional-therapeutic dose by 10 times (300 mg/kg), the preparation has no toxic effect on condition of internals and tissues.
Background: Red cell distribution width (RDW), neutrophil-lymphocyte ratio (NLR), and platelet count (PLT) routinely tested as part of the complete blood count are indicative of systemic inflammation. The prognostic significance of NLR and PLT in cancer was demonstrated in many studies while the role of RDW has been hardly investigated. The present study aimed to assess the association of RDW, NLR, and PLT with survival and tumor grade in glioma patients.Methods: Clinical data from 178 patients with primary gliomas treated in a single institution were retrospectively analyzed. Receiver operating characteristic curves for cutoff value determination, Kaplan-Meier survival analysis, various bivariate tests, and univariate and multivariate Cox regression analyses were performed.Results: Patients with high RDW (>= 13.95) and NLR (>= 4) levels had worse overall survival (OS) (Wilcoxon test, P<0.026 and P<0.003, respectively) while the effect of thrombocytosis (>= 400x10(9)/L) on prognosis was not significant. Besides, a strong association between RDW and NLR was found (Spearman's rho = 0.230, P<0.02; x(2) = 8.887, P<0.03; Mann-Whitney U-test, P<0.017). Moreover, RDW and NLR were significantly associated with tumor grade. In univariate Cox analysis, elevated NLR (hazard ratio, HR 1.385; confidence interval, CI 1.020-1.881, P<0.037), older age (HR 0.452, CI 0.329-0.621, P<0), and higher tumor grade (HR 1.624, CI 1.187-2.223, P<0.002) were associated with poor outcomes. In the multivariate analysis, tumor grade, age, and Karnofsky performance score were identified as being independently prognostic for OS.Conclusion: Preoperative NLR and RDW values can help to evaluate disease progression and outcomes in patients with gliomas, thereby contributing to patient follow-up optimization.