Intracranial Tumors In Children Research Articles

Childhood craniopharyngioma: a retrospective study of children followed in Hôpital Universitaire de Bruxelles.

Craniopharyngiomas (CPs) are benign brain tumors accounting for 5 - 11% of intracranial tumors in children. These tumors often recur and can cause severe morbidity. Postoperative radiotherapy efficiently controls and prevents progression and recurrence. Despite advancements in neurosurgery, endocrinological, visual, and neuropsychological complications are common and significantly lower the quality of life of patients. We performed a retrospective study, including all patients younger than sixteen diagnosed with CP between July 1989 and August 2022 and followed up in Hôpital Universitaire de Bruxelles. Nineteen children with CP were included, with median age of 7 years at first symptoms and 7.5 at diagnosis. Common symptoms at diagnosis were increased intracranial pressure (63%), visual impairment (47%), growth failure (26%), polyuria/polydipsia (16%), and weight gain (10.5%). As clinical signs at diagnosis, growth failure was observed in 11/18 patients, starting with a median lag of 1 year and 4 months before diagnosis. On ophthalmological examination, 27% of patients had papillary edema and 79% had visual impairment. When visual disturbances were found, the average preoperative volume was higher (p=0.039). Only 6/19 patients had gross total surgical resection. After the first neurosurgery, 83% experienced tumor recurrence or progression at a median time of 22 months. Eleven patients (73%) underwent postsurgical radiotherapy. At diagnosis, growth hormone deficiency (GHD) was the most frequent endocrine deficit (8/17) and one year post surgery, AVP deficiency was the most frequent deficit (14/17). Obesity was present in 13% of patients at diagnosis, and in 40% six months after surgery. There was no significant change in body mass index over time (p=0.273) after the first six months post-surgery. CP is a challenging brain tumor that requires multimodal therapy and lifelong multidisciplinary follow-up including hormonal substitution therapy. Early recognition of symptoms is crucial for prompt surgical management. The management of long-term sequelae and morbidity are crucial parts of the clinical path of the patients. The results of this study highlight the fundamental importance of carrying out a complete assessment (ophthalmological, endocrinological, neurocognitive) at the time of diagnosis and during follow-up so that patients can benefit from the best possible care.

Craniopharyngioma – case report and literature review

Craniopharyngiomas (Erdheim tumors) are rare tumors with low histological malignancy. Craniopharyngiomas occur at a rate of 0.5 to 2 cases per million people per year, accounting for 30 to 50% of all cases occurring in childhood and adolescence. Craniopharyngioma accounts for 1.2 to 4% of all intracranial tumors in children [1, 2, 3, 4]. Clinical manifestations usually result from increased intracranial pressure, visual disturbances and endocrine disorders. Although long-term survival is high, patients' quality of life is poor, and patients' neuropsychological functions are often impaired. Choosing an appropriate treatment method, is a major challenge and requires the cooperation of physicians from different specialties [5, 6, 7, 8, 9]. Research priorities should be efforts to make information available on cases of Craniopharyngioma, as these tumors are extremely rare. The purpose of this article is to present a case of a boy with a tumor of the periventricular region based on the analysis of medical records and to analyze the current state of knowledge about craniopharyngioma.

Posterior fossa ependymomas in children and adolescents - the state of the art

Central nervous system tumors in pediatric patients represent the most frequent solid tumor type in this age group, especially ependymomas, which comprise 5% of childhood intracranial tumors, and occur mainly in the posterior fossa. The MEDLINE and ClinicalTrials databases were searched for studies on posterior fossa ependymomas, 64 studies met the search criteria. 
 Symptoms include ataxia, vertigo, headache, vomiting, cranial nerve palsies, or papilledema, resulting from intracranial hypertension due to obstruction of CSF circulation. Imaging examination often reveals posterior fossa ependymomas in the fourth ventricle or cerebellopontine angle; they may extend through the foramina of Luschka, Magendie, and/or magnum. These tumors cause a significant mass effect, displacing rather than invading the hidbrain, and may involve vessels or cranial nerves, making surgical resection difficult. Various radiological features have been studied to distinguish between ependymomas and other histological types, as well as between ependymoma subtypes. 
 The 2021 WHO classification of central nervous system tumors classifies ependymomas by location. Three types occur in the posterior fossa: subependymomas, PF-A, and PF-B. These are differentiated by the degree of DNA methylation and CpG islands, which can be assessed by immunohistochemical analysis with H3K27me3. 
 The consensus on the treatment of posterior fossa ependymomas in children advocates the maximum possible surgical resection, followed by radiotherapy in patients >1 year. Chemotherapy remains controversial, with no survival gain. Recent studies suggest molecular subgroups as an independent prognostic factor in posterior fossa ependymomas. The therapeutic challenge of ependymomas requires new treatment options, based on new technologies and molecular discoveries.

Paediatric supratentorial tumours do not cause microstructural alterations in contralateral white matter: a preliminary study.

Intracranial tumours in children can exhibit different characteristics compared to those in adults. Understanding the microstructural changes in the contralateral normal-appearing white matter (NAWM) in children with primary intracranial masses is essential for optimizing treatment strategies. This study aimed to investigate the apparent diffusion coefficient (ADC) changes in contralateral NAWM using fully automated methods and deep learning algorithms. We included 22 paediatric patients with primary supratentorial intracranial masses (23% high-grade) in the study. ADC values of the contralateral NAWM in the patient group were compared to those of a control group. Deep learning algorithms were utilized to analyse diffusion changes in NAWM. The mean ADC values of contralateral NAWM in the patient group were 0.80 ± 0.03 × 10-3 mm2/s, while the control group had a mean ADC value of 0.81 ± 0.03 × 10-3 mm2/s. There was no statistically significant difference between the groups (p = 0.39). Our findings indicate that there are no significant diffusion changes in the contralateral white matter of children with supratentorial intracranial masses. Primary supratentorial intracranial masses in children do not cause microstructural changes in contralateral normal-appearing white matter. This could be attributed to the less infiltrative nature and different biochemical profile of these tumour groups in the paediatric population. Further studies using advanced imaging techniques could provide additional insights into the distinct characteristics of paediatric intracranial tumours and improve patient management.

An update on multimodal management of craniopharyngioma in children.

Craniopharyngioma (CP) represent 1.2-4.6% of all intracranial tumors in children and carry a significant morbidity due to their lesional intimacy with structures involved in neurological, visual, and endocrinological functions. Variable treatment modalities being available, including surgery, radiation therapy, alternative surgeries, and intracystic therapies or combinations of them, their common goal is to reduce immediate and long-term morbidity while preserving these functions. Multiple attempts have been made to re-evaluate surgical and irradiation strategies in order to optimize their complication and morbidity profile. However, despite significant advances in "function sparing" approaches, such as limited surgery and improved technologies of radiation therapies, achieving interdisciplinary consensus on the optimal treatment algorithm remains a challenge. Furthermore, there remains a significant span of improvement given the number of specialties involved as well as the complex and chronic nature of CP disease. This perspective article aims to summarize recent changes and knowledge gains in the field of pediatric CP, outlining updated treatment recommendations, a concept of integrative interdisciplinary care and the implication of novel potential diagnostic tools. A comprehensive update on the multimodal treatment of pediatric CP is presented, focusing on "function-preserving" therapies and their implications.

Endoscopic endonasal approach for craniopharyngiomas in children

Background. The incidence of craniopharyngiomas is up to 15 % of intracranial tumors in children. The endoscopic endonasal approach (EEA) is widely spread nowadays and especially for the craniopharyngiomas removal in adults. Nevertheless, this approach is still not so common in pediatric patients. The endoscopic technique significantly improves the visualization of the structures located around the tumor.Aim. To determine the possibilities and limitations for the use of EEA in the treatment of pediatric patients with cranio pharyngiomas.Materials and methods. A total five pediatric patients with craniopharyngiomas were operated via EEA Federal Center of Neurosurgery of Ministry of Health of Russia (Novosibirsk): the age of patients ranged from 4 to 17 years, including 3 boys and 2 girls. All patients at the preoperative stage already had endocrine disorders in one volume or another. Postoperative evaluation of the visual functions showed the improvement in one patient, three patients remained unchanged and one patient developed worthening. In addition, the volume of the sphenoid sinus was studied, the degree of pneumatization of which determines the anatomical accessibility of the tumor.Results. Our experience of the EEA for the craniopharyngiomas in pediatric patients has shown a positive result regarding to the tumor’s gross‑total removal and no data of the tumor recurrence at the follow‑up. According to our results it can be judged that endonasal endoscopic approach for the craniopharyngiomas in pediatric patients could be a method of choice based on the anatomical accessibility of the tumor and its relationships with the critical neurovascular structures.Conclusions. Endoscopic endonasal removal of craniopharyngiomas in pediatric patients can be successfully used when the tumor is anatomically accessible. The experience of using EED in surgery for craniopharyngiomas in children has shown positive results, including a high radical removal of the tumor and the absence of relapses during further follow‑up of patients.

Pediatric Brain Tumor Risk Associated with Head Computed Tomography: Systematic Literature Review

Computed tomography (CT) of the brain has changed diagnostic neuroradiology significantly over the past 50 years since it was firstly used back in 1971 to visualize suspected frontal lobe tumour. The safety of head CT is determined by the small amount of radiation and the low sensibility of brain tissue to cytotoxic damage due to ionizing radiation compared to other organs. However, some population groups may be at increased risk. Thus, children are more susceptible to radiation cancer than adults and lifelong attributive risk (LAR) can be more than 10 times higher for an infant than for a middle-aged adult. The authors have reviewed published studies that examined the prevalence and mortality of intracranial tumors in children undergoing head CT in comparison to unaffected individuals. Electronic search of publications in the PubMed database from 1966 to date was carried out. We have carried out intersectoral search for documents containing keywords or medical subject headings (MeSH) related to three wide categories: 1) computed tomography, 2) radiation-induced tumors, 3) risk, morbidity or epidemiology. Further search was performed in manual mode. Available epidemiological data generally confirmed correlation between head CT and tumor growth induction. Thus, current epidemiological data accept the opinion that the risk of tumor induction associated with head CT in children is very small (one tumor per 3,000–10,000 studies). The minimal estimated risk of tumor induction due to head CT in children is mostly offset by its diagnostic imaging benefits considering the clinical indications to minimize radiation dose. Understanding and quantitative risk assessment of carcinogenesis associated with CT imaging led to dose reduction in pediatric CT protocols. This trend should continue and should be implemented in all age groups. Although the decision to perform head CT is often undeniable (injury or hemorrhage), careful assessment of studies frequency is required, especially in patients who need disease monitoring. Cumulative effect in such cases may increase the minimal risk of carcinogenesis. Larger and advanced epidemiological studies are required to better understand these risks.

Gene Expression Data Points to a Role for Hypoxia in Medulloblastoma Pathogenesis


 
 
 Background:
 Medulloblastoma (MB) is the most common intracranial tumor in children. While molecular classification of MB is well-established, detailing cell origin, biological properties, and biomarkers, little research has been performed concerning the MB tumor microenvironment. Hypoxia is significantly associated with tumor spread, poor prognosis, malignant phenotype, and resistance to radiotherapy and chemotherapy in numerous cancer types. The aim of the present study was to assess a possible role for hypoxia in MB and the potential effect on clinical outcomes.
 Methods:
 We, therefore, performed a systematic review examining the role of hypoxia in MB as well as pediatric brain tumors in general. In vitro studies have identified a role for HIF-1α in chemotherapy resistance, while patient samples suggest hypoxia-induced changes in gene expression as well as proteomic, metabolomic, and lipidomic profiles. Based on this literature review, 55 candidate hypoxia-related genes were identified. The PedcBioPortal for Integrated Childhood Cancer Genomics was used to assess expression differences in pediatric patient samples for these genes of interest.
 Results:
 RNA expression was analyzed for correlation with survival, molecular group, and Chang stage. Expression of DDAH1, HYOU1, MYC, and RBX1 were significantly associated with survival. ANOVA and Ttest with a Bonferroni correction were used to assess for expression differences between groups and Chang stage. Multiple hypoxia candidate genes (ARNT2, BHLHE40, CYP3A5, DDAH1, DDIT4L, EGLN3, MT3, MYC, MYCN, TGFBR2, TP53, VEGFA) were significantly correlated with molecular group. Expression levels of TGFBR3 and GPR37 were associated with Chang stage.
 Conclusion and Potential Impact:
 The results of our systematic review and gene expression analyses support a role for hypoxia in the pathogenesis and potentially the clinical outcomes of children with MB. Future studies comparing gene expression levels at normal oxygen tension (21%) and physiologic oxygen tension (1-3%) will allow us to assess the role of hypoxia in medulloblastoma pathogenesis.
 
 

Prognostic value of Ki-67 index in primary intracranial tumors of infants.

Primary intracranial tumors are rare tumors in infants. They differ from those found in other pediatric age groups in terms of clinical presentation, histopathological diagnosis, adjuvant therapies, and outcome. Ki-67 index has also shown promising results as a prognostic factor in different types of intracranial tumors in children and adults. However, the importance and the best cutoff point of Ki-67 index in primary intracranial tumors of infants remains unclear. We aimed to analyze prognostic value of Ki-67 index in primary intracranial tumors of infants. This study retrospectively reviewed the records of 28 infants undergoing surgical resection for primary intracranial tumors between April 2016 and March 2021. We analyzed clinical characteristics, tumor location, extent of resection, histopathological diagnosis, Ki-67 index, and overall survival (OS). To define the most relevant cutoff value for Ki-67 index, "Cutoff Finder" was used. The median age at diagnosis was 188days for all patients. Fifteen of the patients were boys and 13 were girls. Tumors were located supratentorial in 13 patients and infratentorial in 15 patients. Gross total resection was performed in 7 of 13 supratentorial tumors and 9 of 15 infratentorial tumors. The mean Ki-67 index of the supratentorial tumors was 49.6%, the median was 55%; for infratentorial tumors, the mean was 49.9%, and the median was 70%. Tumor grade (p = 0.019) and Ki-67 index (p = 0.003) were found as significant predictors of OS in log-rank testing for Kaplan-Meier survival analysis. Univariate cox regression analysis identified high Ki-67 index as prognostic factor for worse OS, with hazard ratio of 8.852 (95% CI 1.95-64.80; p = 0.0108). High Ki-67 index was found as independent prognostic factor for worse OS in multivariate cox regression analysis (HR 7.036; 95% CI 1.229-65.82; p = 0.0457). High-grade and high Ki-67 index were associated with worse outcome. Ki-67 index did show a distinct prognostic value for OS within our cohort at a cutoff value of 72.5%.

Endoscopic transnasal surgery in optic pathway gliomas located in the chiasma-hypothalamic region: case series of ten patients in a single-center experience and endoscopic literature review.

Optic pathway gliomas (OPGs) constitute approximately 3-5% of childhood intracranial tumors. In this study, the authors presented their experience of using the endoscopic endonasal approach to treat patients with OPG located in the chiasma-hypothalamic region and aimed to use the infrachiasmatic corridor in the endoscopic endonasal approach as an alternative to the transcranial approach in the surgical necessity of OPGs. We retrospectively analyzed the data of ten patients diagnosed with OPG histopathologically among 3757 cases who underwent endoscopic endonasal surgery between August 1997 and March 2021 at Kocaeli University Faculty of Medicine Pituitary Research Center and Department of Neurosurgery. Mean follow-up period 48.5months. During the postoperative follow-up period, 3 of these 10 patients underwent reoperation due to tumor recurrence. Combined (endoscopic endonasal approach + transcranial approach) approach was applied to 2 patients in the same session. Surgical and clinical outcomes were evaluated in detail. Ten patients with a mean patient age of 20.6 ± 11.4 were included in this study. The most common complaint was visual impairment. After surgery, improvement in visual impairment was observed in five patients. No increase in postoperative visual impairment was observed in any of the patients. Postoperative panhypopituitarism was not observed in any of the patients. STR resection was performed in 5 patients and NTR resection in 5 patients. No additional treatment was required during follow-up in 4 of 5 patients who underwent NTR. A total of 6 patients received postoperative radiotherapy treatment. In gliomas located in the chiasma-hypothalamic region, appropriate patient selection and endoscopic endonasal surgical treatment may contribute to the elimination of symptoms due to the mass effect of the tumor. It may also contribute to keeping the disease under control with targeted adjuvant therapies by clarifying the pathological diagnosis of the lesion.

RONC-01. A 10 year, single institution experience of re-irradiation for paediatric intracranial tumours

INTRODUCTION: Re-irradiation has become integral in the management of relapsed and recurrent intracranial tumours in children. It is used as salvage therapy for a number of tumours including; diffuse intrinsic pontine glioma (DIPG), high grade glioma (HGG), ependymoma and medulloblastoma. We report the patient demographics, dose, outcomes and toxicity for patients treated with re-irradiation at our institute. METHODS: 33 patients with a diagnosis of DIPG (n=11, 33%), ependymoma (n=11, 33%), HGG (n=4, 12%) or medulloblastoma/sPNET (n=7, 21%), treated with intracranial re-irradiation since 2012 were analysed in this retrospective study. Statistical survival analysis was performed. RESULTS: The median follow-up was 19 months (range 0–216 months). The median age at re-irradiation was 10 years (2-20 years). The median time from first radiation to re-radiation was 34 months (range 3–113 months). Re-irradiation techniques included; photons (n=26), protons (n=2) and stereotactic radiotherapy (n=6). The median re-irradiation dose was 36.37Gy EQD2 (range 20-95.05Gy), given in dose per fraction between 1.8Gy–2Gy. The median overall survival (OS) and progression free survival (PFS) for the cohort was 15.68 months and 7 months respectively. For DIPG, the median OS and PFS were 6.12 months and 4 months respectively. At 2 years, the OS and PFS rates for the cohort were 34.37% and 32.45% respectively. For the non-DIPG cohort, the 2 year OS and PFS rates were 55.37% and 48.13% respectively. In the DIPG cohort, 88% (n=7) had a reported symptomatic benefit from re-irradiation. A stable or improved radiological response was reported in majority of patients (78%, n=18). No acute or late toxicities ≥ grade 3 were reported within the cohort, specifically, there were no reports of brainstem necrosis. CONCLUSION: Re-irradiation for paediatric intracranial tumours is safe and offers a benefit to patients. Further work is ongoing to evaluate cumulative brainstem doses and predictive variables.

Clinical and Epidemiological Study of Intracranial Tumors in Children and Identification of Diagnostic Biomarkers for the Most Common Tumor Subtype and Their Relationship with the Immune Microenvironment Through Bioinformatics Analysis.

Brain tumors are the second most common pediatric malignancy and have poor prognosis. Understanding the pathogenesis of tumors at the molecular level is essential for clinical treatment. We conducted a retrospective study on the epidemiology of brain tumors in children based on clinical data obtained from a neurosurgical center. After identifying the most prevalent tumor subtype, we identified new potential diagnostic biomarkers through bioinformatics analysis of the public database.All children (0-15years) with brain tumors diagnosed histopathologically between 2010 and 2020 at the Department of Neurosurgery, Xijing Hospital, were reviewed retrospectively for age distribution, sex predilection, native location, tumor location, symptoms, and histological grade, and identified the most common tumor subtypes. Two datasets (GSE44971 and GSE44684) were downloaded from the Gene Expression Omnibus database, whereas the GSE44971 dataset was used to screen the differentially expressed genes between normal and tumor samples. Gene ontology, disease ontology, and gene set enrichment analysis enrichment analyses were performed to investigate the underlying mechanisms of differentially expressed genes in the tumor. Combined with methylation data in the GSE44684 dataset, we further analyzed the correlation between methylation and gene expression levels. Two algorithms, LASSO and SVM-RFE, were used to select the hub genes of the tumor. The diagnostic value of the hub genes was assessed using the receiver operating characteristic (ROC) curve. Finally, we further evaluated the relationship between the hub gene and the tumor microenvironment and immune gene sets.Overall, 650 children from 18 provinces in China were included in this study. The male-to-female ratio was 1.41:1, and the number of patients reached a peak in the 10-15-year-old group (41.4%).The most common symptoms we encountered in our institute were headache and dizziness 250 (28.2%), and nausea and vomiting 228 (25.7%). The predominant location is supratentorial, with a supratentorial to infratentorial ratio of 1.74:1. Low-grade tumors (WHO I/II) constituted 60.9% of all cases and were predominant in every age group. According to basic classification, the most common tumor subtype is pilocytic astrocytoma (PA). A total of 3264 differentially expressed genes were identified in the GSE44971 dataset, which are mainly involved in the process of neural signal transduction, immunity, and some diseases. Correlation analysis indicated that the expression of 45 differentially expressed genes was negatively correlated with promoter DNA methylation. Next, we acquired five hub genes (NCKAP1L, GPR37L1, CSPG4, PPFIA4, and C8orf46) from the 45 differentially expressed genes by intersecting the LASSO and SVM-RFE models. The ROC analysis revealed that the five hub genes had good diagnostic value for patients with PA (AUC > 0.99). Furthermore, the expression of NCKAP1L was negatively correlated with immune, stromal, and estimated scores, and positively correlated with immune gene sets.This study, based on the data analysis of intracranial tumors in children in a single center over the past 10years, reflected the clinical and epidemiological characteristics of intracranial tumors in children in Northwest China to a certain extent. PA is considered the most common subtype of intracranial tumors in children. Through bioinformatics analysis, we suggested that NCKAP1L, GPR37L1, CSPG4, PPFIA4, and C8orf46 are potential biomarkers for the diagnosis of PA.

Cordoid Meningioma A Case Report

Introduction: Meningiomas are tumors of the central nervous system derived from the arachnoid layer of cells, represent less than 3% of all primary intracranial tumors in children, are prevalent in adolescents, and are rare in younger ages. Pediatric cases represent approximately 1.5% of all intracranial meningiomas, and very few cases show aggressive histology. The WHO classification meningioma divided into three grades: Grade I benign; II, atypical; and III, anaplastic / malignant. Chordoid meningioma grade II / atypical meningioma is a rare subtype, which represents only 0.5% of all meningiomas, have high growth rates, higher recurrence and greater possibilities to invade the brain parenchyma compared with meningiomas benign. One possible explanation for the high recurrence rate may be related to the quality mucoid stroma, which facilitates extension of tumor and makes it difficult to achieve a complete resection, which leads to subsequent recurrence. Case Report: We report the case of male patient 4 years of age diagnosed with meningioma Grade II, with clinic 2 months of evolution who presented progressive neurological deterioration, tumoral exeresis was performed in 3 opportunities and placement system ventriculo peritoneal in the course of 1 month and radiotherapy. Comments: Accurate diagnosis, therefore, assumes importance because these tumors have an aggressive clinical course and high probability of recurrence so knowing the pathological Clinical Aspects of this rare neoplasm is essential for the Treatment and Comprehensive monitoring.

A Newborn With a Mass on the Brain.

A Newborn With a Mass on the Brain.

PAEDIATRIC POSTERIOR FOSSA TUMORS : AN EPIDEMIOLOGICAL AND ANALYTICAL STUDY AT TERTIARY CARE CENTRE IN EASTERN INDIA

Introduction: Posterior fossa is the commonest site of primary intracranial tumors in children, accounting for 45-60% of 1 all pediatric tumors . The aims and objectives of the study is to analyse the incidence, clinical features, surgical outcome and complications in paediatric patients with posterior fossa tumor. Material and Methods: The present study is a non-randomized prospective observational study, conducted in the department of neurosurgery, Bangur Institute of Neurosciences (B.I.N), IPGME & R, Kolkata from January 2019 to December 2020. Sample size is 50. Observations & Results: Out of 480 cases of total CNS tumors who presented in the study period, 96 cases(20%) were of paediatric posterior fossa tumors. Male dominance was seen i.e. 32 cases(64%). Most of them were in the age group 5-12 years i.e. 30 cases(60%). Headache and vomiting was the most common presenting complain present in 41 cases(82%). Fourth Ventricle was the most common location, 30 cases(60%) with Medulloblastoma being the most common tumor, 24 cases(48%). Brainstem involvement was seen in 22 cases(44%). Post-op hydrocephalus and cerebellar mutism were seen in 6 cases(12%) each. Overall mortality was 8%(4 cases). Conclusion: Posterior fossa tumors are critical brain lesions with signicant neurological morbidity and mortality. Early diagnosis of posterior fossa tumors is vital to prevent potential risks of Brain stem compression, herniation, hydrocephalus and death. With rapid advancement in radiology and the advent of modern therapeutic modalities, early diagnosis and treatment reduced the morbidity and mortality rate and improved prognosis among the patients.

Posterior fossa tumors in children, Histopathology &extent of excision as prognostic factors

The Posterior fossa is the commonest site of primary intracranial tumors in children, for the last two decades the over-all survival and 5-years progression-free survival of children with posterior fossa tumors (PFT) like Medulloblastoma& Ependymomas has been doubled due to the improvement in the diagnostic tools and the advances in the surgical techniques approaching total or near total resection. The aim of the study is to find the relation of histopathology and the extent of excision with mortality and survival. A total of twenty eight cases with Histologically (26 cases) and two cases radiologically (CT-scan and MRI) confirmed pediatric posterior fossa tumors treated in Erbil Teaching Hospital between ,Jan.2013 and Dec.2015 were included in the study As a result the twenty-eight pediatric patients were included in the study, mean age was (8 years),16 boys and 12 girls, mean follow-up period was 14 months,11 cases had Medulloblastoma (39%), 5 cases had Ependymoma (18%), 9 cases had Astrocytoma(32%) , 2 cases had Brainstem mass (7%) and one case had Choroid plexus papilloma (4%).Tumor resection was performed in 26 patients, Twenty cases had total resection (77%), and six Pts .had subtotal resection (23%), two cases without surgery During the follow-up period out of the 20 cases that had total resection nineteen are still alive(95%) and only one died (5%),six cases that had Subtotal resection; four of them are dead (67%) and only two cases are still alive(33%). Two cases that had no surgery both of them are dead (100%).Eleven cases of Medulloblastoma 8 of them had gross total resection and three of them had subtotal resection, 9 of them still alive (82%) and two are dead (18%). Nine Pts of Astrocytoma, 8 of them had total resection and one subtotal; eight of them are still .(alive (89%) and only one died (11%Five pts with Ependymoma, three had total resection and two had subtotal resection, three are still alive (60%) and two dead (40%). Two cases of Brainstem mass not operated and both are dead, One case of Choroid papilloma totally resected & still alive. In conclusion treatment of posterior fossa tumors in children with surgery yields long survival rates, children with gross total resection or a near total resection had better outcome. Histopathology subtypes of the tumors were associated with a favorable outcome for Astrocytoma which has less mortality and better survival rate than others

WITHDRAWN: Pediatric Giant Craniopharyngioma: Surgical Field Soak in Diluted Nimodipine Solution Reduces Cerebral Vasospasm

Craniopharyngioma is a slow-growing benign tumor, accounting for 5.6%-13% of primary intracranial tumors in children. The present patient showed a huge cyst, and computed tomography scan of the brain revealed a 9.2-× 8.7-× 7.0-cm giant cystic lesion with calcification located in the suprasellar cistern. A review of the literature showed the present case is the biggest craniopharyngioma reported. After the tumor was removed, vasospasm appeared in the surgical area. The surgical field was soaked in diluted nimodipine solution for 12 minutes, including the exposed nerve and blood vessel, especially the optic nerve and middle cerebral artery, which led to vasospasm recovery. At 3-month follow-up, there was slight subdural hygroma; contrast magnetic resonance imaging scan did not reveal any residual tumor or recurrence.

Identification of Hub Genes in Atypical Teratoid/Rhabdoid Tumor by Bioinformatics Analyses.

Atypical teratoid/rhabdoid tumor (ATRT) is a devastating intracranial tumor in children. Currently, its molecular mechanisms cannot be studied effectively because patient samples are limited, and many factors are involved in its pathogenesis. In this study, we analyzed three gene expression profile data sets obtained from the Gene Expression Omnibus (GEO) database to identify genes that participate in ATRT. The datasets were integrated and analyzed using the RobustRankAggreg method to screen for differentially expressed genes (DEGs). We identified 197 DEGs, including 94 downregulated and 103 upregulated genes which were then used for gene set enrichment analysis. The results showed that the downregulated genes were mainly enriched in synaptic vesicle cycle, nicotine addiction, and GABAergic synapse, whereas the upregulated genes were enriched in the cell cycle, p53 signaling pathway, and cellular senescence. Consistent with these results, gene set enrichment analysis showed that E2F targets, G2M checkpoints, and MYC targets were significantly enriched in datasets. Protein-protein interaction (PPI) network revealed that CDK1, CCNA2, BUB1B, CDC20, KIF11, KIF20A, KIF2C, NCAPG, NDC80, NUSAP1, PBK, RRM2, TPX2, TOP2A, and TTK were hub genes. NetworkAnalyst algorithm was used to predict the transcription factor (TF), and the results showed that MYC, SOX2, and KDM5B could regulate these hub genes. In conclusion, the present study brings a new perspective of ATRT pathogenesis and the strategy targeted to cell cycle related gene may be promising treatments for the disease.

Approach to the patient with pancytopenia secondary to panhypopituitarism after craniopharyngioma surgery

Craniopharyngioma is the most common benign intracranial tumor in children. The major post-operative complication is dysfunction of pituitary, which can result in many complicate clinical manifestations with hormonal deficiencies. Normochromic anemia has been reported as a common hematologic abnormality. However, pancytopenia is rarely reported so far. Here we described a 21-year-old inpatient with the main complaint of nasal bleeding, who received craniopharyngioma surgery 9 years ago. Laboratory tests showed pancytopenia secondary to panhypopituitarism. This paper aims to increase the awareness of this disease and accumulate clinical experiences for the clinicians. Key words: Craniopharyngioma; Hypopituitarism; Pancytopenia

Pediatric pituitary adenomas.

Pediatric pituitary adenomas are a rare medical entity that makes up a small portion of intracranial tumors in children and adolescents. Although benign, the majority of these lesions are secreting functional tumors with the potential for physiological sequela that can profoundly affect a child's development. In this review, we discuss the medical and surgical management of these tumors with a focus on clinical presentation, diagnostic identification, surgical approach, and associated adjuvant therapies. We will also discuss our current treatment paradigm using endoscopic, open, and combined approaches to treat these tumors. The management of pituitary tumors requires a multidisciplinary team of surgeons, endocrinologists, and neuroanesthesiologists as well as neurocritical care specialists to deliver comprehensive care.