A nomogram for predicting post-operative hydrocephalus in children with medulloblastoma.

Post-operative hydrocephalus is common among children with medulloblastoma after initial tumor resection. This study aimed to establish a novel model for predicting the development of post-operative hydrocephalus in children with medulloblastoma. Only pediatric patients who received initial medulloblastoma resection at Beijing Tiantan Hospital between January 2018 and May 2021 were included in this study. The potential risk factors associated with post-operative hydrocephalus were identified based on multivariate logistic regression and the nomogram. Receiver operating characteristic (ROC) curve were used to evaluate the performance of the nomogram model based on an independent cohort of medulloblastoma patients who underwent surgery from June 2021 to March 2022. A total of 105 patients were included in the primary cohort. Superior invasion (P = 0.007), caudal invasion (P = 0.025), and intraventricular blood ≥ 5 mm (P = 0.045) were significantly related to the development of post-operative hydrocephalus and thus were assembled into the nomogram model. The model accurately predicted post-operative hydrocephalus based on the calibration curve. The area under the ROC curves for the primary and validation cohorts was 0.849 and 0.855, respectively. In total, the nomogram we developed may aid clinicians in assessing the potential risk of pediatric patients with MB developing post-operative hydrocephalus, especially those who would otherwise not have received a diversionary procedure at presentation.

Treatment and outcome of pediatric intracranial ependymoma after first relapse.

INTRODUCTION: Almost 50% of children with intracranial ependymoma experience disease relapse, and their outcomes are extremely poor. The aim of this study was to investigate optimal salvage treatment for pediatric intracranial ependymoma after the first relapse and to identify prognostic factors affecting survival. METHODS: We conducted a retrospective analysis of 159 children who underwent initial treatment for intracranial ependymoma at Beijing Tiantan Hospital from 2013 to 2017. RESULTS: Relapse was observed in 73 patients (73/159; 45.9%), with a median age of 7.2 ± 3.5 years old. Molecular subgrouping analysis identified H3K27me3-negative PF-EPNs in 74% of patients, ST-RELA EPNs in 21% of patients, and H3K27me3-positive PF-EPNs in 5% of patients. The 5-year event-free survival (EFS) and overall survival (OS) rates after first relapse were 21.1% (95% CI 16.0-26.2) and 30.5% (95% CI 19.8-30.8), respectively. Patients with GTR at first relapse had higher 5-year EFS and 5-year OS than those with STR (P = 0.031 and P = 0.003) or no surgery (P = 0.007 and P = 0.001). Radiotherapy or re-radiotherapy at first relapse significantly prolonged 5-year EFS and OS (both P < 0.001). Patients with H3K27me3-negative PF-EPN had worse 5-year EFS and OS than those with ST-RELA EPN (P = 0.001 and P = 0.002). Multivariate analysis showed that both tumor resection and radiotherapy at first relapse had independent prognostic significance for survival (all P < 0.05). CONCLUSION: Children with recurrent intracranial EPN have poor outcomes, and surgery and radiotherapy at first relapse should be encouraged to improve their prognosis.

Radiomics signature for the prediction of progression-free survival and radiotherapeutic benefits in pediatric medulloblastoma.

PURPOSE: To develop and validate a radiomics signature for progression-free survival (PFS) and radiotherapeutic benefits in pediatric medulloblastoma. MATERIALS AND METHODS: We retrospectively enrolled 253 consecutive children with medulloblastoma from two hospitals. A total of 1294 radiomic features were extracted from the region of tumor on the T1-weighted and contrast-enhanced T1-weighted (CE-T1w) MRI. Radiomic feature selection and machine learning modelling were performed to build radiomics signature for the prediction of PFS on the training set. Moreover, the prognostic performance of the clinical parameters was investigated for PFS. The Concordance index (a value of 0.5 indicates no predictive discrimination, and a value of 1 indicates perfect predictive discrimination) was used to measure and compare the prognostic performance of these models. RESULTS: The radiomics signature for the prediction of the PFS yielded Concordance indices of 0.711, 0.707, and 0.717 on the training and held-out test sets 1 and 2, respectively. The radiomics nomogram integrating the radiomics signature, age, and metastasis performed better than the nomogram incorporating only clinicopathological factors (C-index, 0.723 vs. 0.665 and 0.722 vs. 0.677 on the held-out test sets 1 and 2, respectively), which was also validated by the good calibration and decision curve analysis. Further analysis demonstrated that patients with lower value of radiomics signature were associated with better clinical outcomes after postoperative radiotherapy (p < 0.001). CONCLUSION: The radiomics signature and nomogram performed well for the prediction of PFS and could stratify patients underwent postoperative radiotherapy into the high- and low-risk groups with significantly different clinical outcomes.

The role of imaging features and resection status in the survival outcome of sporadic optic pathway glioma children receiving different adjuvant treatments.

Optic pathway glioma (OPG) is a rare brain tumor affecting children, with no standard treatment strategy. This study described the sporadic OPG survival outcomes after surgical treatment and analyzed the role of imaging features and resection status in children receiving different adjuvant treatments. This retrospective study included 165 OPG patients whose clinical information were obtained from the hospital record system. Tumor volume and residual tumor volume were calculated by delineating the lesion area. Kaplan-Meier method and Cox proportional hazards model were conducted to analyze the independent prognosis factor. A total of 165 patients were included in this study. Respectively, the 5-year overall survival (OS) and progression-free survival (PFS) were 87.58% and 77.87%. Residual tumor size and first adjuvant treatment (AT) after surgery were both associated with PFS. In patients with small-size residual tumors, there was no significant difference in PFS between the AT treatment groups. Moreover, age, exophytic cystic components, leptomeningeal metastases, and AT were associated with OS. In patients with exophytic cystic components and those with leptomeningeal metastases, there was no significant difference in OS. Our results revealed that OPG patients could avoid or defer AT by maximized resection. Age ≤ 2 years was a disadvantageous factor for OS. Patients with exophytic cystic components were more likely to benefit from primary surgery, and CT or RT was not beneficial for these patients. Patients with leptomeningeal metastases had a poor prognosis regardless of the treatment they received. Future prospective clinical studies are needed to develop more effective treatment regimens.

Factors impacting time to diagnosis in pediatric CNS tumors in Chinese children.

OBJECTIVE: This study aimed to investigate the factors impacting time to diagnosis in pediatric central nervous system tumors. METHODS: A descriptive, cross-sectional design was used in this study. A self-developed questionnaire for health-seeking behavior and influencing factors was used in children with intracranial tumors. The factors related to time to diagnosis and the long-term prognosis of children were analyzed. RESULTS: A total of 433 families replied to the questionnaire. The median parental interval was 50 days (range 0 ~ 884), the median diagnostic interval was 97 days (range 4 ~ 1646), and the median prediagnostic symptomatic interval (PSI) was 123 days (range 8 ~ 1844). Higher education was associated with a shorter parental interval (mother: P = 0.048; father: P = 0.035). The diagnostic interval was shortened in patients with dizziness (P = 0.022), abnormal eye movement (P = 0.034), or drowsiness (P = 0.021). A shorter PSI was observed in patients who presented with high intracranial pressure such as headache (P = 0.016), dizziness (P = 0.009), or drowsiness (P = 0.023) and those who went to a higher-level health institution or patients who went to neurology or neurosurgery department as the first medical consultation. No statistically significant difference was found in the interval time (parental interval, diagnostic interval, and PSI) regarding patients' outcomes. CONCLUSION: Different time intervals showed different factors influencing the long delay in diagnosing central nervous system tumors, highlighting the need for increased awareness to improve the treatment efficacy.

Prognostic value of H3K27me3 in children with ependymoma.

OBJECTIVE: To investigate the expression of H3K27me3 in different anatomical sites and analyze its prognostic value in children with ependymoma. METHODS: A total of 188 children diagnosed with ependymoma were admitted to the Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, between 2012 and 2017, and regular follow-up was conducted. Expression of H3K27me3 was analyzed by immunohistochemistry and scored semiquantitatively. The prognostic correlation was analyzed by Kaplan-Meier and Cox regression survival analyses. RESULTS: Of the 188 children with ependymoma, 61.7% were male, and the median and average age was five years (0-17 years) and 6.26 years, respectively. There were 65 cases of supratentorial ependymoma, 115 cases of infratentorial ependymoma, and 8 cases of spinal cord ependymoma. The median follow-up time was 39.95 months (0.3-90.19 months). Five-year progression-free survival (PFS) and overall survival (OS) were 48.5% and 61.4%, respectively. Kaplan-Meier univariate survival analysis showed that H3K27me3 expression had significant effects on PFS (P = 0.0003) and OS (P < 0.0001) in infratentorial ependymoma, but only affected OS (P = 0.03) in supratentorial ependymoma. CONCLUSION: In Chinese children, infratentorial ependymoma with incomplete resection and no adjuvant radiotherapy is associated with poor OS. On the other hand, low expression of H3K27me3 indicates poor prognosis of infratentorial ependymoma, but it has no significant prognostic value for supratentorial ependymoma. In addition, high expression of H3K27me3 in spinal ependymoma may indicate a better prognosis.

68Ga-NOTA-Aca-BBN(7-14) PET imaging of GRPR in children with optic pathway glioma.

PURPOSE: Optic pathway glioma (OPG) is a rare neoplasm that arises predominantly during childhood. Its location in a sensitive region involving the optic pathways, onset in young patients and controversial therapy choice make the management of OPG a challenge in paediatric neuro-oncology. In this study we assessed gastrin-releasing peptide receptor (GRPR)-targeted positron emission tomography (PET) imaging in children with OPG, and the application of a PET/MRI imaging-guided surgery navigation platform. METHODS: Eight children (five boys, mean age 8.81 years, range 5-14 years) with suspicion of optic pathway glioma on MRI were recruited. Written informed consent was obtained from all patients and legal guardians. Brain PET/CT or PET/MRI acquisitions were performed 30 min after intravenous injection of 1.85 MBq/kg body weight of 68Ga-NOTA-Aca-BBN(7-14). Four patients also underwent 18F-FDG brain PET/CT for comparison. All patients underwent surgical resection within 1 week. RESULTS: All 11 lesions (100%) in the eight patients showed prominent 68Ga-NOTA-Aca-BBN(7-14) uptake with excellent contrast in relation to surrounding normal brain tissue. Tumour-to-background ratios (SUVmax and SUVmean) were significantly higher for 68Ga-NOTA-Aca-BBN(7-14) than for 18F-FDG (28.4 ± 5.59 vs. 0.47 ± 0.11 and 18.3 ± 4.99 vs. 0.35 ± 0.07, respectively). Fusion images for tumour delineation were obtained in all patients using the PET/MRI navigation platform. All lesions were pathologically confirmed as OPGs with positive GRPR expression, and 75% were pilocytic astrocytoma WHO grade I and 25% were diffuse astrocytoma WHO grade II. There was a positive correlation between the SUV of 68Ga-NOTA-Aca-BBN(7-14) and the expression level of GRPR (r2 = 0.56, P < 0.01, for SUVmax; r2 = 0.47, P < 0.05, for SUVmean). CONCLUSION: This prospective study showed the feasibility of 68Ga-NOTA-Aca-BBN(7-14) PET in children with OPG for tumour detection and localization. 68Ga-NOTA-Aca-BBN(7-14) PET/MRI may be helpful for assisting surgery planning in OPG patients with severe symptoms, GRPR-targeted PET has the potential to provide imaging guidance for further GRPR-targeted therapy in patients with OPG.

Establishing reproducible predictors of cerebellar mutism syndrome based on pre-operative imaging.

PURPOSE: To establish some explicit, feasible, and reproducible predictors for CMS. MATERIALS AND METHODS: This study was a retrospective case study. Data were obtained from 82 patients with medulloblastoma at a single center, Beijing Tiantan Hospital. Based on medical records, we created two independent samples: the CMS group comprising 23 patients and the non-CMS group comprising 23 patients. Pre-operative imaging was studied by performing quantitative assessments of specific indicators. RESULTS: The CMS group showed greater differences in pre-operative imaging data with the non-CMS group. The Aaxi/daxi ratio in pre-operative MR imaging captured in the axial plane was used to quantify the compression of the cerebellum and brainstem, and significant differences were observed between the CMS group and non-CMS group (p = 0.0002). In the sagittal plane, Dsag*dsag was used to quantify the area of the tumor that invaded the brainstem, and significant differences were observed between the two groups (p = 0.0003). In the coronal plane, Acor/dcor was used to quantify the compression of the upper functional brain region, and significant differences were noted between the two groups (p = 0.0219). Additionally, Evans' index was introduced to quantify the degree of hydrocephalus. The CMS group tended to show an increased Evans' index (p = 0.0027). CONCLUSION: Based on pre-operative imaging data, some reproducible predictors, such as Aaxi/daxi, Dsag*dsag, Acor/dcor, and Evans' index, were established.

Gunshot penetrating brain injury in children: report of three cases with review of the literature.

INTRODUCTION: Gunshot penetrating brain injury is common in military conflict area and in urban violence area, but similar incident in pediatric population is rarely reported. CASE REPORT: We reported three cases of gunshot penetrating brain injury in children. Two patients had a good recovery after surgery and no significant deficit on his neurologic function, the other patient was not having surgery due to the severity of the condition. CONCLUSIONS: We suggest surgery should be performed immediately to prevent further injury and refractory brain edema due to the injury, in any case of penetrating brain injury; a good prognosis can be achieved from early surgery and with appropriate post-operative treatment.

Intracranial Rosai-Dorfman disease mimicking multiple meningiomas in a child: a case report and review of the literature.

OBJECTIVE: Rosai-Dorfman disease (RDD) is a rare idiopathic, non-neoplastic histioproliferative disease. Central nervous system (CNS) manifestations are extremely rare. In this paper, we describe a 6-year-old boy with intracranial RDD mimicking multiple meningiomas both clinically and radiologically. We reviewed the literature to understand the clinical behaviour, clinicopathological features and treatment options. METHODS: A PubMed (US National Library of Medicine) search using the keywords 'Rosai-Dorfman disease' and 'central nervous system' was performed and citations were reviewed. RESULTS: Eighty-five cases of RDD involving the CNS have been reported until date, and only 7 cases involved children. Of the 85 cases, 16 cases mimicked multiple meningiomas. Our case is the first to involve multiple lesions in a child under 14 years old. CONCLUSION: After reviewing the literature, we concluded that RDD should be considered as a differential diagnosis for lesions mimicking multiple meningiomas, especially in children. Resection of the intracranial lesion is the most effective treatment, and a definitive diagnosis should be based on histopathologic and immunocytochemical examinations.

Rosai-Dorfman disease involving the central nervous system: seven cases from one institute.

BACKGROUND: Rosai-Dorfman disease (RDD) is a rare, idiopathic, non-neoplastic histioproliferative disease. Central nervous system (CNS) manifestations are extremely rare. Its low incidence and unknown etiology restrict early diagnosis and optimal therapy. METHODS: In the 1995-2013 period, seven CNS-RDD patients with intracranial and/or spinal lesions were retrospectively analyzed, including the clinical data, laboratory and imaging results, treatment applied and outcome. All seven case samples were screened for the SLC29A3 gene mutation, and the literature was reviewed. RESULTS: Seven RDD patients (6 male/1 female, aged between 7 and 68) with CNS manifestations are reported. Five of the seven patients (71.4 %) had intracranial lesions (1 with skull erosion and 1 with multiple lesions mimicking meningiomas), and two (28.6 %) had spinal subdural lesions. The patients' symptoms included headaches, seizures, visual loss, epileptoid convulsions in the lower legs, fever, spastic paraparesis and paraplegia. An elevated erythrocyte sedimentation rate (ESR) was detected in five of the seven cases. The diagnosis was confirmed by immunohistochemical staining revealing that the characteristic histiocytes were positive for the S100 protein and CD68 and negative for CD1a. All patients were operated on: three recovered completely, two were partially rehabilitated, and two died. No SLC29A3 gene mutations were found in any of the seven samples. CONCLUSION: This short series suggests the following: (1) RDD should be included in the differential diagnosis of lesions mimicking intracranial/spinal meningiomas or inflammatory lesions, especially in children; (2) the definitive diagnosis is based on histopathology and immunocytochemistry; (3) surgical resection seems to be the most effective therapy; (4) the exact etiology and adjuvant therapy for relapsing/incompletely resected lesions remain to be established.

The expressions of Wnt/ß-catenin pathway-related components in brainstem gliomas.

BACKGROUND: The overall prognosis of brainstem gliomas is very poor, and the current treatment cannot significantly prolong the overall survival of these patients; therefore, studying the molecular biological mechanisms of the occurrence and development of brainstem gliomas has important significance for their treatment. The Wnt/ß-catenin signaling pathway is closely associated with the occurrence and development of tumors, but its relationship with brainstem gliomas remains unclear. METHODS: This study used Western blot and immunohistochemistry methods to detect the expressions of Wnt/ß-catenin signaling pathway-related components such as Wnt-1, Wnt-2, ß-catenin and C-myc in six cases of normal brain tissues and 24 cases of brainstem gliomas and analyzed the relationship between their expressions and clinicopathological characteristics. RESULTS: Wnt-1 had no obvious expression in normal brain tissues and did not show any significant difference between high- and low-grade brainstem gliomas; the expressions of Wnt-2, ß-catenin and C-myc in high-grade brainstem gliomas were significantly higher than that in low-grade brainstem gliomas and normal brain tissues and were positively correlated with the expression of Ki-67. Moreover, the expressions of Wnt-2 and C-myc were significantly associated with the prognosis of brainstem glioma patients; additionally, there was a trend toward increased ß-catenin expression with shorter survival, but there was no statistical difference. CONCLUSIONS: Wnt/ß-catenin signaling pathway might be abnormally activated and plays an important role in the occurrence and development of brainstem gliomas. Wnt-2, ß-catenin and C-myc may be potential targets for brainstem glioma treatment.

Expression of ß-catenin and E- and N-cadherin in human brainstem gliomas and clinicopathological correlations.

Brainstem gliomas are usually associated with serious dysfunction and poor prognosis especially for diffuse intrinsic brainstem gliomas; however, the reasons are still unclear. Some clinical studies have suggested that the invasive ability may be different among brainstem gliomas, and the dysfunction of ß-catenin and E- and N-cadherin appears to be connected with tumor invasion and progression. In this study, the expression of ß-catenin and E- and N-cadherin was detected in 40 brainstem glioma samples using immunochemistry and was further analyzed in 18 samples using reverse transcription-polymerase chain reaction. The clinicopathological characteristics were also analyzed. The results show that there was no obvious staining for E-cadherin, but weak expression at the messenger RNA (mRNA) level could be seen in a few samples. The protein and mRNA expression levels of ß-catenin and N-cadherin were significantly associated with the pathological grades of brainstem gliomas. No significant differences in the expression levels of ß-catenin and N-cadherin were observed for age, sex, location or diffuse growing pattern. The overall survival of patients with low ß-catenin expression was longer than that with high ß-catenin expression, and there was a trend toward increased expression of N-cadherin with shorter survival; however, both of them had no statistical differences. These results demonstrate that expression of ß-catenin and N-cadherin is associated with the malignant progression of brainstem gliomas but not correlated with the diffuse and invasive growing pattern. ß-catenin and N-cadherin are potential therapeutic targets and prognostic markers for brainstem glioma, which need to be validated in a larger patient cohort.

Corrigendum: The Visual Acuity Outcome and Relevant Factors Affecting Visual Improvement in Pediatric Sporadic Chiasmatic-Hypothalamic Glioma Patients Who Received Surgery.

[This corrects the article DOI: 10.3389/fneur.2020.00766.].

LC-MS-Based Urine Metabolomics Analysis for the Diagnosis and Monitoring of Medulloblastoma.

Medulloblastoma (MB) is the most common type of brain cancer in pediatric patients. Body fluid biomarkers will be helpful for clinical diagnosis and treatment. In this study, liquid chromatography-mass spectrometry (LC-MS)-based metabolomics was used to identify specific urine metabolites of MB in a cohort, including 118 healthy controls, 111 MB patients, 31 patients with malignant brain cancer, 51 patients with benign brain disease, 29 MB patients 1 week postsurgery and 80 MB patients 1 month postsurgery. The results showed an apparent separation for MB vs. healthy controls, MB vs. benign brain diseases, and MB vs. other malignant brain tumors, with AUCs values of 0.947/0.906, 0.900/0.873, and 0.842/0.885, respectively, in the discovery/validation group. Among all differentially identified metabolites, 4 metabolites (tetrahydrocortisone, cortolone, urothion and 20-oxo-leukotriene E4) were specific to MB. The analysis of these 4 metabolites in pre- and postoperative MB urine samples showed that their levels returned to a healthy state after the operation (especially after one month), showing the potential specificity of these metabolites for MB. Finally, the combination of two metabolites, tetrahydrocortisone and cortolone, showed diagnostic accuracy for distinguishing MB from non-MB, with an AUC value of 0.851. Our data showed that urine metabolomics might be used for MB diagnosis and monitoring.

Epidemiological characteristics of central nervous system tumors in children: a 5-year review of 3180 cases from Beijing Tiantan Hospital.

BACKGROUND: To describe the epidemiological characteristics of central nervous system (CNS) tumors in children, based on the neurosurgery department of Beijing Tiantan Hospital. METHODS: From January 2015 to December 2019, 3180 children were histopathologically diagnosed with CNS tumors based on the 2016 World Health Organization (WHO) classification of tumors. Patients were 0 to 15 years old. We analyzed age-related gender preferences, tumor locations, and the histological grades of the tumors. In addition, the epidemiological characteristics of the five most common intracranial tumors were compared to the previous studies. RESULTS: In this study, intracranial and spinal tumors account for 96.4% (3066) and 3.6% (114) of all tumors, with a preponderance of supratentorial tumors (57.9%). Among all pediatric patients, low-grade tumors comprise 67.1% (2 135). The integral gender ratio of males to females is 1.47: 1 and the average age of patients is 7.59 years old. The five most common intracranial tumors are craniopharyngioma (15.4%), medulloblastoma (14.3%), pilocytic astrocytoma (11.8%), diffuse astrocytoma (9.8%), and anaplastic ependymoma (4.8%). CONCLUSIONS: Due to the lack of national data on childhood brain tumors, we used a large nationally representative population sample based on the largest pediatric neurosurgery center in China. We analyzed the data of the past 5 years, reflecting the incidence of CNS tumors in Chinese children to a certain extent, and laying a data foundation for subsequent clinical studies.

Single-Cell RNA Sequencing Unravels Upregulation of Immune Cell Crosstalk in Relapsed Pediatric Ependymoma.

Ependymoma (EPN) is a malignant glial tumor occurring throughout central nervous system, which commonly presents in children. Although recent studies have characterized EPN samples at both the bulk and single-cell level, intratumoral heterogeneity across subclones remains a confounding factor that impedes understanding of EPN biology. In this study, we generated a high-resolution single-cell dataset of pediatric ependymoma with a particular focus on the comparison of subclone differences within tumors and showed upregulation of cilium-associated genes in more highly differentiated subclone populations. As a proxy to traditional pseudotime analysis, we applied a novel trajectory scoring method to reveal cellular compositions associated with poor survival outcomes across primary and relapsed patients. Furthermore, we identified putative cell-cell communication features between relapsed and primary samples and showed upregulation of pathways associated with immune cell crosstalk. Our results revealed both inter- and intratumoral heterogeneity in EPN and provided a framework for studying transcriptomic signatures of individual subclones at single-cell resolution.

METTL3 regulates m6A methylation of PTCH1 and GLI2 in Sonic hedgehog signaling to promote tumor progression in SHH-medulloblastoma.

SHH subgroup medulloblastoma (SHH-MB) is one of the most common malignant pediatric tumors that arises in the cerebellum. Previously, we showed that RNA m6A methylation participates in regulation of cerebellar development. Here we investigate whether dysregulated m6A methylation contributes to tumorigenesis of SHH-MB. We show that high expression of m6A methyltransferase METTL3 associates with worse survival in the patients with SHH-MB. A large number of hypermethylated transcripts are identified in SHH-MB tumor cells by m6A-seq. We find that METTL3 promotes tumor progression via activating Sonic hedgehog signaling. Mechanistically, METTL3 methylates PTCH1 and GLI2 RNAs and further regulates their RNA stability and translation. Importantly, targeting METTL3 by depleting METTL3 expression or treatment with its catalytic inhibitor STM2457 restrains tumor progression. Collectively, this study shows a critical function for METTL3 and m6A methylation in SHH-MB, indicative of a potential role of METTL3 as therapeutic target in SHH-MB.

Accurate cisAB typing is essential to ensure the safety of a transfusion: A case of a cisAB01 neurosurgery pediatric patient and family study.

The rare cisAB subgroups inherited from a single parent are characterized by the activities of dual A and B glycosyltransferases encoded by a gene on one chromosome. The serological complexity of cisAB challenges clinical blood transfusion practice because of misclassification in ABO grouping.

Simultaneous single-trajectory endoscopic biopsy and third ventriculostomy in pediatric pineal region tumors.

Pineal region tumors have different pathological tumors and their optimal management remains controversial. Advancements in neuroendoscopy have led to the ability to simultaneously treat the hydrocephalus and obtain a tissue diagnosis. A retrospective review of 34 patients with pineal region tumors in Beijing Tiantan hospital from the year 2016 to 2018 was undertaken. A single bur hole for both procedures was used successfully in all patients. Once pathologic diagnosis is made, the subsequent management of different tumors is dependent on response to therapy, the tumor markers and original pathology. Follow-up period was 4-26 months. All 34 cases presented with hydrocephalus and increased intracranial pressure manifestations. Elevated blood tumor markers were found in seven cases. The neuroendoscopic biopsy was diagnostic in 32 samples (94.1%) and nondiagnostic (gliosis) in two patients. 21 cases were germinomas, five cases were tectal astrocytomas, two cases were pineoblastomas, two cases were non-germinomatous germ-cell tumours (NG-GCTs) and 1 case immature teratoma and glioblastoma respectively. During the follow-up period, all germinomas but one case with elevated blood α-fetoprotein received craniotomy with a final diagnosis of NG-GCT received radiotherapy and chemotherapy. Four tectal astocytomas, two pineoblastomas and two NG-GCTs received subsequent open surgery due to progressive development, the pathological data was concordant with the initial endoscopic biopsy sample. An additional VP shunt was inserted in one tectal astrocytoma who have hydrocephalus after craniotomy. Except for 18 cases of transient fever and a case with intratumoral hemorrhage, there was no other significant complications, cognitive disorder and no death. The simultaneous single-trajectory endoscopic technique permits not only to control hydrocephalus but also to obtain histological diagnosis with a low incidence of complication and higher safety. Providing meaningful pathological data, endoscopic biopsies could lead to an appropriate management decision. Especially, it is favored as an early step in the management of patients with marker-negative tumors.