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2.
Pediatr Neurol ; 154: 15-19, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38447508

RESUMO

BACKGROUND: Postoperative pediatric cerebellar mutism syndrome (CMS) may occur following a process affecting the posterior cranial fossa. Recent evidence demonstrates disabling and potentially lasting motor components of this syndrome, including ataxia, hemiparesis, and oculomotor dysfunction. These impairments may contribute to vestibular deficits. METHODS: This case series contributes data to quantify vestibular dysfunction in postoperative CMS. The pair consisted of one female and one male. RESULTS: Vestibular testing demonstrated both peripheral and central dysfunction. CONCLUSIONS: Given these findings, a thorough vestibular assessment may be indicated as part of a comprehensive evaluation following a postoperative CMS diagnosis. Further research is needed to understand the pathophysiology, treatment, and long-term outcomes of postoperative pediatric CMS.


Assuntos
Doenças Cerebelares , Neoplasias Cerebelares , Mutismo , Criança , Humanos , Masculino , Feminino , Mutismo/diagnóstico , Mutismo/etiologia , Neoplasias Cerebelares/cirurgia , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/etiologia , Doenças Cerebelares/diagnóstico , Doenças Cerebelares/etiologia , Fossa Craniana Posterior , Síndrome
3.
Eur J Paediatr Neurol ; 48: 129-141, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38377646

RESUMO

BACKGROUND: Children who underwent posterior fossa tumor removal may have spoken or written language impairments. The present systematic review synthesized the literature regarding the language outcomes in this population. Benefits of this work were the identification of shortcomings in the literature and a starting point toward formulating guidelines for postoperative language assessment. METHODS: A systematic literature search was conducted, identifying studies with patients who had posterior fossa surgery before 18 years of age. Included studies were narratively synthesized to understand language outcomes by language function (e.g., phonology, morphosyntax) at a group and individual level. Furthermore, the influence of several mediators (e.g., postoperative cerebellar mutism syndrome (pCMS), tumor type) was investigated. A critical evaluation of the language assessment tools was conducted. RESULTS: The narrative synthesis of 66 studies showed that a broad spectrum of language impairments has been described, characterized by a large interindividual heterogeneity. Patients younger at diagnosis, receiving treatment for a high-grade tumor and/or radiotherapy and diagnosed with pCMS seemed more prone to impairment. Several gaps in language assessment remain, such as a baseline preoperative assessment and the assessment of pragmatics and morphosyntax. Further, there were important methodological differences in existing studies which complicated our ability to accurately guide clinical practice. CONCLUSION: Children who had posterior fossa surgery seem to be at risk for postoperative language impairment. These results stress the need for language follow-up in posterior fossa tumor survivors.


Assuntos
Neoplasias Encefálicas , Doenças Cerebelares , Neoplasias Cerebelares , Neoplasias Infratentoriais , Mutismo , Criança , Humanos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Neoplasias Encefálicas/complicações , Neoplasias Infratentoriais/cirurgia , Neoplasias Infratentoriais/complicações , Doenças Cerebelares/complicações , Procedimentos Neurocirúrgicos , Mutismo/etiologia , Mutismo/epidemiologia , Mutismo/cirurgia , Neoplasias Cerebelares/complicações , Neoplasias Cerebelares/diagnóstico , Neoplasias Cerebelares/cirurgia
4.
Eur Rev Med Pharmacol Sci ; 28(3): 969-980, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38375704

RESUMO

OBJECTIVE: This study aimed to develop and validate a nomogram and risk stratification system for the overall survival of pediatric patients with medulloblastoma after surgical repair. PATIENTS AND METHODS: In this multicenter, retrospective study, consecutive patients who underwent surgery for medulloblastoma at Shanghai Children's Medical Center and the First Affiliated Hospital of Fujian Medical University from 2010 to 2022 formed the training and external validation datasets, respectively. Univariable and multivariable Cox regression analyses were performed to identify variables associated with mortality in the training dataset. A nomogram prediction model was developed based on independent variables in the multivariable Cox regression analysis to predict the 1-, 3-, and 5-year overall survival. The area under receiver operating characteristic curve (AUC) and calibration curve were used to evaluate the discrimination and calibration of the nomogram. A risk stratification system based on the median risk score was also established to divide patients into two risk groups. RESULTS: In the training dataset, Cox regression analyses identified tumor size, brainstem involvement and chemotherapy as independent predictors for overall survival. The AUC of the nomogram was 0.75 at 1 year, 0. 75 at 3 years, 0.77 at 5 years in the training dataset, 0.74 at 1 year, 0.70 at 3 years, and 0.70 at 5 years in the validation dataset. The calibration curve for the probability of 1-, 3-, and 5-year survival showed good agreement between the nomogram prediction and actual observation in the training and validation datasets. The risk stratification system could perfectly classify patients into two risk groups, and the overall survival in the two groups had a good division. CONCLUSIONS: This low-cost, convenient, and noninvasive nomogram can be translated into clinical practice as a tool for risk stratification and individualized prognosis prediction for children with medulloblastoma.


Assuntos
Neoplasias Cerebelares , Meduloblastoma , Humanos , Criança , Nomogramas , Meduloblastoma/diagnóstico , Meduloblastoma/cirurgia , Estudos Retrospectivos , China/epidemiologia , Fatores de Risco , Neoplasias Cerebelares/diagnóstico , Neoplasias Cerebelares/cirurgia , Medição de Risco
5.
Medicine (Baltimore) ; 103(6): e37162, 2024 Feb 09.
Artigo em Inglês | MEDLINE | ID: mdl-38335432

RESUMO

RATIONALE: Hemangioblastomas occur both sporadically and as an important component of von Hippel-Lindau (VHL) disease. The typical MRI features of hemangioblastoma are cysts with enhanced cystic wall nodules in the cerebellum or lesions with uniform enhancement on the surface or inside the spinal cord. If there is edema around hemangioblastoma, it is easy to be misdiagnosed as brain metastasis on MRI. PATIENT CONCERNS: A 41-year-old male patient was found to have a lump in the pancreas during a health examination 3 months ago. Subsequently, the patient underwent surgical treatment. The postoperative pathology suggests that the pancreatic lesion is a neuroendocrine tumor. The patient subsequently came to our hospital for consultation on further treatment plans. Abnormal signals were found in the right cerebellum during pituitary magnetic resonance imaging (MRI) before the development of a treatment plan for neuroendocrine tumors. Subsequently, the patient underwent cerebellar mass resection surgery. The pathological result after the surgery was hemangioblastoma. DIAGNOSIS: The patient underwent surgery to remove the tumor and was diagnosed with hemangioblastoma by pathological examination. Subsequently, the patient's genetic testing results confirmed the diagnosis of VHL syndrome. INTERVENTIONS: The patient underwent cerebellar mass resection surgery. OUTCOMES: The patient recovered after surgical resection. LESSONS: In this report, we emphasize the atypical MRI manifestations of hemangioblastoma. For patients with VHL syndrome-related hemangioblastoma, genetic testing is necessary for the patient and their family members.


Assuntos
Neoplasias Cerebelares , Hemangioblastoma , Tumores Neuroendócrinos , Doença de von Hippel-Lindau , Adulto , Humanos , Masculino , Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/cirurgia , Hemangioblastoma/diagnóstico por imagem , Hemangioblastoma/cirurgia , Imageamento por Ressonância Magnética , Tumores Neuroendócrinos/patologia , Medula Espinal/patologia , Doença de von Hippel-Lindau/complicações , Doença de von Hippel-Lindau/genética
6.
Childs Nerv Syst ; 40(5): 1339-1347, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38279985

RESUMO

BACKGROUND: Cerebellar mutism (CM) is characterized by a significant loss of speech in children following posterior fossa (PF) surgery. The biological origin of CM remains unclear and is the subject of ongoing debate. Significant recovery from CM is less likely than previously described despite rigorous multidisciplinary neuro-rehabilitational efforts. METHODS: A national multi-centered retrospective review of all children undergoing PF resection in four midsized Canadian academic pediatric institutions was undertaken. Patient, tumor and surgical factors associated with the post-operative development of CM were reviewed. Retrospective identification of PF surgery patients including those developing and those that did not (internal control). RESULTS: The study identified 258 patients across the 4 centers between 2010 and 2020 (mean age 6.73 years; 42.2% female). Overall, CM was experienced in 19.5% of patients (N = 50). Amongst children who developed CM histopathology included medulloblastoma (35.7%), pilocytic astrocytoma (32.6%) and ependymoma (17.1%). Intraoperative impression of adherence to the floor of the 4th ventricle was positive in 36.8%. Intraoperative abrupt changes in blood pressure and/or heart rate were identified in 19.4% and 17.8% of cases. The clinical resolution of CM was rated to be complete, significant resolution, slight improvement, no improvement and deterioration in 56.0%, 8.0%, 20.0%, 14.0% and 2.0%, respectively. In the cohort of children who experienced post-operative CM as compared to their no-CM counterpart, proportionally more tumors were felt to be adherent to the floor of the 4th ventricle (56.0% vs 49.5%), intraoperative extent of resection was a GTR (74% vs 68.8%) and changes in heart rate were noted (≥ 20% from baseline) (26.0% vs 15.9%). However, a multiple regression analysis identified only abrupt changes in HR (OR 5.97, CI (1.53, 23.1), p = 0.01) to be significantly associated with the development of post-operative CM. CONCLUSION: As a devastating surgical complication after posterior fossa tumor surgery with variable clinical course, identifying and understanding the operative cues and revising intraoperative plans that optimizes the child's neurooncological and clinical outcome are essential.


Assuntos
Neoplasias Cerebelares , Neoplasias Infratentoriais , Meduloblastoma , Mutismo , Humanos , Criança , Feminino , Masculino , Estudos Retrospectivos , Mutismo/etiologia , Complicações Pós-Operatórias , Canadá , Neoplasias Infratentoriais/cirurgia , Meduloblastoma/cirurgia , Síndrome , Neoplasias Cerebelares/cirurgia
7.
J Neurosurg Pediatr ; 33(2): 174-178, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38000061

RESUMO

OBJECTIVE: Postoperative cerebellar mutism syndrome (CMS) develops in up to 40% of children with medulloblastoma. The Rotterdam model (RM) has been reported to predict a 66% risk of CMS in patients with a score of ≥ 100. The aim of this study was to retrospectively apply the RM to an independent cohort of patients with newly diagnosed medulloblastoma and study the applicability of the RM in predicting postoperative CMS. METHODS: Participants had to have their first tumor resection at the authors' institution and be enrolled in the SJMB12 protocol (NCT01878617). All participants underwent structured serial neurological evaluations before and then periodically after completing radiation therapy. Imaging was reviewed by the study neurologist who was blinded to CMS status when reviewing the scans and retrospectively applied RM score to each participant. RESULTS: Forty participants were included (14 females and 26 males). Four (10%) patients had CMS. The median age at tumor resection was 11.7 years (range 3.5-17.8 years). Tumor location was midline in 30 (75%), right lateral in 6 (15%), and left lateral in 4 (10%). The median Evans index was 0.3 (range 0.2-0.4), and 34 (85%) patients had an Evans index ≥ 0.3. Five participants required a ventricular shunt. The median tumor volume was 51.97 cm3 (range 20.13-180.58 cm3). Gross-total resection was achieved in 35 (87.5%) patients, near-total resection in 4 (10%), and subtotal in 1. The median RM score was 90 (range 25-145). Eighteen participants had an RM score of ≥ 100, and of these 16.7% (n = 3) had CMS. Of the 22 patients with an RM score < 100, 1 child developed CMS (4.5%, CI 0.1%-22.8%); 3 of the 18 patients with an RM score ≥ 100 developed CMS (16.7%, CI 3.6%-41.4%). The observed rate of CMS in the cohort of children with an RM score ≥ 100 was significantly lower than the observed rate in the original RM cohort (66.7%, CI 51%-80.0%, p < 0.001). A greater risk of CMS in patients with an RM score ≥ 100 could not be confirmed (p = 0.31). CONCLUSIONS: At the authors' institution, the incidence of CMS in patients who had an RM ≥ 100 was significantly lower than the RM cohort. These findings raise questions regarding generalizability of RM; however, fewer cases of CMS and a relatively small cohort limit this conclusion.


Assuntos
Doenças Cerebelares , Neoplasias Cerebelares , Meduloblastoma , Mutismo , Criança , Masculino , Feminino , Humanos , Pré-Escolar , Adolescente , Meduloblastoma/diagnóstico por imagem , Meduloblastoma/cirurgia , Meduloblastoma/epidemiologia , Estudos Retrospectivos , Mutismo/etiologia , Mutismo/diagnóstico , Mutismo/epidemiologia , Doenças Cerebelares/diagnóstico , Doenças Cerebelares/etiologia , Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/cirurgia , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/epidemiologia
8.
Childs Nerv Syst ; 40(1): 239-243, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37594564

RESUMO

Paediatric patients receiving cranial irradiation therapy for brain tumours are at increased risk of cerebrovascular complications. Radiation-induced moyamoya syndrome (MMS) is a well-recognised complication of this. We present a case of an 8-year-old boy with a history of medulloblastoma, who underwent surgical excision followed by post-operative adjuvant oncological treatment. Six years later, he developed cerebellar/intraventricular haemorrhage. He underwent an emergency external ventricular drain (EVD) insertion followed by posterior fossa suboccipital craniotomy. On dural opening, an abnormal vessel was visualised on the surface of the right cerebellar hemisphere, which was not disturbed. No obvious abnormalities were identified intra-operatively. Cerebral catheter angiography confirmed the presence of a right-sided occipital artery (OA) to posterior inferior cerebellar artery (PICA) extracranial to intracranial (EC-IC) bypass with a zone of the distal PICA territory supplied by this EC-IC bypass. A presumed flow aneurysm originated from the bypass in the distal PICA, identified as cause for the haemorrhage. We highlight a rare cause for intracranial haemorrhage in this cohort of patients. Children who have undergone radiotherapy may have exquisitely sensitive cerebral vasculature and need careful vigilance and evaluation for vasculopathic complications following spontaneous haemorrhage.


Assuntos
Doenças Cerebelares , Neoplasias Cerebelares , Aneurisma Intracraniano , Masculino , Humanos , Criança , Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/radioterapia , Neoplasias Cerebelares/cirurgia , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/etiologia , Aneurisma Intracraniano/cirurgia , Cerebelo , Doenças Cerebelares/complicações , Hemorragia
9.
J Neurosurg ; 140(2): 404-411, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-37542443

RESUMO

OBJECTIVE: The purpose of this study was to identify rates of and risk factors for local tumor progression in patients who had undergone surgery or radiosurgery for the management of cerebellar hemangioblastoma and to describe treatments pursued following tumor progression. METHODS: The authors conducted a retrospective single-center review of patients who had undergone treatment of a cerebellar hemangioblastoma with either surgery or stereotactic radiosurgery (SRS) between 1996 and 2019. Univariate and multivariate regression analyses were performed to examine factors associated with local tumor control. RESULTS: One hundred nine patients met the study inclusion criteria. Overall, these patients had a total of 577 hemangioblastomas, 229 of which were located in the cerebellum. The surgical and SRS cohorts consisted of 106 and 123 cerebellar hemangioblastomas, respectively. For patients undergoing surgery, tumors were treated with subtotal resection and gross-total resection in 5.7% and 94.3% of cases, respectively. For patients receiving SRS, the mean target volume was 0.71 cm3 and the mean margin dose was 18.0 Gy. Five-year freedom from lesion progression for the surgical and SRS groups was 99% and 82%, respectively. The surgical and SRS cohorts contained 32% versus 97% von Hippel-Lindau tumors, 78% versus 7% cystic hemangioblastomas, and 12.8- versus 0.56-cm3 mean tumor volumes, respectively. On multivariate analysis, factors associated with local tumor progression in the SRS group included older patient age (HR 1.06, 95% CI 1.03-1.09, p < 0.001) and a cystic component (HR 9.0, 95% CI 2.03-32.0, p = 0.001). Repeat SRS as salvage therapy was used more often for smaller tumor recurrences, and no tumor recurrences of < 1.0 cm3 required additional salvage surgery following repeat SRS. CONCLUSIONS: Both surgery and SRS achieve high rates of local control of hemangioblastomas. Age and cystic features are associated with local progression after SRS treatment for cerebellar hemangioblastomas. In cases of local tumor recurrence, salvage surgery and repeat SRS are valid forms of treatment to achieve local tumor control, although resection may be preferable for larger recurrences.


Assuntos
Neoplasias Cerebelares , Hemangioblastoma , Radiocirurgia , Humanos , Hemangioblastoma/cirurgia , Resultado do Tratamento , Estudos Retrospectivos , Recidiva Local de Neoplasia/cirurgia , Neoplasias Cerebelares/radioterapia , Neoplasias Cerebelares/cirurgia , Seguimentos
10.
Clin Neurol Neurosurg ; 236: 108073, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38091704

RESUMO

INTRODUCTION: The pineal region is a hard-to-reach part of the brain. There is no unequivocal opinion on the choice of a surgical approach to the pineal region. The surgical approaches described differ in both trajectory (infra- and supratentorial, interhemispheric) and size of craniotomy. They have advantages and disadvantages. The minimally invasive lateral occipital infracortical supra-/transtentorial (OICST) approach we have described has all the advantages of the standard supratentorial approach and minimizes its disadvantages, namely, compression and contusion of the occipital lobe. The minimally invasive craniotomy and small surgical corridor facilitate that. METHODS: We describe 11 consecutive patients with various pineal region tumors (7 cases of pineal cysts, 2 cases of pinealocytoma, 1 case of medulloblastoma, and 1 case of meningioma) who were operated on in our hospital using the lateral OICST approach. Preoperative planning was performed using Surgical Theater®. The surgical corridor was formed using a retractor made from half of a syringe shortened according to the length of the surgical corridor. Preoperative lumbar drain was used. RESULTS: The pineal region tumors were completely resected in all cases. The mean craniotomy size was 2.22 × 1.79 cm. No long-term neurological deficits were reported. CONCLUSIONS: The use of semicircular retractors and intraoperative CSF drainage via a lumbar drain allows to form a small surgical corridor to the pineal region via minimally invasive craniotomy. This reduces traction and traumatization of the occipital lobe, as well as minimizes intra- and postoperative risks.


Assuntos
Neoplasias Encefálicas , Neoplasias Cerebelares , Neoplasias Meníngeas , Glândula Pineal , Pinealoma , Neoplasias Supratentoriais , Humanos , Pinealoma/diagnóstico por imagem , Pinealoma/cirurgia , Pinealoma/patologia , Procedimentos Neurocirúrgicos , Neoplasias Supratentoriais/cirurgia , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/patologia , Glândula Pineal/cirurgia , Glândula Pineal/patologia , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/cirurgia , Neoplasias Meníngeas/patologia , Neoplasias Cerebelares/cirurgia
11.
World Neurosurg ; 183: e386-e394, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38154682

RESUMO

BACKGROUND: Maximizing surgical resection of medulloblastoma (MB) affects overall survival; nevertheless, surgical resection remains a because of the infiltrative behavior of this tumor. Several dyes have been tested for improving tumor visualization; however, few reports with different protocols of fluorophores use are available and the results are inconsistent. Hence, we report our experience with sodium fluorescein in MB surgery, aiming to assess the role of this technique on the extent of resection. Furthermore, we performed a literature review of this topic. METHODS: Fluorescence characteristics, extent of resection, and clinical outcome were analyzed in 9 consecutively operated patients with MB. A comprehensive literature search and review for English-language articles concerning fluorescein application in MB was conducted. RESULTS: In our cohort, no side effect related to fluorescein occurred; all tumors presented with an intense or moderate yellow-green enhancement, and fluorescein was judged fundamental in distinguishing tumors from viable tissue in 7 of 9 cases. Gross total resection or near-total resection (i.e., a residual tumor volume <1.5 cm3) was achieved in 8 patients. The review explored the different techniques and surgical interpretations as well as surgical radicality; we did not find a homogenous protocol for fluorescein injection in the published articles. Fluorescence appeared moderate or intense in almost all cases, with a high percentage of usefulness and consensual achievement of a high rate of gross total resection. CONCLUSIONS: Based on these results, we can infer that fluorescein-guided surgical resection is a safe and valuable method for patients with MB.


Assuntos
Neoplasias Encefálicas , Neoplasias Cerebelares , Meduloblastoma , Humanos , Fluoresceína , Meduloblastoma/diagnóstico por imagem , Meduloblastoma/cirurgia , Estudos de Viabilidade , Neoplasias Encefálicas/cirurgia , Procedimentos Neurocirúrgicos/métodos , Corantes Fluorescentes , Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/cirurgia
12.
Childs Nerv Syst ; 40(4): 1031-1044, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38112693

RESUMO

Medulloblastoma, the most common malignant brain tumor in children, presents a complex treatment challenge due to its propensity for infiltrative growth within the posterior fossa and its potential attachment to critical anatomical structures. Central to the management of medulloblastoma is the surgical resection of the tumor, which is a key determinant of patient prognosis. However, the extent of surgical resection (EOR), ranging from gross total resection (GTR) to subtotal resection (STR) or even biopsy, has been the subject of extensive debate and investigation within the medical community. Today, the impact of neurosurgical EOR on the prognosis of medulloblastoma patients remains a complex and evolving area of investigation. The conflicting findings in the literature, the challenges posed by critical surrounding anatomical structures, the potential for surgical complications and neurologic morbidity, and the nuanced interactions with molecular subgroups all contribute to the complexity of this issue. As the field continues to advance, the imperative to strike a delicate balance between maximizing resection and preserving quality of life remains central to the management of medulloblastoma patients.


Assuntos
Neoplasias Encefálicas , Neoplasias Cerebelares , Meduloblastoma , Criança , Humanos , Meduloblastoma/cirurgia , Qualidade de Vida , Procedimentos Neurocirúrgicos , Neoplasias Encefálicas/cirurgia , Neoplasias Cerebelares/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
14.
Acta Neuropathol Commun ; 11(1): 153, 2023 09 25.
Artigo em Inglês | MEDLINE | ID: mdl-37749662

RESUMO

BACKGROUND: One of the most significant challenges in patients with medulloblastoma is reducing the dose of craniospinal irradiation (CSI) to minimize neurological sequelae in survivors. Molecular characterization of patients receiving lower than standard dose of CSI therapy is important to facilitate further reduction of treatment burden. METHODS: We conducted DNA methylation analysis using an Illumina Methylation EPIC array to investigate molecular prognostic markers in 38 patients with medulloblastoma who were registered in the Japan Pediatric Molecular Neuro-Oncology Group and treated with reduced-dose CSI. RESULTS: Among the patients, 23 were classified as having a standard-risk and 15 as high-risk according to the classic classification based on tumor resection rate and presence of metastasis, respectively. The median follow-up period was 71.5 months (12.0-231.0). The median CSI dose was 18 Gy (15.0-24.0) in both groups, and 5 patients in the high-risk group received a CSI dose of 18.0 Gy. Molecular subgrouping revealed that the standard-risk cohort included 5 WNT, 2 SHH, and 16 Group 3/4 cases; all 15 patients in the high-risk cohort had Group 3/4 medulloblastoma. Among the patients with Group 3/4 medulloblastoma, 9 of the 31 Group 3/4 cases were subclassified as subclass II, III, and V, which were known to an association with poor prognosis according to the novel subtyping among the subgroups. Patients with poor prognostic subtype showed worse prognosis than that of others (5-year progression survival rate 90.4% vs. 22.2%; p < 0.0001). The result was replicated in the multivariate analysis (hazard ratio12.77, 95% confidence interval for hazard ratio 2.38-99.21, p value 0.0026 for progression-free survival, hazard ratio 5.02, 95% confidence interval for hazard ratio 1.03-29.11, p value 0.044 for overall survival). CONCLUSION: Although these findings require validation in a larger cohort, the present findings suggest that novel subtyping of Group 3/4 medulloblastoma may be a promising prognostic biomarker even among patients treated with lower-dose CSI than standard treatment.


Assuntos
Neoplasias Cerebelares , Radiação Cranioespinal , Meduloblastoma , Criança , Humanos , Neoplasias Cerebelares/classificação , Neoplasias Cerebelares/patologia , Neoplasias Cerebelares/radioterapia , Neoplasias Cerebelares/cirurgia , Radiação Cranioespinal/efeitos adversos , População do Leste Asiático , Meduloblastoma/classificação , Meduloblastoma/patologia , Meduloblastoma/radioterapia , Meduloblastoma/cirurgia , Prognóstico , Biomarcadores Tumorais , Metilação de DNA
15.
Neurosurg Rev ; 46(1): 246, 2023 Sep 18.
Artigo em Inglês | MEDLINE | ID: mdl-37723329

RESUMO

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.


Assuntos
Neoplasias Cerebelares , Hidrocefalia , Meduloblastoma , Humanos , Criança , Meduloblastoma/complicações , Meduloblastoma/cirurgia , Nomogramas , Hidrocefalia/cirurgia , Período Pós-Operatório , Neoplasias Cerebelares/complicações , Neoplasias Cerebelares/cirurgia
16.
No Shinkei Geka ; 51(5): 858-866, 2023 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-37743337

RESUMO

In the 5th edition of the WHO classification, medulloblastomas, which are representative pediatric brain tumors, are categorized into four groups: WNT, SHH-TP53 wild, SHH-TP53 mutant, and non-WNT/non-SHH, based on their molecular background. While the histopathological findings still hold importance in predicting prognosis, the histopathological classification is no longer utilized in this edition. SHH medulloblastomas are further subdivided into two groups based on the presence or absence of TP53 mutation, as their clinical characteristics and prognosis differ. Group 3 and Group 4 medulloblastomas, recognized as distinct molecular groups in clinical practice, are combined into a single group called "non-WNT/non-SHH", because they lack specific molecular pathway activation. Furthermore, based on methylation profiling, dividing SHH medulloblastoma into four subgroups and non-WNT/non-SHH medulloblastoma into eight subgroups was proposed. Understanding the unique clinical characteristics and prognosis associated with each group is crucial. However, it is important to acknowledge that our current understanding of prognosis is based on treatment approaches guided by clinical risk factors such as postoperative residual tumor volume and the presence of metastatic disease. This molecular-based classification holds promise in guiding the development of optimal treatment strategies for patients with medulloblastoma.


Assuntos
Neoplasias Encefálicas , Neoplasias Cerebelares , Meduloblastoma , Criança , Humanos , Meduloblastoma/diagnóstico , Meduloblastoma/genética , Meduloblastoma/cirurgia , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/cirurgia , Mutação , Neoplasia Residual , Neoplasias Cerebelares/diagnóstico , Neoplasias Cerebelares/genética , Neoplasias Cerebelares/cirurgia
17.
J Neurooncol ; 164(2): 271-286, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37624529

RESUMO

Despite aggressive management consisting of surgery, radiation therapy (RT), and systemic therapy given alone or in combination, a significant proportion of patients with brain tumors will experience tumor recurrence. For these patients, no standard of care exists and management of either primary or metastatic recurrent tumors remains challenging.Advances in imaging and RT technology have enabled more precise tumor localization and dose delivery, leading to a reduction in the volume of health brain tissue exposed to high radiation doses. Radiation techniques have evolved from three-dimensional (3-D) conformal RT to the development of sophisticated techniques, including intensity modulated radiation therapy (IMRT), volumetric arc therapy (VMAT), and stereotactic techniques, either stereotactic radiosurgery (SRS) or stereotactic radiotherapy (SRT). Several studies have suggested that a second course of RT is a feasible treatment option in patients with a recurrent tumor; however, survival benefit and treatment related toxicity of reirradiation, given alone or in combination with other focal or systemic therapies, remain a controversial issue.We provide a critical overview of the current clinical status and technical challenges of reirradiation in patients with both recurrent primary brain tumors, such as gliomas, ependymomas, medulloblastomas, and meningiomas, and brain metastases. Relevant clinical questions such as the appropriate radiation technique and patient selection, the optimal radiation dose and fractionation, tolerance of the brain to a second course of RT, and the risk of adverse radiation effects have been critically discussed.


Assuntos
Neoplasias Encefálicas , Neoplasias Cerebelares , Radiocirurgia , Radioterapia Conformacional , Reirradiação , Humanos , Reirradiação/métodos , Recidiva Local de Neoplasia/radioterapia , Recidiva Local de Neoplasia/cirurgia , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/cirurgia , Radioterapia Conformacional/métodos , Neoplasias Cerebelares/cirurgia
18.
Acta Neurochir (Wien) ; 165(12): 4279-4292, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37535206

RESUMO

BACKGROUND: Tumors of the fourth ventricle are frequently treated pathologies in pediatric neurosurgery. Data regarding predictors for permanent neurological deficits, long-term functional outcomes, cerebellar mutism (CM), the extent of resection (EOR), and oncological outcomes are scarce. We attempt to contribute to this topic with an analysis of our institutional cohort. METHODS: A retrospective single-center study of patients aged ≤ 19 years who underwent primary surgical resection of a fourth ventricular tumor over a 15-year period (2006-2021). Predictors analyzed included age, gender, surgical approach, anatomical pattern, tumor grade, EOR, tumor volume, and others as appropriate. RESULTS: One hundred six patients were included (64 males, mean age 7.3 years). The rate of permanent neurological deficit was 24.2%; lateral tumor extension (p = 0.036) and tumor volume greater than 38 cm3 (p = 0.020) were significant predictors. The presence of a deficit was the only significant predictor of reduced (less than 90) Lansky score (p = 0.005). CM occurred in 20.8% of patients and was influenced by medulloblastoma histology (p = 0.011), lateral tumor extension (p = 0.017), and male gender (p = 0.021). No significant difference between the transvermian and telovelar approach in the development of CM was detected (p = 0.478). No significant predictor was found for the EOR. EOR was not found to be a significant predictor of overall survival for both low-grade and high-grade tumors; however, gross total resection (GTR) was protective against tumor recurrence compared to near-total or subtotal resection (p < 0.001). In addition, survival was found to be better in older patients (≥ 7.0 years, p = 0.019). CONCLUSION: The overall rate of postoperative complications remains high due to the eloquent localization. Older patients (> 7 years) have been found to have better outcomes and prognosis. Achieving GTR whenever feasible and safe has been shown to be critical for tumor recurrence. CM was more common in patients with medulloblastoma and in patients with tumors extending through the foramen of Luschka. The telovelar approach uses a safe and anatomically sparing corridor; however, it has not been associated with a lower incidence of CM and neurological sequelae in our series, showing that each case should be assessed on an individual basis.


Assuntos
Neoplasias Cerebelares , Meduloblastoma , Humanos , Criança , Masculino , Idoso , Quarto Ventrículo/diagnóstico por imagem , Quarto Ventrículo/cirurgia , Estudos Retrospectivos , Procedimentos Neurocirúrgicos/efeitos adversos , Meduloblastoma/cirurgia , Recidiva Local de Neoplasia/cirurgia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Neoplasias Cerebelares/cirurgia , Neoplasias Cerebelares/etiologia , Resultado do Tratamento
19.
Pediatr Blood Cancer ; 70(11): e30613, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37561355

RESUMO

BACKGROUND/OBJECTIVE: In children, medulloblastoma (MB) is the most prevalent posterior fossa tumor. The first line of treatment is maximal safe resection. Therefore, symptoms of ataxia are commonly seen. Training the brain on balance and cognitive tasks makes balance more automatic than without cognitive tasks. The goal was to assess the effectiveness of dual-task practice on balance after MB excision in children with ataxia. METHODS: Thirty children with ataxia after MB resection at Children Cancer Hospital Egypt were randomized into two equal groups. Exercises for mobility, balance, and gait training were given to both groups. The research group underwent a specific dual-task program (balance and cognitive). The program ran 3 days per week for 8 weeks. Children were evaluated before and after the treatment regimen using the Scale of Assessment and Rating of Ataxia (SARA), the HUMAC Balance System, Pediatric Balance Scale, and Functional Independent Measurement. All children's legal guardians signed an ethical agreement. RESULTS: A notable improvement in balance was found in the dual group in Pediatric Balance Scale (PBS) (p = .028) and stability test (p = .0001) in favor of the study group. No discernible difference was observed in the Functional Independent Measurement score among the two groups (p = .158), although there was a statistically significant increase in both groups after treatment. CONCLUSION: Dual-task program is more effective than traditional physical therapy alone in improving balance in children with ataxia after MB resection.


Assuntos
Neoplasias Cerebelares , Meduloblastoma , Humanos , Criança , Marcha , Meduloblastoma/cirurgia , Terapia por Exercício , Ataxia/etiologia , Neoplasias Cerebelares/cirurgia
20.
Arch. esp. urol. (Ed. impr.) ; 76(6): 475-480, 28 aug. 2023. ilus
Artigo em Inglês | IBECS | ID: ibc-224901

RESUMO

Abstract Background: Clear cell renal cell carcinoma (ccRCC) constitutes the most frequently encountered sporadic class of kidney cancer in adults. Recently, a rare form of clear cell kidney cancer known as ccRCC with hemangioblastoma-like features was proposed, with unique immunological characteristics and a good prognosis. The tumor expressed alpha-inhibin and carbonic anhydrase Ⅸ (CA9) as examined by immunohistochemistry. Methods: Herein, we report a clinical instance of ccRCC with hemangioblastoma-like features. A 49-year-old woman presenting with a chief complaint of hematuria underwent a comprehensive and meticulous assessment. Imaging findings indicated the presence of a mass in the right kidney. Subsequently, she underwent a partial nephrectomy. Results: Histopathological analysis of the resected specimen confirmed the presence of ccRCC with hemangioblastoma-like features. The patient was discharged from the hospital six days post-surgery and could resume her daily activities. During a one-year follow-up after surgery, no signs of recurrence were detected. Conclusions: This case demonstrates the importance of including ccRCC with hemangioblastoma-like features in the differential diagnosis of renal masses in patients with hematuria, and suggests partial nephrectomy as an effective treatment modality for this rare subtype of renal cell carcinoma. However, because of the small number of reported cases and insufficient follow-up time, further investigation is necessary to determine the optimal therapeutic approach and to identify the molecular and genetic characteristics of this tumor (AU)


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Carcinoma de Células Renais/patologia , Neoplasias Renais/patologia , Hemangioblastoma/patologia , Neoplasias Cerebelares/patologia , Carcinoma de Células Renais/cirurgia , Neoplasias Renais/cirurgia , Hemangioblastoma/cirurgia , Neoplasias Cerebelares/cirurgia , Imuno-Histoquímica
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