Asunto(s)
Neoplasias Encefálicas , Proteínas de Unión al ARN , Humanos , Masculino , Femenino , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/metabolismo , Neoplasias Encefálicas/genética , Preescolar , Estudios Retrospectivos , Proteínas de Unión al ARN/metabolismo , Proteínas de Unión al ARN/genética , Proteína Ácida Fibrilar de la Glía/metabolismo , Proteína Ácida Fibrilar de la Glía/genética , Nestina/metabolismo , Nestina/genética , Lóbulo Parietal/patología , Lóbulo Parietal/metabolismo , Proteínas del Tejido Nervioso/metabolismo , Proteínas del Tejido Nervioso/genética , Proteínas Cromosómicas no Histona/metabolismo , Proteínas Cromosómicas no Histona/genética , Neoplasias del Tronco Encefálico/patología , Neoplasias del Tronco Encefálico/genética , Neoplasias del Tronco Encefálico/metabolismo , Neoplasias del Tronco Encefálico/cirugía , Factores de Transcripción/metabolismo , Factores de Transcripción/genética , Proteína SMARCB1/metabolismo , Proteína SMARCB1/genética , Lóbulo Frontal/patología , Lóbulo Frontal/metabolismo , Formación de Roseta , Estudios de Seguimiento , Antígeno Ki-67/metabolismo , Proteínas de Unión al ADN/metabolismo , Proteínas de Unión al ADN/genéticaRESUMEN
This letter to the editor discusses the findings of Yu et al. (2024), which highlight the prognostic significance of volumetric assessments over cross-product measurements in pediatric diffuse intrinsic pontine glioma (DIPG). The study's methodology enhances precision in monitoring therapeutic responses, offering insights into treatment adjustments based on detailed imaging features. Emphasizing the value of volumetric MRI, this letter suggests its potential to improve surgical planning and therapeutic strategies, thereby optimizing patient management. This approach could revolutionize treatment paradigms, emphasizing personalized care through advanced imaging techniques.
Asunto(s)
Neoplasias del Tronco Encefálico , Glioma Pontino Intrínseco Difuso , Imagen por Resonancia Magnética , Humanos , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Neoplasias del Tronco Encefálico/cirugía , Glioma Pontino Intrínseco Difuso/terapia , Niño , Pronóstico , Glioma/diagnóstico por imagen , Glioma/terapia , Resultado del TratamientoRESUMEN
BACKGROUND: Diffuse intrinsic pontine gliomas are aggressive tumors that carry a poor prognosis with a 2-year survival rate of <10%. The imaging appearance is often pathognomonic, and surgical biopsy is not mandatory to initiate treatment in children. Studies of biopsy samples provide insight into the disease's molecular pathobiology and open prospects for targeted therapy. This study was conducted to determine the diagnostic yield and safety of stereotactic biopsies. METHODS: This is a prospective observational study from a single tertiary health care center. All patients with clinical and radiological features diagnostic of diffuse intrinsic pontine gliomas (DIPGs) who underwent biopsy from July 2018 to June 2023 were included. Biopsies were performed using either stereotactic frame-based, frameless, or endoscopic techniques. RESULTS: A total of 165 patients with DIPGs were evaluated in the study period. The option of biopsy with its associated risks and benefits was offered to all patients. A total of 76 biopsies were performed in 74 patients (40 children and 34 adults, including 2 repeat biopsies). The median age was 15 years. Diffuse midline gliomas, H3K27M altered, was the most common histopathological diagnosis (85% pediatric and 55.9% adults). The diagnostic efficacy of the procedure was 94.7%. The complication rate was 10.8%, with no permanent neurological deficits due to surgery. There was no procedure-related mortality. CONCLUSIONS: Establishing the safety of the procedure could be an important step toward popularizing the concept, which might offer a better understanding of the disease. Brainstem eloquence and a lack of direct benefit to patients are the primary obstacles to brainstem biopsy.
Asunto(s)
Neoplasias del Tronco Encefálico , Glioma Pontino Intrínseco Difuso , Humanos , Masculino , Femenino , Adolescente , Niño , Neoplasias del Tronco Encefálico/patología , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Adulto , Estudios Prospectivos , Biopsia/métodos , Biopsia/efectos adversos , Adulto Joven , Glioma Pontino Intrínseco Difuso/patología , Preescolar , Persona de Mediana Edad , Técnicas Estereotáxicas/efectos adversos , Puente/patologíaRESUMEN
An 8-year-old boy presenting with left-angle paralysis, tremor in upper and lower extremities, and diplopia was diagnosed with hemorrhage from a mesencephalic cavernous hemangioma. He underwent hemangiomectomy through the occipital transtentorial approach 4 weeks post-hemorrhage, after which Holmes tremor (HT) markedly reduced. A year later, hemangioma has not recurred; he is now independent in his daily activities. Early intervention in the subacute stage allows for the complete removal of brainstem cavernomas (BSCs), with minimal risk of complications or sequelae. Proper timing and surgical approach for BSCs can prevent re-bleeding and improve HT after an initial hemorrhage, without any lasting negative consequences.
Asunto(s)
Neoplasias del Tronco Encefálico , Hemangioma Cavernoso del Sistema Nervioso Central , Temblor , Humanos , Masculino , Niño , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/complicaciones , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Temblor/etiología , Temblor/cirugía , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Hemangioma Cavernoso del Sistema Nervioso Central/complicaciones , Hemangioma Cavernoso del Sistema Nervioso Central/diagnóstico por imagen , Hemangioma Cavernoso/cirugía , Hemangioma Cavernoso/complicaciones , Hemangioma Cavernoso/diagnóstico por imagen , Procedimientos Neuroquirúrgicos/métodos , Tronco Encefálico/cirugía , Tronco Encefálico/diagnóstico por imagenRESUMEN
BACKGROUND: Brainstem cavernous malformations (BCMs) are benign lesions that typically have an acute onset and are associated with a high rate of morbidity. The selection of the optimal surgical approach is crucial for obtaining favorable outcomes, considering the different anatomical locations of various brainstem lesions. Endoscopic surgery is increasingly utilized in treating of BCMs, owing to its depth illumination and panoramic view capabilities. For intra-axial ventral BCMs, the best surgical options are endoscopic endonasal approaches, following the "two-point method. For cavernous hemangiomas on the dorsal side of the brainstem, endoscopy proves valuable by providing enhanced visualization of the operative field and minimizing the need for brain retraction. METHODS: In this review, we gathered data on the fully endoscopic approach for the resection of BCMs, and outlined technical notes and tips. Total of 15 articles were included in this review. The endoscopic endonasal approach was utilized in 19 patients, and the endoscopic transcranial approach was performed in 3 patients. RESULTS: The overall resection rate was 81.8% (18/22). Among the 19 cases of endoscopic endonasal surgery, postoperative cerebrospinal fluid (CSF) leakage occurred in 5 cases, with lesions exceeding 2 cm in diameter in 3 patients with postoperative CSF rhinorrhea. Among the 20 patients with follow-up data, 2 showed no significant improvement after surgery, whereas the remaining 18 patients showed significant improvement compared to their admission symptoms. CONCLUSIONS: This systematic literature review demonstrates that a fully endoscopic approach is a safe and effective option for the resection of BCMs. Further, it can be considered an alternative to conventional craniotomy, particularly when managed by a neurosurgical team with extensive experience in endoscopic surgery, addressing these challenging lesions.
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Hemangioma Cavernoso del Sistema Nervioso Central , Humanos , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Neuroendoscopía/métodos , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Tronco Encefálico/cirugía , Resultado del Tratamiento , Procedimientos Neuroquirúrgicos/métodosAsunto(s)
Hemangioma Cavernoso del Sistema Nervioso Central , Mesencéfalo , Procedimientos Neuroquirúrgicos , Humanos , Mesencéfalo/cirugía , Mesencéfalo/diagnóstico por imagen , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Hemangioma Cavernoso del Sistema Nervioso Central/diagnóstico por imagen , Procedimientos Neuroquirúrgicos/métodos , Masculino , Femenino , Imagen por Resonancia Magnética , Adulto , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/diagnóstico por imagenAsunto(s)
Bulbo Raquídeo , Microcirugia , Humanos , Bulbo Raquídeo/cirugía , Bulbo Raquídeo/diagnóstico por imagen , Microcirugia/métodos , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Hemangioma Cavernoso del Sistema Nervioso Central/diagnóstico por imagen , Procedimientos Neuroquirúrgicos/métodos , Imagenología Tridimensional/métodos , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Masculino , FemeninoRESUMEN
BACKGROUND: Ependymal cysts (EC) typically present supra-tentorially near the lateral ventricle, juxta ventricular, or temporoparietal regions. Previous cases have also identified infratentorial EC of the brainstem, cerebellum, and subarachnoid spaces. They are mostly asymptomatic. In this paper, we present the first-ever case of a symptomatic medullary ependymal cyst treated with surgery, along with a comprehensive review of the literature on EC of other parts of the brain stem. CASE DESCRIPTION: This 51-year-old female presented with hearing loss, dizziness, diplopia, and ataxia. Radiographic imaging indicated the presence of a non-enhancing lesion in the medulla with a mass effect on the brainstem. Pathological examination confirmed its characterization as an ependymal cyst. The patient underwent a suboccipital craniotomy for the fenestration of the medullary ependymal cyst under neuro-navigation, Intra-op ultrasound and intra-operative neuro-monitoring. Histopathological examination confirmed the diagnosis of an ependymal cyst. At one month follow-up, her KPS is 90, ECOG PS 1, and her ataxia has improved with complete resolution of diplopia. CONCLUSION: Due to their rarity and potential similarity to other cystic structures, EC may be overlooked or incorrectly diagnosed resulting in mismanagement and surgical disaster. Therefore, a comprehensive understanding and awareness of their distinct characteristics are essential for accurate diagnosis and appropriate management.
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Bulbo Raquídeo , Humanos , Femenino , Persona de Mediana Edad , Bulbo Raquídeo/cirugía , Bulbo Raquídeo/patología , Epéndimo/cirugía , Epéndimo/patología , Quistes del Sistema Nervioso Central/cirugía , Quistes del Sistema Nervioso Central/diagnóstico , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/diagnóstico , Neoplasias Infratentoriales/cirugía , Neoplasias Infratentoriales/diagnóstico , Imagen por Resonancia Magnética , Craneotomía/métodosRESUMEN
INTRODUCTION: Brain cavernomas or cavernous angiomas are a rare vascular malformation in the general population, even more so in pediatric patients. Their incidence in this group is less than 5% of all vascular malformations. They are typically found in the cerebral hemispheres in cortico-subcortical locations and, more rarely, in the brainstem. OBJECTIVE: To describe the diagnosis, treatment, and follow-up of a case involving a pediatric patient with a giant cavernoma in the brainstem at J.P. Garrahan Hospital. MATERIALS AND METHODS: The clinical history of the case was retrieved from the database of J.P. Garrahan Pediatric Hospital. Additionally, a literature search was conducted in high-impact factor journals using the PubMed database. CONCLUSION: Both the authors of this study and experts consulted through the literature agree that, given the eloquence of the affected area and its challenging accessibility, close monitoring and an expectant approach are advisable for such patients. Nevertheless, when the onset of the case warrants it, surgical intervention is deemed necessary in emergency situations and following the acute phase for complete resolution of the pathology.
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Neoplasias del Tronco Encefálico , Hemangioma Cavernoso del Sistema Nervioso Central , Humanos , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Neoplasias del Tronco Encefálico/cirugía , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Hemangioma Cavernoso del Sistema Nervioso Central/diagnóstico por imagen , Masculino , Niño , Imagen por Resonancia Magnética , FemeninoAsunto(s)
Bulbo Raquídeo , Microcirugia , Humanos , Bulbo Raquídeo/cirugía , Bulbo Raquídeo/diagnóstico por imagen , Microcirugia/métodos , Procedimientos Neuroquirúrgicos/métodos , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Hemangioma Cavernoso del Sistema Nervioso Central/diagnóstico por imagen , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Imagenología Tridimensional/métodosRESUMEN
BACKGROUND AND OBJECTIVES: Microsurgical resection is the only curative intervention for symptomatic brainstem cavernous malformations (BSCMs), but the management of these lesions in older adults (≥65 years) is not well described. This study sought to address this gap by examining the safety and efficacy of BSCM resection in a cohort of older adults. METHODS: Records of patients who underwent BSCM resection over a 30-year period were reviewed retrospectively. Baseline characteristics and outcomes were compared between older (≥65 years) and younger (<65 years) patients. RESULTS: Of 550 patients with BSCM who met inclusion criteria, 41 (7.5%) were older than 65 years. Midbrain (43.9% vs 26.1%) and medullary lesions (19.5% vs 13.6%) were more common in the older cohort than in the younger cohort ( P = .01). Components of the Lawton BSCM grading system (ie, lesion size, crossing axial midpoint, developmental venous anomaly, and timing of hemorrhage) were not significantly different between cohorts ( P ≥ .11). Mean (SD) Elixhauser comorbidity score was significantly higher in older patients (1.86 [1.06]) than in younger patients (0.66 [0.95]; P < .001). Older patients were significantly more likely than younger patients to have poor outcomes at final follow-up (28.9% vs 13.8%, P = .01; mean follow-up duration, 28.7 [39.1] months). However, regarding relative neurological outcome (preoperative modified Rankin Scale to final modified Rankin Scale), rate of worsening was not significantly different between older and younger patients (23.7% vs 14.9%, P = .15). CONCLUSION: BSCMs can be safely resected in older patients, and when each patient's unique health status and life expectancy are taken into account, these patients can have outcomes similar to younger patients.
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Hemangioma Cavernoso del Sistema Nervioso Central , Microcirugia , Humanos , Anciano , Masculino , Femenino , Microcirugia/métodos , Persona de Mediana Edad , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Adulto , Neoplasias del Tronco Encefálico/cirugía , Procedimientos Neuroquirúrgicos/métodos , Tronco Encefálico/cirugía , Anciano de 80 o más Años , Factores de Edad , Estudios de CohortesAsunto(s)
Hemangioma Cavernoso del Sistema Nervioso Central , Procedimientos Neuroquirúrgicos , Humanos , Hemangioma Cavernoso del Sistema Nervioso Central/cirugía , Hemangioma Cavernoso del Sistema Nervioso Central/diagnóstico por imagen , Procedimientos Neuroquirúrgicos/métodos , Puente/cirugía , Puente/diagnóstico por imagen , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Femenino , Imagen por Resonancia Magnética , Masculino , Cerebelo/cirugía , Cerebelo/diagnóstico por imagen , AdultoRESUMEN
OBJECTIVE: To investigate the treatment plan and prognosis of children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle. METHODS: In this retrospective study, the clinical information of 10 consecutively collected children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle was analyzed. All 10 children underwent pontine tumour resection through a trans-cerebellomedullary fissure approach; 4 children underwent preoperative diffusion tensor imaging scans to determine the relationship between the tumour and facial nerve nucleus, and the other 6 children underwent intraoperative deep electroencephalography (EEG) tumour monitoring, in which the tumour electrical discharge activity of the tumour was recorded. A voxel distribution map was established to describe the distribution of the tumour location, and patient prognosis was evaluated through clinical and imaging follow-up. RESULTS: All 10 children achieved total tumour resection; 9 tumours were pathologically suggested to be ganglioglioma (WHO grade I), and 1 was a hamartoma. The symptoms of the original ocular dyskinesia and hemifacial spasm disappeared immediately after the operation. The children were followed up for 4-75 months, and none of the symptoms recurred; four cases with preoperative diffusion tensor imaging showed that the tumour was close to the facial nerve. Four in six intraoperative electrophysiological monitoring showed that the tumour had electrical discharge behaviour, and the tumour distribution map indicates a high density of tumour presence in the facial nerve nucleus and the nucleus of the abducens nerve. CONCLUSIONS: In paediatric patients, the facial symptoms are related to the location and abnormal electrical discharge of the tumour. There is no significant correlation between ocular dyskinesia and the location of the tumour. Conventional antiepileptic therapy for this disease is ineffective, and early surgical intervention for total tumour resection can achieve a clinical curative effect.
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Espasmo Hemifacial , Humanos , Femenino , Masculino , Espasmo Hemifacial/etiología , Espasmo Hemifacial/cirugía , Niño , Preescolar , Estudios Retrospectivos , Neoplasias del Tronco Encefálico/complicaciones , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Cuarto Ventrículo/cirugía , Cuarto Ventrículo/diagnóstico por imagen , Lactante , Trastornos de la Motilidad Ocular/etiología , Trastornos de la Motilidad Ocular/cirugía , AdolescenteRESUMEN
BACKGROUND: Tectal plate gliomas (TPGs) are a heterogeneous group of uncommon brain tumors. TPGs are considered indolent and are usually managed conservatively but they have the potential to transform into higher-grade tumors. The aims of this study were to investigate the natural history of adult TPG, treatment outcomes, and overall survival. METHODS: A retrospective cohort analysis was performed of adult patients with TPG between 1993 and 2021. Baseline clinical, radiologic, and management characteristics were collected. The primary outcome was tumor progression, defined as increasing size on radiologic assessment or new gadolinium contrast enhancement. Secondary outcomes included management and mortality. RESULTS: Thirty-nine patients were included, of whom 23 (52.2%) were men. Median age at diagnosis was 35 years (interquartile range, 27-53). Radiologic tumor progression was observed in 8 patients (20.5%). The 10-year progression-free survival was 72.6% (95% confidence interval [CI], 0.58-0.91). The 10-year overall survival was 86.5% (95% confidence interval, 0.75-1.0). Cerebrospinal fluid diversion procedures were used in 62% of the cohort (n = 24). Seventeen patients (43.6%) underwent at least 1 endoscopic third ventriculostomy, whereas only 6 patients (15.4%) underwent at least 1 ventriculoperitoneal shunt. CONCLUSIONS: TPG has an overall favorable clinical prognosis, although progression occurs in 1 in 5 patients. Showing accurate factors by which patients with TPG may be risk stratified should be a key area of further research. A follow-up duration of 10 years would be a reasonable window based on the radiologic progression rates in this study; however, larger cohort studies are needed to answer both questions definitively.
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Neoplasias del Tronco Encefálico , Glioma , Hidrocefalia , Masculino , Adulto , Humanos , Femenino , Estudios Retrospectivos , Estudios de Seguimiento , Neoplasias del Tronco Encefálico/cirugía , Glioma/diagnóstico por imagen , Glioma/cirugía , Glioma/patología , Ventriculostomía/métodos , Techo del Mesencéfalo/patología , Hidrocefalia/cirugíaRESUMEN
OBJECTIVE: Surgery for midbrain pilocytic astrocytoma (PA) remains a formidable challenge. To facilitate decision-making and achieve a better outcome in the management of patients with midbrain PA, the authors have proposed a novel radiological classification of midbrain PAs with long-term follow-up. METHODS: Fifty-seven midbrain PA patients who underwent surgery at Beijing Tiantan Hospital, Capital Medical University, from January 2008 to June 2021, were reviewed. Based on tumor location and the topological anatomical change identified on MRI, midbrain PAs were categorized into four types: crural (12/57, 21.1%), tegmental (25/57, 43.9%), aqueductal (5/57, 8.8%), and tectal (15/57, 26.3%) PAs. The relevant clinical, radiological, and pathological data; surgical procedures and results; and long-term outcomes were collected and analyzed. RESULTS: The 1-, 3-, and 5-year survival rates reached 98%, 96%, and 96%, respectively, with gross-total resection achieved in 66.7% of cases, followed by near-total resection in 17.5% cases. The clinical and radiological features, selection of surgical approaches, and long-term postoperative deficits were distinct among each type. Crural PAs were associated with younger age (median 9 years, IQR 5.0-12.8 years); the largest tumor volume (median 31.9 cm3, IQR 17.2-42.6 cm3); the lowest preoperative Karnofsky Performance Scale (KPS) score (median 65, IQR 50-70); the most frequent preoperative motor deficit (91.7%); a mixed solid-cystic component (75%); occupation of the crural cistern; elevation and rotation of the thalamus (medial and/or lateral); displacement of the anterior third ventricle, uncus, and anterior commissure; the most diverse surgical approaches; more frequent use of multimodality image-guided surgery (58.3%); and the most remarkable improvement in KPS score at long-term follow-up. Tegmental PAs were associated with adolescents and young adults (median age 21 years, IQR 8-33 years); tumor volume (median 13.9 cm3, IQR 9.5-20.5 cm3); a good preoperative KPS score (median 80, IQR 70-80); a mixed solid-cystic component (72%); occupation of the ambient cistern and cerebellomesencephalic fissure; a close relationship with the dorsal pons, superior cerebellar peduncle, and posterior inferior third ventricle; and a higher probability of permanent postoperative sensory deficits (40%). Aqueductal and tectal PAs were associated with small tumor volume (median 9.14 cm3, IQR 5.1-17.4 cm3 and median 11.84 cm3, IQR 5.7-18.3 cm3, respectively), a higher percentage of hydrocephalus (80% and 86.7%, respectively), and a straightforward selection of limited surgical approaches. CONCLUSIONS: A novel and comprehensive radiological classification of midbrain PAs was established, which will serve as a valuable tool in patient management and promote uniform communication and comparison across different studies and publications.
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Astrocitoma , Imagen por Resonancia Magnética , Mesencéfalo , Procedimientos Neuroquirúrgicos , Humanos , Astrocitoma/cirugía , Astrocitoma/diagnóstico por imagen , Astrocitoma/patología , Masculino , Femenino , Adulto , Adolescente , Niño , Adulto Joven , Mesencéfalo/diagnóstico por imagen , Mesencéfalo/cirugía , Mesencéfalo/patología , Procedimientos Neuroquirúrgicos/métodos , Persona de Mediana Edad , Preescolar , Estudios Retrospectivos , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Neoplasias del Tronco Encefálico/patología , Resultado del Tratamiento , Estudios de SeguimientoRESUMEN
OBJECTIVE: Brainstem tumors present a significant challenge in surgical treatment, and the prognostic factors in children are lacking. This study aimed to investigate clinical characteristics and prognostic factors of surgical treatment in children with brainstem tumors. PATIENTS AND METHODS: 50 children with brainstem tumors who underwent surgical treatment, including frameless- or frame-based stereotactic biopsy and resection, were included and followed up for clinical and biological analysis. Factors of outcomes were assessed by univariate and multivariate analysis. RESULTS: 27 cases (54.0%) underwent resection in all children with brainstem tumors. The rate of resection reached as high as 81.8% in children with non-diffuse intrinsic pontine glioma (DIPG), while in children with DIPG, biopsy was performed in the majority, and resection was obtained in the minority with focal necrosis. A rare complication was found following the surgery. Multivariate analysis considered World Health Organization (WHO) grade 3-4, with hazard ratio (HR)=4.48, 95% confidence interval (CI) of 2.84-8.69, p=0.001, H3K27M mutation (HR=2.50, 95% CI 1.73-5.69, p=0.015), and hydrocephalus (HR=2.17, 95% CI 1.08-5.32, p=0.014) as independent adverse prognostic factors. For Kaplan-Meier analysis, children with WHO grade 3-4, Ki-67 LI ≥ 20%, TP53 mutation, H3K27M mutation, DIPG, and hydrocephalus had significantly decreased overall survival (OS). CONCLUSIONS: A high rate of resection has been obtained in non-DIPG, and surgical intervention is remarkably safe and efficient for children with brainstem tumors. WHO grade 3-4, H3K27M mutation, and hydrocephalus indicate poor prognosis in children with brainstem tumors.
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Neoplasias del Tronco Encefálico , Glioma , Hidrocefalia , Humanos , Niño , Glioma/patología , Pronóstico , Neoplasias del Tronco Encefálico/genética , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/patología , BiopsiaRESUMEN
BACKGROUND: Brainstem cavernomas occasionally require surgical treatment. Appropriate patient selection and thorough understanding of the anatomy and technical nuances involved in microsurgical resection is a pre-requisite in undertaking these challenging cases. CASE DESCRIPTION: We present a video case of a patient with a recurrent haemorrhagic pontine cavernoma. A step-by-step commentary of surgical footage is provided along with clinical, anatomical and technical learning points pertinent to the safe surgical management of these lesions.
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Neoplasias del Tronco Encefálico , Hemangioma Cavernoso , Humanos , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Neoplasias del Tronco Encefálico/cirugía , Neoplasias del Tronco Encefálico/patología , Microcirugia , Hemangioma Cavernoso/cirugía , Puente/diagnóstico por imagen , Puente/cirugía , Puente/patología , Hemorragia/cirugíaRESUMEN
OBJECT: Pediatric diffuse intrinsic pontine glioma (DIPG) is a radiologically heterogeneous disease entity, here we aim to establish a multimodal imaging-based radiological classification and evaluate the outcome of different treatment strategies under this classification frame. METHODS: This retrospective study included 103 children diagnosed with DIPGs between January 2015 and August 2018 in Beijing Tiantan Hospital (Beijing, China). Multimodal radiological characteristics, including conventional magnetic resonance imaging (MRI), diffuse tensor imaging/diffuse tensor tractography (DTI/DTT), and positron emission tomography (PET) were reviewed to construct the classification. The outcome of different treatment strategies was compared in each DIPG subgroup using Kaplan-Meier method (log-rank test) to determine the optimal treatment for specific DIPGs. RESULTS: Four radiological DIPG types were identified: Type A ("homocentric", n=13), Type B ("ventral", n=41), Type C ("eccentric", n=37), and Type D ("dorsal", n=12). Their treatment modalities were grouped as observation (43.7%), cytoreductive surgery (CRS) plus radiotherapy (RT) (24.3%), RT alone (11.7%), and CRS alone (20.4%). CRS+RT mainly fell into type C (29.7%), followed by type B1 (21.9%) and type D (50%). Overall, CRS+RT exhibited a potential survival advantage compared to RT alone, which was more pronounced in specific type, but this did not reach statistical significance, due to limited sample size and unbalanced distribution. CONCLUSION: We proposed a multimodality imaging-based radiological classification for pediatric DIPG, which was useful for selecting optimal treatment strategies, especially for identifying candidates who may benefit from CRS plus RT. This classification opened a window into image-guided integrated treatment for pediatric DIPG.
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Neoplasias del Tronco Encefálico , Glioma Pontino Intrínseco Difuso , Glioma , Niño , Humanos , Glioma/diagnóstico por imagen , Glioma/terapia , Estudios Retrospectivos , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Neoplasias del Tronco Encefálico/cirugía , Imagen MultimodalRESUMEN
INTRODUCTION: Diffuse midline brainstem gliomas have a poor prognosis and are generally not amenable to surgical resection. Occasionally, palliative surgical procedures can be performed to improve the quality of life of these patients. We describe three patients with solid-cystic brainstem gliomas in whom an Ommaya reservoir catheter was placed to reduce mass effect. OBJECTIVES: To describe the characteristics, indications for, and operative technique of Ommaya reservoir catheter placement in patients with solid-cystic diffuse midline glioma. MATERIALS AND METHODS: A review was conducted of the medical records of pediatric patients with solid-cystic diffuse midline glioma H3 K27-altered, treated with an Ommaya reservoir at Hospital J.P. Garrahan between 2014 and 2021 together with a search of the literature. RESULTS: Three cases of stereotaxic Ommaya placement in solid-cystic diffuse midline gliomas, H3 K27M-altered were identified. After the procedure, clinical improvement and reduction of the size of the tumor cyst size was achieved. No associated complications were seen. At the time of the study, one patient died, and the remaining two patients continued in follow-up at our hospital. CONCLUSION: We believe that the placement of an intratumoral Ommaya reservoir catheter may be considered a therapeutic option to improve symptoms and quality of life of selected patients with solid-cystic diffuse midline glioma.
Asunto(s)
Neoplasias Encefálicas , Neoplasias del Tronco Encefálico , Glioma , Humanos , Niño , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/patología , Histonas/genética , Calidad de Vida , Mutación , Glioma/diagnóstico por imagen , Glioma/cirugía , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Neoplasias del Tronco Encefálico/cirugíaRESUMEN
Glial tumors are a relatively new indication for stereotactic radiosurgery (SRS). Traditionally, SRS has been considered to be an inadequate treatment for glial tumors as these are diffuse tumors, but SRS is a highly focused treatment. Tumor delineation can be challenging given the diffuse nature of the gliomas. It has been recommended to include the T2/fluid-attenuated inversion recovery (FLAIR) altered signal intensity areas in addition to the contrast enhancing part in the treatment plan of glioblastoma in order to increase the coverage. Some have recommended to include 5 mm margins to cover up for the diffusely infiltrative nature of the glioblastoma. The most common indication of SRS in patients with glioblastoma multiforme is tumor recurrence. SRS has also been used as a boost to the residual tumor or tumor bed after surgical excision before conventional radiotherapy. The addition of bevacizumab has been recently tried along with SRS in patients with recurrent glioblastoma to decrease radiation toxicity. Besides, SRS has also been used in patients with low-grade gliomas following recurrence. Brainstem gliomas, which are usually low-grade gliomas, are another indication for SRS. Outcomes following the use of SRS are comparable with external beam radiotherapy in brainstem gliomas, whereas the risks of radiation-induced complications is less. SRS has also been used in other glial tumors such as gangliogliomas and ependymomas.