-
World Neurosurgery Dec 2022Bilateral thalamic gliomas (BTGs) are rare central nervous system tumors, and the outcome is usually dismal. BTG often harbors an EGFR mutation; however, a mutation in...
BACKGROUND
Bilateral thalamic gliomas (BTGs) are rare central nervous system tumors, and the outcome is usually dismal. BTG often harbors an EGFR mutation; however, a mutation in H3K27M is rare. We described 5 cases of BTGs harboring concomitant alterations of EGFR and H3K27M and retrospectively analyzed the clinicopathological features and prognosis of this rare entity.
METHODS
Clinical data of patients were retrieved, and immunohistochemistry and molecular analyses were performed. In addition, a systematic review of literature was conducted using PubMed.
RESULTS
Median patient age was 6 years (range, 3-9 years). The male-to-female ratio was 3:2. Tremors and disturbed speech were the main clinical manifestations. All lesions were located at bilateral thalami, and in 3 of 4 patients, the more significant thalamic lesion was on the left. Two patients harbored insertion mutations in exon 20 of EGFR, 1 missense mutation in exon 7 of EGFR, and 2 EGFR amplifications. After a median overall survival of 8 months, 3 patients died as a result of tumor progression.
CONCLUSIONS
Concomitant alterations of EGFR and H3K27M might indicate a new subtype of diffuse midline glioma, H3K27M-altered. In addition, EGFR alterations could provide potential molecular therapeutic strategies to improve the dismal prognosis of BTGs. Due to the rarity of these tumors, more cases must be collected to study the pathogenesis, treatment, and clinical outcomes of BTGs with double alteration phenotypes.
Topics: Female; Humans; Male; Brain Neoplasms; ErbB Receptors; Glioma; Mutation; Prognosis; Retrospective Studies; Supratentorial Neoplasms; Child, Preschool; Child
PubMed: 36195182
DOI: 10.1016/j.wneu.2022.09.118 -
Journal of the Neurological Sciences Jun 2024Sarcoidosis can be associated with stroke. Whether granulomatous vasculitis directly causes stroke in patients with sarcoidosis remains unclear. This systematic review...
BACKGROUND
Sarcoidosis can be associated with stroke. Whether granulomatous vasculitis directly causes stroke in patients with sarcoidosis remains unclear. This systematic review aims to consolidate reports of concurrent sarcoidosis and stroke.
METHODS
Medline and Embase were searched for terms encompassing sarcoidosis and stroke with a censoring date of March 25, 2023. Cases were reviewed by two authors, with the inclusion criteria: biopsy-confirmed systemic sarcoidosis, stroke confirmed by imaging or pathology, clinical description of individual patient history, and English language publications.
RESULTS
Of 1628 articles screened, 51 patients from 49 articles were included (65% male, mean age 41 years). Seventy-one percent of strokes were ischemic and 29% were hemorrhagic. Lesions were supratentorial in 78% of cases, infratentorial in 34%, and multifocal in 45%. Presenting symptoms were variable, with the most common being headache (38%) followed by weakness (35%). 10 patients had recurrent strokes. Stroke was the presenting symptom of sarcoidosis in 65%. 21 patients had brain biopsies. The most common neuropathologic findings were perivascular (33%) or intramural (33%) non-caseating granulomas. On imaging, 32 patients had findings suggestive of neurosarcoidosis, including 35% with evidence of meningeal enhancement. 63% of patients were treated with corticosteroids and/or other immunomodulatory therapy, with varying clinical improvement.
CONCLUSIONS
Stroke associated with sarcoidosis generally follows trends in stroke incidence, with infarction being more common than hemorrhage and male sex carrying a higher risk. Most patients were diagnosed with sarcoidosis during or following their stroke episode. Brain biopsy infrequently shows clear granulomatous vasculitis.
PubMed: 38850770
DOI: 10.1016/j.jns.2024.123080 -
Pediatrics Nov 2018The effect of neonatal cerebellar hemorrhage on neurodevelopmental outcome (NDO) in the absence of supratentorial injury is still largely unknown.
CONTEXT
The effect of neonatal cerebellar hemorrhage on neurodevelopmental outcome (NDO) in the absence of supratentorial injury is still largely unknown.
OBJECTIVE
To evaluate the influence of isolated neonatal cerebellar hemorrhage on cognitive, motor, language, and behavioral NDOs and assess the effect of location and size on outcome.
DATA SOURCES
Embase, Medline, and Scopus were searched from inception to September 30, 2017.
STUDY SELECTION
Studies in which a diagnosis of isolated cerebellar hemorrhage was reported in preterm infants (<32 weeks' gestation) with a standardized NDO at ≥12 months of age were included.
DATA EXTRACTION
Patient characteristics, location, and size of bleeding and NDO (defined as severe [yes or no] on the basis of given cutoff points) in 4 domains were extracted.
RESULTS
Of the 1519 studies identified, 8 were included in final analyses. Of infants with isolated cerebellar hemorrhage, 128 were described (cumulative incidence: 2.3%). The incidence of severe delay in cognition, motor, language, and behavioral development was 38%, 39%, 41%, and 38%, respectively. The overall incidence of severe neurodevelopmental delay in ≥1 domain ranged from 43% to 75% and was most seen in infants with vermis involvement (87%-93%) and with large bleeds (46%-82%).
LIMITATIONS
Different neurodevelopmental scales lead to data heterogeneity, and reporting of data on a group level limited possibilities for an outcome description on an individual level.
CONCLUSIONS
Of infants with isolated cerebellar hemorrhage, 43% to 75% were severely delayed in cognition, motor, language, and/or behavioral development, with the highest incidence with vermis involvement and with large bleeds.
Topics: Cerebral Hemorrhage; Developmental Disabilities; Humans; Incidence; Infant; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases
PubMed: 30341153
DOI: 10.1542/peds.2018-0609 -
Child's Nervous System : ChNS :... Mar 2021Choroid plexus tumors are uncommon intraventricular tumors that develop from the choroid plexus of the central nervous system. Choroid plexus papillomas arising from the...
INTRODUCTION
Choroid plexus tumors are uncommon intraventricular tumors that develop from the choroid plexus of the central nervous system. Choroid plexus papillomas arising from the cerebellopontine angle have been reported to almost exclusively occur in adults and are rarely found in children.
METHODS
We report a systematic review conducted in accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines of SCOPUS and PubMed databases for case reports and case series of choroid plexus papillomas arising in the cerebellopontine angle in the pediatric population and discuss clinical presentation, imaging features, management options, and outcomes. We also report a case managed at our center.
RESULTS
Ten cases of pediatric choroid plexus papillomas arising in the cerebellopontine angle were identified from the systematic review in addition to the case reported here, resulting in a total of eleven cases. The patients' median age was 8 years with a slight female sex predilection (1.2:1). Patients most commonly presented with headache, cerebellar signs, and cranial nerve palsies with median duration of symptoms at 4 months. All patients underwent surgical treatment with majority achieving gross total excision. No deaths were reported at median follow-up of 12 months. Complete neurologic recovery was attained in seven cases while partial recovery was seen in two cases.
CONCLUSION
Choroid plexus papillomas found in the cerebellopontine angle in the pediatric population are extremely rare but they should be considered in the differential diagnosis. Complete surgical resection is the mainstay of treatment with excellent outcomes achievable in majority of patients.
Topics: Adult; Cerebellopontine Angle; Cerebral Ventricle Neoplasms; Child; Choroid Plexus; Choroid Plexus Neoplasms; Female; Humans; Papilloma, Choroid Plexus; Supratentorial Neoplasms
PubMed: 32980905
DOI: 10.1007/s00381-020-04896-w -
Frontiers in Surgery 2022Metastasis of ovarian carcinoma to the central nervous system occurs in <2% of cases and classically localizes within the brain parenchyma. Moreover, leptomeningeal...
Metastasis of ovarian carcinoma to the central nervous system occurs in <2% of cases and classically localizes within the brain parenchyma. Moreover, leptomeningeal spread of these tumors is an exceedingly rare phenomenon. Here, we conduct a systematic review of the current literature on the natural history, treatment options, and proposed pathogenic mechanisms of leptomeningeal carcinomatosis in ovarian carcinoma. We also report a case of a 67-year-old female with stage IV metastatic ovarian serous carcinoma initially confined to the peritoneal cavity with a stable disease burden over the course of three years. Follow-up imaging demonstrated an intracranial lesion, which was resected via craniotomy, and pathology was consistent with the original diagnosis. Three months after surgery, she developed rapidly progressive dizziness, generalized weakness, fatigue, and ataxia. Repeat MRI demonstrated interval development of extensive and diffusely enhancing dural nodularity, numerous avidly enhancing supratentorial and infratentorial lesions, enhancement of the bilateral trigeminal nerves, internal auditory canals, and exit wound from the surgical site into the posterior aspect of the right-sided neck musculature consistent with diffuse leptomeningeal dissemination. The present case highlights that leptomeningeal dissemination of ovarian carcinoma is a potential yet rare consequence following surgical resection of an ovarian parenchymal metastasis. Progressive clinical symptomatology that develops postoperatively in this patient population should prompt urgent workup to rule out leptomeningeal disease and an expedited radiation oncology consultation if identified.
PubMed: 35548192
DOI: 10.3389/fsurg.2022.850050 -
Neuro-oncology Feb 2018Epilepsy is the most common symptom in patients with supratentorial low-grade gliomas (LGGs), which adversely affects the patient's quality of life. Poor seizure control... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Epilepsy is the most common symptom in patients with supratentorial low-grade gliomas (LGGs), which adversely affects the patient's quality of life. Poor seizure control with anti-epileptic therapy is an indication for surgery in these patients. Recent studies have sought to identify predictors of postoperative seizure control after surgical resection of LGG; gross total resection was shown to be a significant predictor in this respect. However, the prognostic value of other factors is not clear.
METHODS
We performed a systematic review and meta-analysis of 23 studies with a combined study population of 2641 patients with LGG, in order to identify potential factors associated with favorable postoperative seizure control. Data were extracted on age and sex of patient, tumor location, tumor histology, type of seizure, seizure duration, extent of resection, and imaging characteristics.
RESULTS
Patients ≥45 years of age achieved better postoperative seizure control (risk ratio [RR], 0.89; 95% CI, 0.81-0.99). Focal seizures were associated with poor seizure control (RR, 1.32; 95% CI, 1.18-1.49) compared with generalized seizures (RR, 0.77; 95% CI, 0.68-0.87). Prolonged history of seizures (≥1 y) had a negative impact on postoperative seizure control (RR, 1.22; 95% CI, 1.10-1.34). Gross total resection was superior to subtotal resection with respect to postoperative seizure control (RR, 0.68; 95% CI, 0.63-0.73).
CONCLUSIONS
This systematic review and meta-analysis identified predictors of postoperative seizure control in patients undergoing surgical resection of LGGs. Our results provide a reference for clinical treatment of LGG-related epilepsy.
Topics: Adult; Brain Neoplasms; Glioma; Humans; Neoplasm Grading; Neurosurgical Procedures; Postoperative Complications; Quality of Life; Seizures; Treatment Outcome
PubMed: 29016869
DOI: 10.1093/neuonc/nox130 -
Neurocritical Care Feb 2018Patients who have undergone intracranial neurosurgical procedures have traditionally been admitted to an intensive care unit (ICU) for close postoperative neurological...
BACKGROUND
Patients who have undergone intracranial neurosurgical procedures have traditionally been admitted to an intensive care unit (ICU) for close postoperative neurological observation. The purpose of this study was to systematically review the evidence for routine ICU admission in patients undergoing intracranial neurosurgical procedures and to evaluate the safety of alternative postoperative pathways.
METHODS
We were interested in identifying studies that examined selected patients who presented for elective, non-emergent intracranial surgery whose postoperative outcomes were compared as a function of ICU versus non-ICU admission. A systematic review was performed in July 2016 using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist of the Medline database. The search strategy was created based on the following key words: "craniotomy," "neurosurgical procedure," and "intensive care unit."
RESULTS
The nine articles that satisfied the inclusion criteria yielded a total of 2227 patients. Of these patients, 879 were observed in a non-ICU setting. The most frequent diagnoses were supratentorial brain tumors, followed by patients with cerebrovascular diseases and infratentorial brain tumors. Three percent (30/879) of the patients originally assigned to floor or intermediate care status were transferred to the ICU. The most frequently observed neurological complications leading to ICU transfer were delayed postoperative neurological recovery, seizures, worsening of neurological deficits, hemiparesis, and cranial nerves deficits.
CONCLUSION
Our systematic review demonstrates that routine postoperative ICU admission may not benefit carefully selected patients who have undergone elective intracranial neurosurgical procedures. In addition, limiting routine ICU admission may result in significant cost savings.
Topics: Brain Neoplasms; Cerebrovascular Disorders; Craniotomy; Critical Care; Humans; Intensive Care Units; Outcome and Process Assessment, Health Care; Patient Admission; Postoperative Care; Postoperative Complications
PubMed: 28808901
DOI: 10.1007/s12028-017-0433-4 -
Chinese Clinical Oncology Apr 2024The role of adjuvant radiotherapy (RT) after gross total resection (GTR) of the World Health Organization (WHO) grade II ependymoma is controversial. Therefore, we aimed... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The role of adjuvant radiotherapy (RT) after gross total resection (GTR) of the World Health Organization (WHO) grade II ependymoma is controversial. Therefore, we aimed to compare the outcomes of adjuvant RT against observation after GTR of WHO grade II ependymoma. We also compared the outcomes of adjuvant RT against observation after subtotal resection (STR) of WHO grade II ependymoma and performed further subgroup analysis by age and tumor location.
METHODS
PubMed and Embase were systematically reviewed for studies published up till 25 November 2022. Studies that reported individual-participant data on patients who underwent surgery followed by adjuvant RT/observation for WHO grade II ependymoma were included. The exposure was whether adjuvant RT was administered, and the outcomes were recurrence and overall survival (OS). Subgroup analyses were performed by the extent of resection (GTR or STR), tumor location (supratentorial or infratentorial), and age at the first surgery (<18 or ≥18 years old).
RESULTS
Of the 4,647 studies screened, three studies reporting a total of 37 patients were included in the analysis. Of these 37 patients, 67.6% (25 patients) underwent GTR, and 51.4% (19 patients) underwent adjuvant RT. Adjuvant RT after GTR was not significantly associated with both recurrence (odds ratio =5.50; 95% confidence interval: 0.64-60.80; P=0.12) and OS (P=0.16). Adjuvant RT was also not significantly associated with both recurrence and OS when the cohort was analyzed as a whole and on subgroup analysis by age and tumor location. However, adjuvant RT was associated with significantly longer OS after STR (P=0.03) with the median OS being 6.33 years, as compared to 0.40 years for patients who underwent STR followed by observation.
CONCLUSIONS
Based on our meta-analysis of 37 patients, administration of adjuvant RT after GTR was not significantly associated with improvement in OS or recurrence in patients with WHO grade II ependymoma. However, due to the small number of patients included in the analysis, further prospective controlled studies are warranted.
Topics: Humans; Ependymoma; Radiotherapy, Adjuvant; Female; Male; Neoplasm Grading; World Health Organization
PubMed: 38644544
DOI: 10.21037/cco-23-136 -
World Neurosurgery Nov 2018We present an illustrative case of pediatric intracranial anaplastic ganglioglioma and systematically reviewed the current reported data of anaplastic ganglioglioma in...
OBJECTIVE
We present an illustrative case of pediatric intracranial anaplastic ganglioglioma and systematically reviewed the current reported data of anaplastic ganglioglioma in the pediatric population.
METHODS
A comprehensive literature search for our review was conducted using PubMed, Scopus, Web of Science, PsycINFO, Cochrane, and Embase databases. The search terms included "ganglioglioma," "anaplastic," "pediatrics," "children," and "intracranial." We used no limitations for date, type, or language. Reports of pediatric patients (age <19 years) with intracranial anaplastic gangliogliomas were included. Baseline patient demographic characteristics, clinical presentations, imaging characteristics, management strategies, and outcomes data were extracted.
RESULTS
We included 24 studies involving 34 patients in the quantitative synthesis. The mean patient age was 9.18 ± 5.46 years (range, 0.16-18). A male predominance was observed (approximate male/female ratio, 1.61:1). Increased intracranial pressure was the most common symptom (n = 19; 55%), followed by seizures (n = 11; 32%). These tumors were most often found in the supratentorial compartment (n = 26; 76%). All 34 patients had undergone surgical removal. The mean follow-up was 22.2 months (range, 2-72). The mean overall survival duration was 43 months (95% confidence interval, 32.45%-55.31%; 1- and 3-year overall survival, 76.6% and 45.5%, respectively). The median event-free survival was 34 months (95% confidence interval, 10.6%-57.3%; 1- and 3-year event-free survival rate, 76.6% and 42.4%, respectively).
CONCLUSIONS
Our results contribute to our understanding of the characteristics of this rare malignant tumor and show that anaplastic ganglioglioma should be considered in the differential diagnosis of intracranial tumors in pediatric patients.
Topics: Adolescent; Brain; Brain Neoplasms; Child; Diagnosis, Differential; Disease Management; Female; Ganglioglioma; Humans; Infant; Male
PubMed: 30098436
DOI: 10.1016/j.wneu.2018.07.278 -
Neuroradiology Jun 2022Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) is a newly recognized brain tumor with genetic abnormalities frequently involving either BRAF or...
PURPOSE
Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) is a newly recognized brain tumor with genetic abnormalities frequently involving either BRAF or FGFR2/FGFR3. There are few publications available about the neuroradiological features of PLNTY. In this systematic review, we assessed the demographic, clinical, and neuroradiological features of PLNTY.
METHODS
Literature data were extracted from database searches in MEDLINE and SCOPUS databases up to June 10, 2021. Studies reporting on pathologically proven PLNTY with neuroradiological findings were included. After reviewing 103 abstracts, 9 articles encompassing 19 cases met the inclusion criteria. We also added five patients from our hospital. The correlations between the presence of "transmantle-like sign" and the following three factors: duration of seizures; tumor size; and pathologically proven cortical dysplasia, were examined.
RESULTS
The median patient age was 15.5 years (range, 5-57 years), and 15/24 (62.5%) were female. All tumors were localized supratentorialy. The main radiological features included cortical or subcortical masses (95.8%) in the temporal lobe (66.7%), calcification (83.3%), well-defined margins (72.7%), solid and cystic components (66.6%), and T2-weighted imaging (T2WI) hyperintensity (50.0%). The duration of seizure was significantly longer (positive vs. negative (median [range]), 24 months [6 - 96 months] vs. 5 months [1 - 12 months], p = 0.042), and the presence of the cortical dysplasia was significantly more frequent (3/8 vs 0/16, p = 0.042) in the patients with transmantle-like sign.
CONCLUSION
PLNTY typically represents a calcified, well-defined mass in the supratentorial cortical or subcortical regions. The radiological findings defined here could facilitate the diagnosis of PLNTY.
Topics: Adolescent; Adult; Brain Neoplasms; Child; Child, Preschool; Female; Humans; Magnetic Resonance Imaging; Male; Malformations of Cortical Development; Middle Aged; Neoplasms, Neuroepithelial; Seizures; Young Adult
PubMed: 35001164
DOI: 10.1007/s00234-021-02879-5