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INTRODUCTION: Atypical teratoid rhabdoid tumor (ATRT) is among the most aggressive central nervous system malignancies. Although rare, this tumor typically afflicts young children and results in mortality within months. Here, we aim to determine key clinical features and treatment options that impact the survival of patients with ATRT. METHODS: From the year 2000 to 2019, 363 patients with ATRT were identified from the Surveillance, Epidemiology, and End Results database. Univariate analysis was used to identify variables that had a significant impact on the primary endpoint of overall survival (OS). Multivariable analysis was then used to identify independent predictors of survival. RESULTS: The median OS of the entire cohort was 13 months. Univariate analysis identified ages between 1 and 3 years, ages between 4 and 17 years, years of diagnosis between 2010 and 2019, and the receipt of treatment to have a significant impact on survival. In multivariable analysis, ages between 1 and 3 years and receipt of treatment were the only significant independent predictors of survival. The median OS was significantly greater in patients who received surgical treatment, chemotherapy, or radiation when compared to those who did not receive any treatment. In general, the receipt of any combination of therapies improved the median OS significantly. The receipt of triple therapy had the greatest impact on survival. DISCUSSION: This study highlights the survival benefit of a multimodal approach in the treatment of ATRT. The use of triple therapy, including surgery, radiation, and chemotherapy, was found to have the greatest survival benefit for patients. Overall, these findings may guide future care for patients with ATRT.
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Neoplasias do Sistema Nervoso Central , Tumor Rabdoide , Teratoma , Criança , Humanos , Pré-Escolar , Lactente , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/patologia , Teratoma/terapia , Teratoma/tratamento farmacológico , Tumor Rabdoide/patologia , Tumor Rabdoide/cirurgia , Terapia CombinadaRESUMO
INTRODUCTION: Grade 3 solitary fibrous tumor, previously known as anaplastic hemangiopericytoma, is a rare and highly malignant intracranial tumor with a limited understanding of its natural history and treatment outcomes. METHODS: We conducted a retrospective analysis using the Surveillance, Epidemiology, and End Results (SEER) database spanning 2000-2019 to evaluate the clinical characteristics and treatment modalities that influence overall survival in this tumor entity. A cohort of 249 patients with intracranial grade 3 solitary fibrous tumors was identified. Univariate and multivariable Cox proportional hazard models were employed to determine significant prognostic factors for overall survival. Kaplan-Meier models were used to visualize survival curves, and a nomogram was constructed to predict survival probabilities at 6- and 12-month following diagnosis. RESULTS: Our findings indicated that patient age (<65 years), localized or regional disease burden, surgical resection, and radiation therapy were significant predictors of better overall survival. Combination therapies showed improved survival, with surgery and radiation therapy having the most significant impact. However, chemotherapy alone or in combination did not demonstrate a significant survival benefit, likely due to the limited sample size. The nomogram provided personalized prognostic predictions based on significant clinical factors. CONCLUSIONS: These data emphasize the importance of surgical resection and radiation therapy in the management of grade 3 solitary fibrous tumors, supporting the use of combination therapies to improve overall survival in this rare and aggressive intracranial neoplasm.
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Hemangiopericitoma , Programa de SEER , Tumores Fibrosos Solitários , Humanos , Estudos Retrospectivos , Masculino , Feminino , Tumores Fibrosos Solitários/terapia , Tumores Fibrosos Solitários/mortalidade , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/epidemiologia , Pessoa de Meia-Idade , Hemangiopericitoma/terapia , Hemangiopericitoma/mortalidade , Hemangiopericitoma/patologia , Hemangiopericitoma/epidemiologia , Idoso , Prognóstico , Adulto , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/mortalidade , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/epidemiologia , Nomogramas , Gradação de Tumores , Estimativa de Kaplan-Meier , Adulto Jovem , Idoso de 80 Anos ou mais , Terapia CombinadaRESUMO
Traumatic brain injury (TBI) affects over 48 million people worldwide each year. Suicide is common in TBI, and there are several known contributing factors, including severe TBI, depression, alcohol use, and male sex. Impulsivity, or the tendency to act quickly with little thought, may be an early predictor of suicidality following TBI. The purpose of this study was to evaluate the risk of suicidality in patients with a prior history of impulsivity following a traumatic brain injury (TBI). Using de-identified electronic health records from the TriNetX United States Collaborative Network with Natural Language Processing, three cohorts were generated: the impulsivity TBI cohort (I+TBI+) included subjects with a diagnosis of impulsivity before a diagnosis of TBI; the no impulsivity TBI cohort (I-TBI+) included patients with TBI but no impulsivity; the impulsivity no TBI cohort (I+TBI-) included patients with impulsivity but TBI. Two analyses were conducted, including analysis 1 (impulsivity TBI vs. no impulsivity TBI) and analysis 2 (impulsivity TBI vs. impulsivity no TBI). Patients were 1:1 propensity score matched by age, sex, race, ethnicity, psychiatric diagnoses, and antidepressant use. Outcomes included a diagnosis of self-harm, suicidal ideation, or a suicide attempt within one year after the index event. The all-time incidence of each outcome was assessed across different age categories. The chi-square test (categorical variables) and t-test (numerical variables) were used to assess for differences between groups. A total of 1,292,776 TBI patients were identified in the study. After 1:1 propensity score-matching, there were 20,694 patients (mean [SD] age, 48.1 [21.8]; 8,424 females [40.7%]) with impulsivity and TBI (I+TBI+), 1,272,082 patients (mean [SD] age, 46.0 [23.1]; 562,705 females [44.2%]) with TBI alone (I-TBI+), and 90,669 patients (mean [SD] age, 43.7 [22.6]; 45,188 females [49.8%]) with impulsivity alone (I+TBI-). Within the first year after a TBI, patients with impulsivity were more likely to exhibit self-harm (P < 0.001), suicidal ideation (P < 0.001) or a suicide attempt (P < 0.001). Compared to TBI patients without impulsivity, those with impulsivity had a four-fold increase in the incidence of self-harm (2.81% vs. 0.63%), an eight-fold increase in suicidal ideation (52.42% vs. 6.41%), and a twenty-one-fold increase in suicide attempts (32.02% vs. 1.50%). This study suggests that impulsivity diagnosed before a TBI may increase the risk of post-traumatic suicidality, with a four-fold increased risk of self-harm, an eight-fold increased risk of suicidal ideation and a twenty-one-fold increased risk of suicide attempts. This characterizes a group of at-risk individuals who may benefit from early psychiatric support and targeted interventions following a TBI.
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OBJECTIVE: The natural history, treatment options, and clinical outcomes of pancreatic metastases to the brain remain largely unknown. Here, we seek to investigate characteristics that influence OS in pancreatic metastases to the brain. METHODS: This is a population-based retrospective study of OS in 508 patients with pancreatic metastases to the brain using the SEER database. Univariate and multivariate Cox regression analyses were utilized, and a predictive nomogram was developed. RESULTS: There were 508 patients identified for this study, with a median OS of 2 months. In the univariate analysis, patients older than 65 years had significantly reduced OS (P < 0.001). Patients with liver metastases (P < 0.001) and liver and lung metastases (P < 0.001) exhibited significantly reduced OS. Treatment of the primary tumor with chemotherapy only (P < 0.001), radiation only (P = 0.01), radiation and chemotherapy (P < 0.001), and surgery only (P = 0.01) were associated with increased OS. Resection of a distant metastasis site (P = 0.009) and of a brain metastasis (P = 0.03) were associated with increased OS. In the multivariable analysis, factors that remained significant included patient age (P = 0.01), liver metastases (P < 0.001), liver and lung metastases (P < 0.001), treatment with chemotherapy (P < 0.001), treatment with radiation and chemotherapy (P < 0.001), and treatment with surgery and chemotherapy (P < 0.001). The nomogram had a C-index of 0.766, suggesting congruence between the findings on the nomogram and the results in the internal verification. CONCLUSIONS: Median OS is influenced by age, multiorgan metastases, and treatment of the primary tumor. These data highlight the marginal benefit of treatment, yet improved quality of life (QOL) remains to be elucidated.
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Neoplasias Hepáticas , Neoplasias Pulmonares , Neoplasias Pancreáticas , Humanos , Prognóstico , Estudos Retrospectivos , Qualidade de Vida , Neoplasias Pancreáticas/terapia , Neoplasias Hepáticas/terapia , Neoplasias Pulmonares/terapia , Programa de SEERRESUMO
Background: Choroid plexus tumors (CPTs) are rare neoplasms found in the central nervous system, comprising 1% of all brain tumors. These tumors include choroid plexus papilloma (CPP), atypical choroid plexus papilloma (aCPP), and choroid plexus carcinoma (CPC). Although gross total resection for choroid plexus papillomas (CPPs) is associated with long-term survival, there is a scarcity of prospective data concerning the role and sequence of neoadjuvant therapy in treating aCPP and CPC. Methods: From the years 2000 to 2019, 679 patients with CPT were identified from the Surveillance, Epidemiology, and End Result (SEER) database. Among these patients, 456 patients had CPP, 75 patients had aCPP, and 142 patients had CPC. Univariate and multivariable Cox proportional hazard models were run to identify variables that had a significant impact on the primary endpoint of overall survival (OS). A predictive nomogram was built for patients with CPC to predict 5-year and 10-year survival probability. Results: Histology was a significant predictor of OS, with 5-year OS rates of 90, 79, and 61% for CPP, aCPP, and CPC, respectively. Older age and African American race were prognostic for worse OS for patients with CPP. Older age was also associated with reduced OS for patients with aCPP. American Indian/Alaskan Native race was linked to poorer OS for patients with CPC. Overall, treatment with gross total resection or subtotal resection had no difference in OS in patients with CPP or aCPP. Meanwhile, in patients with CPC, gross total resection (GTR) was associated with significantly better OS than subtotal resection (STR) only. However, there is no difference in OS between patients that receive GTR and patients that receive STR with adjuvant therapy. The nomogram for CPC considers types of treatments received. It demonstrates acceptable accuracy in estimating survival probability at 5-year and 10-year intervals, with a C-index of 0.608 (95% CI of 0.446 to 0.77). Conclusions: This is the largest study on CPT to date and highlights the optimal treatment strategies for these rare tumors. Overall, there is no difference in OS with GTR vs. STR in CPP or aCPP. Furthermore, OS is equivalent for CPC with GTR and STR plus adjuvant therapy.
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Background: The coexistence of meningioma and dural arteriovenous fistula (dAVF) is a rare, but highly complex condition. Various pathophysiological mechanisms underlie intracranial meningiomas with continuous or distant dAVFs. We describe a case of coexisting meningioma and dAVF with a systematic review of the literature. Result: Including the present case, there are 21 reported cases of coexisting intracranial dAVF and meningioma. The patients' ages ranged from 23 to 76 years, with a mean age of 61 years. The most common presenting symptom was headache. The dAVFs were commonly located at the transverse-sigmoid sinus (43%) and superior sagittal sinus (24%). The most common meningioma locations were the tentorium and parietal convexity. In 76% of the cases, the meningioma occluded the sinus. The most common dAVF treatment was transcatheter arterial embolization, followed by tumor resection (52%). Among the 20 cases with available outcome data, 90% reported favorable outcomes. Conclusion: This report highlights some of the features of coexisting dAVF and meningioma and presents a systematic review of other reports on this phenomenon. Through an in-depth analysis of the literature, we highlight some of the leading theories regarding the causes of concomitant dAVF and meningiomas. Our report supports one of the leading theories that impaired venous return, whether through the occlusion of sinuses or sinus manipulation during surgery, plays a role in the development of dAVF. Further understanding may help guide future clinical decision-making and surgical planning.
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OBJECTIVE: The authors sought to investigate the association between white blood cell counts and acute hydrocephalus in spontaneous nonaneurysmal subarachnoid hemorrhage (nSAH). METHODS: We conducted a retrospective analysis of 105 consecutive patients with spontaneous nSAH. Univariate and multivariable logistic regression analyses were performed to investigate factors associated with hydrocephalus. Receiver operating characteristic curve analysis determined the optimal cutoff to differentiate between patients with and without hydrocephalus. The admission characteristics of hydrocephalic patients with aneurysmal and nSAH were compared. RESULTS: A total of 70 patients met inclusion criteria, of which 21 (30%) presented with hydrocephalus. In univariate logistic regression, leukocytes, neutrophils, lymphocytes, neutrophil-to-lymphocyte ratio, lymphocyte-to-monocyte ratio, neutrophil-monocyte-to-lymphocyte ratio, and the systemic immune-inflammation (SII) index ([neutrophils × platelets/lymphocytes]/1000) were associated with hydrocephalus. After adjustments, the SII index independently predicted acute hydrocephalus with the highest odds among laboratory values (odds ratio 2.184, P = 0.006). Receiver operating characteristic curve analysis revealed the SII index differentiated between patients with and without hydrocephalus (area under the curve = 0.799, 95% CI: 0.688-0.909, P < 0.001) with an optimal cutoff of 1.385 103/µL. SII indices did not differ between aneurysmal and nSAH patients with hydrocephalus (3.5 vs. 3.6 103/µL, P = 0.795). CONCLUSIONS: A SII index ≥1.385 103/µL on admission predicts acute hydrocephalus in spontaneous nSAH. Hydrocephalic patients with aneurysmal and nSAH exhibit similar SII indices, and thus, an exaggerated inflammatory and thrombotic response follows spontaneous subarachnoid hemorrhage irrespective of hemorrhage etiology.
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Hidrocefalia , Hemorragia Subaracnóidea , Humanos , Hemorragia Subaracnóidea/complicações , Estudos Retrospectivos , Inflamação/complicações , Linfócitos , Hidrocefalia/complicações , PrognósticoRESUMO
BACKGROUND: Diffuse midline glioma with histone H3K27M mutation (H3K27M DMG) is a recently recognized World Health Organization grade IV glioma with a dismal prognosis. Despite maximal treatment, this high-grade glioma exhibits an estimated median survival of 9-12 months. However, little is known with regards to prognostic risk factors for overall survival (OS) for patients with this malignant tumor. The aim of the present study is to characterize risk factors influencing survival in H3K27M DMG. METHODS: This is a population-based retrospective study of survival in patients with H3K27M DMG. The Surveillance, Epidemiology, and End Results database was examined from the years 2018 to 2019 and data from 137 patients were collected. Basic demographics, tumor site, and treatments regimens were retrieved. Univariate and multivariable analyses were conducted to assess for factors associated with OS. Nomograms were built based on the results of the multivariable analyses. RESULTS: Median OS of the entire cohort was 13 months. Patients with infratentorial H3K27M DMG exhibited worse OS compared to their supratentorial counterparts. Any form of radiation treatment resulted in a significantly improved OS. Most combination treatments significantly improved OS with the exception of the surgery plus chemotherapy group. The combination of surgery and radiation had the greatest impact on OS. CONCLUSIONS: Overall, the infratentorial location of H3K27M DMG portends a worse prognosis compared to their supratentorial counterparts. The combination of surgery and radiation had the greatest impact on OS. These data highlight the survival benefit in utilizing a multimodal treatment approach for H3K27M DMG.
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Neoplasias Encefálicas , Glioma , Humanos , Prognóstico , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/terapia , Estudos Retrospectivos , Glioma/genética , Glioma/terapia , Histonas/genética , Mutação/genéticaRESUMO
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.
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BACKGROUND: Opsoclonus-myoclonus syndrome (OMS) is a rare neuroimmunologic disorder characterized by opsoclonus, myoclonic jerks mostly in the face and limbs, cerebellar ataxia, tremors, and encephalopathy. OMS is rare in adults and exceedingly rarer in pregnancy, as only a few cases in pregnancy have been reported. We present what we understand is the first case of postpartum OMS. METHODS AND RESULTS: We report and discuss a challenging case of OMS which started 6 weeks postpartum. Despite extensive infectious and malignancy evaluation, an underlying etiology was not readily apparent thus we treated her with high dose intravenous steroids and intravenous immunoglobulin (IVIG) for presumed idiopathic autoimmune OMS. She relapsed and additional workup identified new enhancing lesion on MRI brain, positive MOG-IgG, and CSF negative for oligoclonal bands. She was transitioned to maintenance IVIG and ultimately to rituximab with better results. At 2 year follow up her exam was improved and without objective evidence of abnormal movement or opsoclonus on maintenance Rituximab infusion 1,000 mg every 6 months. CONCLUSION: In OMS, an autoimmune response is usually thought to occur by molecular mimicry with neuronal cell surface antigens in association with infections. Since a preceding infection was absent in this case, we propose that the immune response here was initiated due to immunological changes in pregnancy and postpartum period possibly due to fetal tissue exposure (fetal microchimerism). The presence of the MOG antibody raises the possibility that OMS is another clinical manifestation of MOG-associated disease (MOG-AD), which in our case is supported by characteristic CSF and radiographic findings of MOG-AD.
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Síndrome de Opsoclonia-Mioclonia , Adulto , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Neurônios , Síndrome de Opsoclonia-Mioclonia/tratamento farmacológico , Período Pós-Parto , Gravidez , RituximabRESUMO
Introduction Access to healthcare and the utilization of health services at both the state and national levels are frequent areas of study, specifically in major ethnic populations such as White, Black, and Hispanic/Latino. However, there are few studies assessing healthcare access and utilization in the Nepalese communities in the United States (U.S.), despite the rapidly growing population of Nepalese immigrants. Methods To explore this issue, we conducted a questionnaire-based survey of Nepalese adults in Connecticut (CT). Results When compared to the reporting of the general CT population, a greater percentage of this survey's respondents report having trouble accessing necessary care (21.1% vs 11.0% in CT). Despite this, more Nepalese adults report satisfaction with the provider in terms of time spent during the visit (81.8% vs 76.0% in CT) and a consideration of values and beliefs during treatment (86.2% vs 70.0% in CT). In comparison with previous national reporting of the general U.S. population, Nepalese adults in the survey tend to have fewer total health care visits annually (87.1% reporting between zero and three visits vs. 64.7% in the U.S.). They also reported fewer dental visits (60.6% vs 71.0% in the U.S.). Conclusion While this novel study is one of the few examining health in the Nepalese population in the U.S., it serves as a foundation for future research in this area. Additionally, the results of the study highlight important disparities that local Nepalese organizations can use to design initiatives to improve this population's health.
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Introduction A massive earthquake on April 25, 2015, resulted in physical and emotional devastation in Nepal. This study aims to determine the prevalence of psychological distress among adolescents in Kathmandu and Sindhupalchowk districts within Nepal one year after the earthquake. Methods The Brief Symptoms Inventory tool was used to measure the level of psychological distress. The participants were students of four randomly selected schools from both districts. Surveys were conducted involving 200 students aged 13 to 17 years. Participants had diverse socioeconomic and cultural backgrounds. Results The prevalence of clinical threshold varied from 10% to 50% depending on each of the nine symptoms scales. Between the two districts, there was a statically significant difference in the prevalence of major psychological distresses. Sindhupalchowk had a higher percentage of students meeting the clinical threshold in each of the nine symptom scales than Kathmandu. Female students tended to have higher symptoms levels than male students. Conclusion The prevalence of psychological distress among adolescents living in areas of large impact is greater compared to the prevalence of psychological distress in adolescents living in less impacted areas. Given the current literature with respect to adolescent psychology in Nepal, more studies must be done to assess the level of distress in other regions of the country.