Male Predisposition in Cerebellar Mutism Syndrome: a Cohort Study.

The aim of this study was to explore the association between sex and cerebellar mutism syndrome and to examine other potential risk factors. This ambispective cohort study examined 218 pediatric patients (132 boys) with a posterior fossa tumor who underwent tumor resection from July 2013 to March 2021. The patients' demographics and tumor characteristics were examined and statistically analyzed to explore the associations among the variables. Multivariable and subgroup analyses were conducted to validate the independent risk factors for cerebellar mutism syndrome (CMS). The male and female patients did not differ significantly in terms of age, tumor size, tumor location, tumor consistency, VP shunt placement before resection, extent of resection, or surgeon, as well as with respect to the presence of hydrocephalus or paraventricular edema. The overall incidence of CMS was 32.6%. The incidence of CMS was significantly higher in male patients than that in female patients (41.7% vs. 18.6%; P = 0.001). In the multivariable analysis, male sex (adjusted odds ratio [OR], 3.27; P = 0.001), solid tumor consistency (adjusted OR, 5.61; P = 0.001), midline location (adjusted OR, 3.78; P = 0.004), and hydrocephalus (adjusted OR, 2.56; P = 0.047) were independent risk factors for the CMS. Chi-square analysis revealed that solid tumor consistency and midline location were associated with medulloblastoma (P < 0.001). Male patients had a higher risk of developing CMS after a posterior fossa tumor resection. Midline location, solid tumor consistency, and hydrocephalus were independent risk factors for CMS.

Older age is a protective factor for academic achievements irrespective of treatment modalities for posterior fossa brain tumours in children.

The treatment of children with posterior fossa brain tumours (PFBT) impacts their long term functional and imaging outcomes. This study aimed to evaluate academic achievement correlated with long-term sequelae after different PFBT treatment modalities. The study cohort consisted of 110 survivors (median age at diagnosis 10.1 years and median time of follow up 13.2 years) who completed hearing questionnaires, neurological assessment and MRI of the brain ≥5 years after the end of treatment. There were three treatment groups. A cisplatin group which underwent cisplatin chemotherapy, radiotherapy and surgery (medulloblastoma N = 40), a radiotherapy group which underwent radiotherapy and surgery (astrocytoma/ependymoma N = 30), and a surgery group (astrocytoma N = 40). Academic achievement was correlated to the age at diagnosis, ototoxicity, Karnofsky score (KS), and MRI findings (Fazekas Score (FS)- treatment related parenchymal changes). For a modelled age at diagnosis of five years, the cisplatin group had lower academic achievements compared to the radiotherapy (p = 0.028) and surgery (p = 0.014) groups. Academic achievements evaluated at a modelled age of 10 years at diagnosis did not significantly differ among the treatment groups. The cisplatin group exhibited a higher occurrence of ototoxicity than the radiotherapy (p<0.019) and surgery groups (p<0.001); however, there was no correlation between ototoxicity and academic achievements (p = 0.722) in older age at diagnosis. The radiotherapy group exhibited lower KS than the surgery group (p<0.001). KS significantly influenced academic achievements in all groups (p<0.000). The cisplatin group exhibited higher FS than the surgery group (p<0.001) while FS did not correlate with academic achievement (p = 0.399). Older age is a protective factor for academic achievements irrespective of a treatment modality.

Genetic predisposition to longer telomere length and risk of childhood, adolescent and adult-onset ependymoma.

Ependymoma is the third most common brain tumor in children, with well-described molecular characterization but poorly understood underlying germline risk factors. To investigate whether genetic predisposition to longer telomere length influences ependymoma risk, we utilized case-control data from three studies: a population-based pediatric and adolescent ependymoma case-control sample from California (153 cases, 696 controls), a hospital-based pediatric posterior fossa type A (EPN-PF-A) ependymoma case-control study from Toronto's Hospital for Sick Children and the Children's Hospital of Philadelphia (83 cases, 332 controls), and a multicenter adult-onset ependymoma case-control dataset nested within the Glioma International Case-Control Consortium (GICC) (103 cases, 3287 controls). In the California case-control sample, a polygenic score for longer telomere length was significantly associated with increased risk of ependymoma diagnosed at ages 12-19 (P = 4.0 × 10-3), but not with ependymoma in children under 12 years of age (P = 0.94). Mendelian randomization supported this observation, identifying a significant association between genetic predisposition to longer telomere length and increased risk of adolescent-onset ependymoma (ORPRS = 1.67; 95% CI 1.18-2.37; P = 3.97 × 10-3) and adult-onset ependymoma (PMR-Egger = 0.042), but not with risk of ependymoma diagnosed before age 12 (OR = 1.12; 95% CI 0.94-1.34; P = 0.21), nor with EPN-PF-A (PMR-Egger = 0.59). These findings complement emerging literature suggesting that augmented telomere maintenance is important in ependymoma pathogenesis and progression, and that longer telomere length is a risk factor for diverse nervous system malignancies.

Improved risk-stratification for posterior fossa ependymoma of childhood considering clinical, histological and genetic features - a retrospective analysis of the HIT ependymoma trial cohort.

INTRODUCTION: Risk stratification of children with ependymomas of the posterior fossa in current therapeutic protocols is mainly based on clinical criteria. We aimed to identify independent outcome predictors for this disease entity by a systematic integrated analysis of clinical, histological and genetic information in a defined cohort of patients treated according to the German HIT protocols. METHODS: Tumor samples of 134 patients aged 0.2-15.9 years treated between 1999 and 2010 according to HIT protocols were analyzed for histological features including mitotic activity, necrosis and vascular proliferation and genomic alterations by SNP and molecular inversion probe analysis. Survival analysis was performed by Kaplan-Meier method with log rank test and multivariate Cox regression analysis. RESULTS: Residual tumor after surgery, chromosome 1q gain and structural genomic alterations were identified as predictors of significantly shorter event-free (EFS) and overall survival (OS). Furthermore, specific histological features including vascular proliferation, necrosis and high mitotic activity were predictive for shorter OS. Multivariate Cox regression revealed residual tumor, chromosome 1q gain and mitotic activity as independent predictors of both EFS and OS. Using these independent predictors of outcome, we were able to build a 3-tiered risk stratification model that separates patients with standard, intermediate and high risk, and which outperforms current stratification procedures. CONCLUSION: The integration of defined clinical, histological and genetic parameters led to an improved risk-stratification model for posterior fossa ependymoma of childhood. After validation in independent cohorts this model may provide the basis for risk-adapted treatment of children with ependymomas of the posterior fossa.

Outcome after surgery in supratentorial and infratentorial solitary brain metastasis.

BACKGROUND: The aim of this retrospective study was to investigate and compare the outcome after surgery in patients with a supratentorial solitary metastasis (SSM) and an infratentorial solitary metastasis (ISM). A worse prognosis has been reported in ISM. METHODS: Fifty-two patients with a newly diagnosed solitary brain metastasis on MRI were included to identify risk factors affecting the outcome. Key variables included tumor size, staging of the primary tumor, time span of presurgical work-up, and surgical technique. Outcome variables included postoperative complications, tumor recurrence, and mortality. Kaplan-Meier survival analysis was applied. RESULTS: Thirty patients with a SSM and 22 patients with an ISM underwent gross total resection. The tumor size did not have a statistical significant effect on survival. Presurgical work-up time was similar in SSM and ISM. Postoperative complications were more frequently encountered in ISM. Recurrence rate was comparable in SSM and ISM. Carcinomatous meningitis (CM) was more frequently seen in ISM, and CM was seen more often with the piecemeal resection technique. There was no statistical difference in overall survival between SSM and ISM. CONCLUSIONS: This study identified factors that play a role in the outcome after surgery in patients with ISM and SSM on MRI. Postoperative complications seemed to be higher in ISM and CM was more often seen in ISM, but the worse prognosis in patients with ISM compared with SSM could not be confirmed.

Visual Complications of Pediatric Posterior Fossa Tumors: Analysis of Outcomes.

OBJECTIVE: Visual abnormalities are common among children with posterior fossa tumors, resulting from disruption of ocular afferent and efferent systems due to the tumor or surgery. This study describes the visual complications and outcomes associated with these tumors. METHODS: Clinical and radiographic data of patients who underwent index surgery for resection of posterior fossa tumor from 2007 to 2016 were reviewed. Descriptive statistics, univariate, and multivariate regression were performed to assess factors contributing to visual acuity and postoperative strabismus. RESULTS: There were 182 patients who underwent posterior fossa craniotomy for neoplasm were included. Ophthalmologic symptoms were the fourth most common presenting complaint; initial ophthalmologic examination was abnormal in 40% of patients. Evaluation of visual acuity demonstrated a good outcome in 88% of patients following treatment. The most common postoperative oculomotor finding was esotropia (29%) which resolved spontaneously in more than half of patients. A good outcome was obtained in all patients who underwent surgery for esotropia. Hypertropia was noted in 14% of the cohort and less than half resolved spontaneously; less than half undergoing strabismus surgery for hypertropia had a good outcome. Multivariate analysis confirmed the association between cerebellar mutism and postoperative esotropia and hypertropia. Clinically significant pathological nystagmus was seen in 8% of the cohort. CONCLUSIONS: Our results indicate a good visual outcome in the majority of pediatric patients undergoing resection of posterior fossa tumors. Ophthalmologic complications should be appropriately evaluated and addressed to allow for the best possible vision to survivors of posterior fossa tumors.

[Brain tumors in children: about 136 cases]. / Tumeurs cérébrales de l'enfant: à propos de 136 cas.

Brain tumors are the most diagnosed solid tumors in children under the age of 15 years worldwide. However, the epidemiological and anatomopathological profile of these tumors has been poorly described in African and, particularly, in Moroccan literature. This study highlights the epidemiological and anatomopathological peculiarities of primary brain tumors in children living in the region of Marrakech (south Morocco). We conducted a retrospective study in the Division of Anatomic Pathology at the Mohammed VI University Hospital, Marrakech from 2004 to 2016. One hundred and thirty-six patients were diagnosed with primary brain tumor, a mean of 11.33 cases per year. The average age of patients was 8.28 years. Sex-ratio (M/F) was 1.6 with a slight male predominance. Infratentorial tumors were found in 61,53% of cases while supratentorial tumors were found in 38.47% of cases. Infratentorial tumors mainly occurred in the cerebellar hemisphere (61.4%). Eighteen histological types were diagnosed. Astrocytoma and medulloblastoma accounted for 46,32% (29.41% and 16.91%, respectively). In our context, the majority of brain tumors in children was predominant in both age groups: 5-9 years and 10-15 years. The epidemiological data of these tumors from south Morocco are mostly consistent with those already published in North Country's literature and in other non-african countries' literature.

Suboccipital Craniotomy Versus Craniectomy: A Survey of Practice Patterns.

OBJECTIVE: Open surgical access to the posterior fossa traditionally has been achieved by permanent bone removal and remains the mainstay of posterior fossa surgery, although craniotomy is an alternative. Considerable variation exists at both the national and international levels within a variety of neurologic and neurosurgical disciplines. In this study, we surveyed current practice patterns regarding preference of suboccipital craniotomy or craniectomy. METHODS: The membership directory of the American Academy of Neurological Surgeons was reviewed. SurveyMonkey was used to distribute the survey to members of the American Academy of Neurological Surgeons via a modified Dillman method for e-mail correspondence. Comparisons of frequency distributions, means, and medians, as well as multiple logistic regression were used to determine surgical preferences for craniotomy versus craniectomy. RESULTS: We received 1102 responses (19.6%). Overall, 542 (49.7%) respondents prefer craniotomy and 548 (50.3%) prefer craniectomy. Respondents who prefer craniotomy had completed a residency more recently than respondents who preferred craniectomy (15.9 vs. 21.1 years, P < 0.0001) and were more likely to practice outside of North America (P < 0.01). Some 81.4% of pediatric neurosurgeons prefer craniotomy compared with 43.6% of adult neurosurgeons (P < 0.0001). Craniotomy was most highly preferred for tumor resection and vascular malformation. Within the United States, there was significant variation in preference for craniotomy based on geographic region, with New England most commonly preferring craniotomy and the Mid-Atlantic region most commonly preferring craniectomy. CONCLUSIONS: Our results show that preference for suboccipital craniotomy or craniectomy varies according to geographic location of practice, time since completing residency, and age of patient population.

A Review of Visual and Oculomotor Outcomes in Children With Posterior Fossa Tumors.

Tumors of the posterior fossa represent the most common solid malignancy of childhood and can affect the visual system in several ways. This article outlines the relevant visual anatomy affected by these tumors and reviews the visual and oculomotor outcomes associated with the following 3 most common tumor types-medulloblastoma, juvenile pilocytic astrocytoma, and ependymoma. The available data suggest that the rate of permanent vision loss is low (5.9%-8.3%), with patients having juvenile pilocytic astrocytoma demonstrating the best outcomes. The rate of long-term strabismus (25%-29.1%) and nystagmus (12.5%-18%) is higher and associated with significant morbidity.

Cerebellar mutism syndrome in children with brain tumours of the posterior fossa.

BACKGROUND: Central nervous system tumours constitute 25% of all childhood cancers; more than half are located in the posterior fossa and surgery is usually part of therapy. One of the most disabling late effects of posterior fossa tumour surgery is the cerebellar mutism syndrome (CMS) which has been reported in up to 39% of the patients but the exact incidence is uncertain since milder cases may be unrecognized. Recovery is usually incomplete. Reported risk factors are tumour type, midline location and brainstem involvement, but the exact aetiology, surgical and other risk factors, the clinical course and strategies for prevention and treatment are yet to be determined. METHODS: This observational, prospective, multicentre study will include 500 children with posterior fossa tumours. It opened late 2014 with participation from 20 Nordic and Baltic centres. From 2016, five British centres and four Dutch centres will join with a total annual accrual of 130 patients. Three other major European centres are invited to join from 2016/17. Follow-up will run for 12 months after inclusion of the last patient. All patients are treated according to local practice. Clinical data are collected through standardized online registration at pre-determined time points pre- and postoperatively. Neurological status and speech functions are examined pre-operatively and postoperatively at 1-4 weeks, 2 and 12 months. Pre- and postoperative speech samples are recorded and analysed. Imaging will be reviewed centrally. Pathology is classified according to the 2007 WHO system. Germline DNA will be collected from all patients for associations between CMS characteristics and host genome variants including pathway profiles. DISCUSSION: Through prospective and detailed collection of information on 1) differences in incidence and clinical course of CMS for different patient and tumour characteristics, 2) standardized surgical data and their association with CMS, 3) diversities and results of other therapeutic interventions, and 4) the role of host genome variants, we aim to achieve a better understanding of risk factors for and the clinical course of CMS - with the ultimate goal of defining strategies for prevention and treatment of this severely disabling condition. TRIAL REGISTRATION: Clinicaltrials.gov : NCT02300766 , date of registration: November 21, 2014.

Posterior cranial fossa tumours in children at National Cancer Institute, Sudan: a single institution experience.

BACKGROUND: Posterior cranial fossa tumours (PCF) comprise 54-70% of childhood brain tumours. The clinical profile and outcomes of these tumours differ in area of the world. The aim of this study is to describe clinical characteristics, treatment and outcome of posterior cranial fossa tumours in Sudan. MATERIAL AND METHODS: A retrospective study of children diagnosed with posterior cranial fossa tumours and treated between January 1998 and December 2015 at National Cancer Institute, Sudan. RESULTS: A total of 31 paediatric patients with a posterior cranial fossa brain tumour were identified over the study period. The mean age was 7.9 years (standard deviation (SD) = 3.37). Females were slightly more affected (51%) than males (49%), and the majority of patients were from rural areas (71%). Brainstem tumours were the most frequent tumour type (48%) followed by medulloblastoma (36%). The mean pre-diagnostic symptomatic interval was 3.6 months (SD = 3.95). Overall survival rate of our total study population at 1, 2 and 5 years was 23, 19 and 13%, respectively. CONCLUSION: Overall, this study draws attention to the situation of paediatric brain tumours in Sudan. Late presentation, misdiagnosis and limited diagnostic and treatment resources are challenges that may contribute to poor outcome in these patients.

Presentation and management of lateral sinus thrombosis following posterior fossa surgery.

OBJECTIVE There are no guidelines for the management of postoperative lateral sinus thrombosis following posterior fossa surgery. Introducing treatment-dose anticoagulant therapy during the immediate postoperative period increases the risk of intracranial bleeding. This study assessed the incidence of and risk factors associated with postoperative lateral sinus thrombosis and the complications related to thrombosis and/or anticoagulation. METHODS This study was a retrospective monocentric analysis of adult patients who underwent surgical removal of a posterior fossa space-occupying lesion with available postoperative imaging. Postoperative lateral sinus thrombosis was defined as a T2* hypointensity within the venous sinus and/or a filling defect on postcontrast MRI or CT scan. RESULTS Among 180 patients, 12 (6.7%; 95% CI 3.0-10.4) were found to have lateral sinus thrombosis on postoperative imaging, none of whom were symptomatic. Unadjusted risk factors for postoperative lateral sinus thrombosis were a history of deep venous thrombosis (p = 0.016), oral contraceptive pill (p = 0.004), midline surgical approach (p = 0.035), and surgical exposure of the sinus (p < 0.001). Seven of the patients (58.3%) with a postoperative lateral sinus thrombosis received immediate treatment-dose anticoagulant therapy. Lateral sinus recanalization occurred radiologically at a mean time of 272 ± 23 days in 85.7% of patients (6 of 7) undergoing treatment-dose anticoagulant therapy and in 20% of patients (1 of 5) not receiving treatment-dose anticoagulant therapy. Postoperative complications occurred in 56.2% of patients (9 of 16) who received treatment-dose curative anticoagulant therapy and in 27% of patients (45 of 164) who did not. CONCLUSIONS Incidental radiological lateral sinus thrombosis following posterior fossa surgery has an incidence of 6.7%. To further define the benefit-to-risk ratio of a treatment-dose anticoagulant therapy, a prospective trial should be considered.

Intraoperative neurophysiological monitoring during resection of infratentorial lesions: the surgeon's view.

OBJECTIVE Methods of choice for neurophysiological intraoperative monitoring (IOM) within the infratentorial compartment mostly include early brainstem auditory evoked potentials, free-running electromyography, and direct cranial nerve (CN) stimulation. Long-tract monitoring with somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) is rarely used. This study investigated the incidence of IOM alterations during posterior fossa surgery stratified for lesion location. METHODS Standardized CN and SEP/MEP IOM was performed in 305 patients being treated for various posterior fossa pathologies. The IOM data were correlated with lesion locations and histopathological types as well as other possible confounding factors. RESULTS Alterations in IOM were observed in 158 of 305 cases (51.8%) (CN IOM alterations in 130 of 305 [42.6%], SEP/MEP IOM alterations in 43 of 305 [14.0%]). In 15 cases (4.9%), simultaneous changes in long tracts and CNs were observed. The IOM alterations were followed by neurological sequelae in 98 of 305 cases (32.1%); 62% of IOM alterations resulted in neurological deficits. Sensitivity and specificity for detection of CN deficits were 98% and 77%, respectively, and 95% and 85%, respectively, for long-tract deficits. Regarding location, brainstem and petroclival lesions were closely associated with concurrent CN IOM and SEP/MEP alterations. CONCLUSIONS The incidence of IOM alterations during surgery in the posterior fossa varied widely between different lesion locations and histopathological types. This analysis provides crucial information on the necessity of IOM in different surgical settings. Because MEP/SEP and CN IOM alterations were commonly observed during posterior fossa surgery, the authors recommend the simultaneous use of both modalities based on lesion location.

Central nervous system tumours in children in Ibadan, Nigeria: a histopathologic study.

INTRODUCTION: Contrary to some earlier teachings that central nervous system (CNS) tumours are uncommon in black children, these neoplasms are the fourth most common paediatric tumours in Ibadan. Our centre is the major referral centre for CNS tumours in Nigeria. The last major study of paediatric CNS neoplasms from Ibadan was in 1985. An update of the data on paediatric CNS neoplasms at our centre is presented. METHODS: A retrospective review of all histologically diagnosed CNS tumours in children (0-14 years) from January 2001 to December 2010 from the database of the Department of Pathology, University College Hospital, Ibadan, Nigeria was done. The cases were classified using the 2007 WHO Classification of Tumours of the Central Nervous System and were also based on their supratentorial and infratentorial locations. RESULTS: Seventy-seven tumours, 44 in males, were included in the study. Astrocytic tumour comprised 20 cases, embryonal tumours 15, ependymal tumours 15, germ cell tumours 6, sellar tumours (all craniopharyngiomas) 9 and other histological types- 12 cases. Thirty-seven were WHO Grade 1, eleven Grade 2, ten Grade 3 and nineteen Grade 4 neoplasms. Thirty-six cases were supratentorial and thirty-eight were infratentorial in location. The most common tumours in this series were pilocytic astrocytomas, medulloblastomas, craniopharyngiomas and ependymomas in that order. CONCLUSION: Childhood CNS tumours are being increasingly diagnosed in our centre. This is largely explained by the recent expansion of the available neurosurgical services.

Perioperative Predictors of Extubation Failure and the Effect on Clinical Outcome After Infratentorial Craniotomy.

BACKGROUND The purpose of the study was to analyze the risk factors for failed extubation in subjects submitted to infratentorial craniotomy. MATERIAL AND METHODS Patients aged over 18 years who received infratentorial craniotomy for brain tumor resection were consecutively included in this study. Perioperative variables were collected and analyzed. Univariate analyses and multiple logistic regression were used to derive factors related to failed extubation. Patients had follow-up care until either out of hospital or death. RESULTS Throughout the course of the study, 2118 patients were eligible and 94 (4.4%) suffered from extubation failure at some point during their hospital stay. Five factors were recognized as independent risk factors for postoperative failed extubation: craniotomy history, preoperative lower cranial nerve dysfunction, tumor size, tumor position, and maximum change in blood pressure (BP) during the operation. Failed extubation was related to a higher incidence rate of pneumonia, mortality, unfavorable Glasgow Outcome Scale score, longer stay in the neuro-intensive care unit (ICU) and hospitalization, and higher hospitalization costs compared with successful extubation. CONCLUSIONS History of craniotomy, preoperative lower cranial nerve dysfunction, tumor size, tumor position, and maximum change in BP during the operation were independent risk factors related to postoperative failed extubation in patients submitted to infratentorial craniotomy. Extubation failure raises the incidences of postoperative pneumonia, mortality, and higher hospitalization costs, and prolongs neuro-ICU and postoperative length of stay.

Treatment and survival of supratentorial and posterior fossa ependymomas in adults.

Ependymoma is a rare primary brain or spinal cord tumor that arises from the ependyma, a tissue of the central nervous system. This study analyzed a large cohort of adult supratentorial and posterior fossa ependymoma tumors in order to elucidate factors associated with overall survival. We utilized the USA National Cancer Database to study adult World Health Organization grade II/III supratentorial and posterior fossa ependymoma patients treated between 1998 and 2011. Overall survival was estimated by the Kaplan-Meier method and factors associated with survival were determined using a multivariate Cox proportional hazards model. Among 1318 patients, 1055 (80.0%) had grade II and 263 (20.0%) anaplastic tumors located in the posterior fossa (64.3%) and supratentorial region (35.7%). Overall average age was 44.3years, 48.0% of patients were female, 86.5% were Caucasian, and 36.8% underwent near/gross total surgical resection. Radiotherapy was given to 662 patients (50.8%) and 75 (5.9%) received chemotherapy. Older age at diagnosis (hazard ratio [HR] 1.51, p<0.0001), high tumor grade (HR 1.82, p=0.005), and large tumor size (HR 1.66, p=0.008) were associated with poor survival. Females compared to males (HR 0.67, p=0.03) and patients with posterior fossa tumors versus supratentorial (HR 0.64, p=0.04) had a survival advantage. Our study showed that older patients, with supratentorial tumors, and high histological grade had an increased risk of mortality. A survival benefit was captured in females and patients with posterior fossa tumors. Adjuvant radiotherapy and chemotherapy did not confer a survival benefit among all patients, even after stratification by tumor grade or anatomical location.

[Epidemiology and etiology of posterior fossa tumors with particular emphasis on astrocytomas from the Malopolska and Podkarpacki regions]. / Epidemiologia i etiologia guzów tylnej jamy czaszki ze szczególnym uwzglednieniem gwiazdziaków z terenu województw malopolskiego i podkarpackiego.

Brain tumors in children are rare compared with other diseases childhood. Virtually every pediatrician working at the local hospital has been in contact or brain tumor diagnosis was that more half of them recognized the posterior fossa tumor. Analysis of surveys showed difficulties in the interpretation of basic neurological symptoms. Lessons learned from these studies point to the fact that the success of the surgery affects mainly the histological type of tumor and its location. It is extremely important to analyze complaints and symptoms young patients, we should listen to the parents of children with, because usually just parents are the first to notice that their offspring something was wrong.

Astroblastomas: a Surveillance, Epidemiology, and End Results (SEER)-based patterns of care analysis.

OBJECTIVE: This study sought to report patient characteristics, risk factors, and trends in management for astroblastoma patients. METHODS: A retrospective analysis was conducted utilizing the Surveillance, Epidemiology and End Results Program of the National Cancer Institute. RESULTS: Two hundred and thirty-nine patients were identified, with 206 patients receiving treatment. The median age at diagnosis was 35 years (range 0 to 84 years). Tumor location was available for 177 patients, and the majority were supratentorial (n = 144, 81.3%). The median overall survival and cause-specific survival for the cohort receiving treatment was 55 and 65 months, respectively. On univariate analysis, patients receiving surgery alone compared to only radiotherapy displayed improved overall survival (OS) and cause-specific survival (CSS) with a 5-year OS of 62.2% vs. 27.3%, P < .001, and CSS of 67.3% vs. 31.9%, P = .003. Supratentorial tumor location was associated with worse survival, with an estimated 5-year OS of 44.9% for supratentorial tumors compared to 75% for infratentorial tumors (hazard ratio 3.41 [95% CI, 1.76 to 6.62]; P < .001) and CSS of 47.5% (supratentorial) to 82% (infratentorial) (hazard ratio 3.95 [95% CI, 1.81 to 8.62]; P = .001). Age >60 years at diagnosis and treatment before 1990 were correlated with decreased survival on both the univariate and the multivariate analyses. CONCLUSIONS: To our knowledge, this is the largest report of astroblastoma patients described in the literature. Supratentorial tumor location, older age, and treatment prior to 1990 were poor prognostic factors.

Natural history and role of radiation in patients with supratentorial and infratentorial WHO grade II ependymomas: results from a population-based study.

Patients with World Health Organization (WHO) grade II supratentorial ependymomas are commonly observed after gross total resection (GTR), although supporting data are limited. We sought to characterize the natural history of such tumors. We used the Surveillance, Epidemiology, and End Results program to identify 112 patients ages 0-77 diagnosed with WHO grade II ependymomas between 1988 and 2007, of whom 63 (56 %) and 49 (44 %) had supratentorial and infratentorial primaries, respectively. Inclusion criteria were strict to ensure patient homogeneity. Of 33 patients with supratentorial tumors after GTR, 18 (55 %) received adjuvant radiation therapy and 15 (45 %) did not. Ependymoma-specific mortality (ESM) was the primary endpoint. With a median follow up of 4.5 years, only 1 of 33 patients with supratentorial ependymoma died of their disease after GTR; the 5-year estimate of ESM in this population was 3.3 % (95 % CI 0.2-14.8 %). Among patients with infratentorial ependymomas after GTR, the 5-year estimate of ESM was 8.7 % (95 % CI 1.4-24.6 %). In patients with subtotally resected tumors, 5-year estimates of ESM in patients with supratentorial and infratentorial primaries were 20.1 % (95 % CI 8.0-36.2 %) and 12.3 % (95 % CI 2.9-28.8 %), respectively. Among the whole cohort, on both univariable and multivariable regression, extent of resection was predictive of ESM, while tumor location and use of radiation were not. After GTR, patients with WHO grade II supratentorial ependymomas have a very favorable natural history with low associated cancer-specific mortality. Observation, with radiation reserved as a salvage option, may be a reasonable postoperative strategy in this population.