Intracranial Ependymomas In Children Research Articles

Therapeutic effects of intracranial ependymoma in children

Objective To investigate the treatment and prognosis of intracranial ependymoma in children. Methods The clinical data of 48 children with intracranial ependymoma who were first diagnosed and treated at Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from September 2012 to September 2016 were retrospectively analyzed. The tumors were solitary in 48 patients, including under-tentorial tumors in 34 cases and supratentorial tumors in 14 cases. All patients underwent surgical resection, adjuvant radiotherapy and/or chemotherapy, and regular outpatient and telephone follow-up. Kaplan-Meier method was used to analyze the survival rate of patients, and Cox regression analysis was used to explore the influencing factors of survival. Results Of the 48 patients, 31 achieved gross total resection at the first operation and 17 received subtotal resection. Postoperative pathological results showed that classical ependymomas (WHO grade Ⅱ) was in 4 cases, anaplastic ependymomas (WHO grade Ⅲ) in 26 cases, and partial anaplastic ependymomas (between WHO grade Ⅱ and Ⅲ) in 18 cases. Postoperative local recurrence occurred in 21 cases, of which 20 cases underwent reoperation. 7 cases developed local recurrence with distant dissemination, of which 4 cases underwent reoperation. Of 48 patients, radiotherapy were performed in 46 cases/times and chemotherapy in 42 patients/times after operation. 2 cases died of hydrocephalus and 14 cases died of recurrence of tumors. The follow-up time of 48 patients ranged from 5 to 68 months, with a median of 34 months. The 3-year progression-free survival rate and overall survival rate were 29.3% and 70.8% respectively. The 3-year overall survival rate of 28 patients with recurrence was 62.1%. Univariate analysis showed that for children with ependymoma, the degree of first surgical excision was a factor affecting progression free survival(P=0.027). The age of diagnosis, the location of the tumors and the degree of last surgical excision might be the influencing factors of overall survival in patients with ependymoma (all P<0.05). Multivariate analysis showed that the degree of resection (RR=3.568, 95%CI: 1.638-7.775, P=0.001) was an independent factor affecting the survival time of patients. Conclusions Surgical resection is still the most important treatment for children with ependymoma. The degree of tumor resection is related to survival outcomes. Key words: Ependymoma; Child; Neurosurgical procedures; Chemotherapy, adjuvant; Radiotherapy, adjuvant; Prognosis

Outcome of postoperative radiation therapy for pediatric intracranial ependymoma: a single-institution review.

To report outcome of postoperative radiotherapy (RT) in both new and recurrent grade II and III intracranial ependymomas in children treated at Ramathibodi Hospital. Between 2006 and 2017, 24 pediatric intracranial ependymomas treated with postoperative RT were retrospectively reviewed. The median age at diagnosis was 44.5months (range, 4-165months). There were 14 (58%) males. Fourteen (58%) patients had infratentorial tumor. The median maximal diameter of tumor at diagnosis was 4.45cm (range, 2.2-10cm). Fourteen (58%) patients had anaplastic tumor. Gross total resections were performed in 14 (58%) patients. The median prescribed dose was 54Gy (range, 45-60Gy). The median total treatment time was 43days (range, 37-78days). The median clinical follow-up time was 44.5months (range, 1-146months). There were nine recurrences, five of which occurred at the primary tumor site. The estimated 5-year progression-free survival rate was 56%. The estimated 5-year overall survival rate was 75%. Extent of resection was the only factor associated with improved progression-free survival and overall survival after univariate testing. Six from nine patients with recurrent diseases underwent further surgery or further RT. These six patients had better median overall survival than the three who did not. Acute complication was mostly transient and tolerable. No late radiation effect was found. Postoperative radiation is an effective treatment. GTR is associated with better PFS and OS. Aggressive salvage local treatments for recurrent patients can result in good overall survival. Longer follow-up is needed in account for late relapse.

A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas

Ependymoma is the third most common primary brain tumor in children. Tumors are classified according to the WHO pathological grading system. Prior studies have shown high levels of variability in patient outcomes within and across pathological grades. The authors reviewed the results from the published literature on intracranial ependymomas in children to describe clinical outcomes as they relate to treatment modality, associated mortality, and associated progression-free survival (PFS). A search of English language peer-reviewed articles describing patients 18 years of age or younger with intracranial ependymomas yielded data on 182 patients. These patients had undergone treatment for ependymoma with 1 of 5 modalities: 1) gross-total resection (GTR), 2) GTR as well as external beam radiation therapy (EBRT), 3) subtotal resection (STR), 4) STR as well as EBRT, or 5) radiosurgery. Mortality and outcome data were analyzed for time to tumor progression in patients treated with 1 of these 5 treatment modalities. Of these 182 patients, 69% had supratentorial ependymomas and 31% presented with infratentorial lesions. Regardless of tumor location or pathological grade, STR was associated with the highest rates of mortality. In contrast, GTR was associated with the lowest rates of mortality, the best overall survival, and the longest PFS. Children with WHO Grade II ependymomas had lower mortality rates when treated more aggressively with GTR. However, patients with WHO Grade III tumors had slightly better survival outcomes after a less aggressive surgical debulking (STR+EBRT) when compared with GTR. Mortality, PFS, and overall survival vary in pediatric patients with intracranial ependymomas. Pathological classification, tumor location, and method of treatment play a role in outcomes. In this study, GTR was associated with the best overall and PFS rates. Patients with WHO Grade II tumors had better overall survival after GTR+EBRT and better PFS after GTR alone. Patients with WHO Grade III tumors had better overall survival after STR+EBRT. Patients with infratentorial tumors had improved overall survival compared with those with supratentorial tumors. Progression-free survival was best in those patients with infratentorial tumors following STR+EBRT. Consideration of all of these factors is important when counseling families on treatment options.

Abstract 3002: Identification of frequently altered protein networks in ependymomas

Abstract Ependymomas are glial tumors that originate from the walls of the cerebral ventricles or from the spinal canal. The five-year survival for intracranial ependymomas in children is approximately 65%. Ependymomas exhibit a wide range of DNA copy number alterations that vary greatly in size and location. We hypothesize that an underlying protein network exists that is consistently compromised by such DNA copy number alterations in ependymomas. In this study, we analyzed an array-comparative genomic hybridization dataset generated by Johnson and colleagues [Nature 466(7306):632-6, 2010] to determine whether a protein interaction network could be identified that might be consistently functionally compromised in pediatric ependymomas. This cohort of 203 primary ependymomas consisted of 106 posterior fossa, 56 supratentorial, and 41 spinal ependymomas. The genes within each altered region were used to perform network analyses with GeneGo and Ingenuity to uncover protein networks that were altered in a significant number of tumors. For calculation of statistical significance, 100,000 samplings of randomly selected regions - equal in gene number to the regions of gain or loss - were performed. Indeed, some protein networks were found to be over-represented in the structural alterations exhibited by this cohort (p&amp;lt;0.05). These results warrant future investigations to demonstrate the role of the identified networks in the development and progression of ependymomas. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 103rd Annual Meeting of the American Association for Cancer Research; 2012 Mar 31-Apr 4; Chicago, IL. Philadelphia (PA): AACR; Cancer Res 2012;72(8 Suppl):Abstract nr 3002. doi:1538-7445.AM2012-3002

Molecular Staging of Intracranial Ependymoma in Children and Adults

The biologic behavior of intracranial ependymoma is unpredictable on the basis of current staging approaches. We aimed at the identification of recurrent genetic aberrations in ependymoma and evaluated their prognostic significance to develop a molecular staging system that could complement current classification criteria. As a screening cohort, we studied a cohort of 122 patients with ependymoma before standardized therapy by using array-based comparative genomic hybridization. DNA copy-number aberrations identified as possible prognostic markers were validated in an independent cohort of 170 patients with ependymoma by fluorescence in situ hybridization analysis. Copy-number aberrations were correlated with clinical, histopathologic, and survival data. In the screening cohort, age at diagnosis, gain of 1q, and homozygous deletion of CDKN2A comprised the most powerful independent indicators of unfavorable prognosis. In contrast, gains of chromosomes 9, 15q, and 18 and loss of chromosome 6 were associated with excellent survival. On the basis of these findings, we developed a molecular staging system comprised of three genetic risk groups, which was then confirmed in the validation cohort. Likelihood ratio tests and multivariate Cox regression also demonstrated the clear improvement in predictive accuracy after the addition of these novel genetic markers. Genomic aberrations in ependymomas are powerful independent markers of disease progression and survival. By adding genetic markers to established clinical and histopathologic variables, outcome prediction can potentially be improved. Because the analyses can be conducted on routine paraffin-embedded material, it will now be possible to prospectively validate these markers in multicenter clinical trials on population-based cohorts.

Intracranial ependymoma in children: current status and future trends on diagnosis and management

One of the most exciting elements of the practice of pediatric neurosurgery has been the advances made in the realm of neuro-oncology over the past three decades. Moving from a time in which brain tumors were diagnosed primarily on clinical grounds, the understanding of cellular and molecular events were based on the appearance of paraffin-embedded specimens viewed under glass, the radiological tools necessary to define the extent of the disease were rudimentary, and the surgical technology available to make tumor removal safe and to offer the survivor a reasonable quality of life were rudimentary, we have now moved into a fast-paced arena in which each year brings new research with better understanding of tumor genomics, tumor suppressors and promotors, stem cells, vascular supply, etc. Advances in neuroimaging now allow accurate preoperative diagnosis and staging, image-guided surgery offers improved operative planning and execution, and better postoperative surveillance tools allow earlier detection and more effective treatments for recurrent disease. More sensitive tools now allow earlier detection of secondary events such as learning disabilities, quality of life measures, and hormonal deficits, which can be treated effectively through improved pharmacology. No area of neuro-oncology serves as a better model for these statements than does the treatment of childhood ependymoma. It is anticipated that this compendium will offer current knowledge on the status of ependymoma therapy for the neurosurgeon, that it will correct certain misperceptions related to ependymoma therapy, and that it will give direction for future advances in the treatment of this disease. I would like to thank in advance all of the contributors who spent long hours working on their manuscripts for this Supplement. That those asked to contribute are world leaders in the field of pediatric neuro-oncology becomes apparent as one peruses the table of contents of this journal. There are a number of truths which we can now apply to pediatric ependymoma. First is that in 90% of cases, the disease will be localized at presentation. At that stage, the focal disease process will usually respond to focal treatments [1]. Thus, the days of craniospinal irradiation or even whole brain irradiation for ependymomas should be well F. A. Boop (*) Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, TN, USA e-mail: faboop@aol.com

Relapsed intracranial ependymoma in children in the UK: Patterns of relapse, survival and therapeutic outcome

Relapsed ependymoma in children poses difficult dilemmas in management. Clinico-pathological and treatment data of 108 children with relapsed ependymoma in the United Kingdom (UK) treated between 1985 and 2002 were reviewed to identify prognostic factors affecting survival. The primary site was the most common site of relapse (84%). Overall 25% had metastatic relapse. Surgery at relapse was attempted in only 55%. Radiotherapy was delivered at relapse in 66% infants and 50% of older children were re-irradiated. Overall 5-year survival was 24% and 27% for children less than 3 years of age at initial diagnosis and older children, respectively. Multivariate analysis showed that, for infants, surgery ( p = 0.01) and radiotherapy ( p = 0.001) at relapse were independent predictors of survival. For older children regardless of the previous radiotherapy, repeat irradiation was associated with better outcome ( p = 0.05). Relapse was associated with poor outcome in both age groups. A survival advantage conferred by both radiotherapy and surgery at relapse is independently significant.

Intracranial Ependymomas in Children: Society of Pediatric Oncology Experience With Postoperative Hyperfractionated Local Radiotherapy

To prospectively investigate the role of local hyperfractionated radiotherapy (RT) after surgical resection in the treatment of intracranial ependymomas in children. Postoperative local hyperfractionated RT was proposed for every child (>5 years old at diagnosis) with localized intracranial ependymoma. The planned dose was 60 Gy after complete resection (CR) and 66 Gy after partial resection, delivered in two daily fractions of 1 Gy, according to the early postoperative imaging findings. Between November 1996 and December 2002, 24 children with infratentorial (n = 20) or supratentorial (n = 4) intracranial ependymoma were included. The median age was 8.6 years (range, 5-17). The World Health Organization grade was anaplastic in 10 of the 24 patients (not assessable in 1). After a retrospective central review, a CR was reported in 16 patients, partial resection in 4, and doubtful resection in 4. The radiation dose was 60 Gy in 18 cases (one partial resection), 66 Gy in 5 cases (one CR), and 54 Gy in 1 case (CR). The 5-year overall survival rate was 74.8%, and the progression-free survival rate was 54.2%. Of the 24 patients, 11 developed a relapse: 7 local only and 4 metastatic and local. The histological grade and extent of resection were not prognostic factors. More than 3 in 4 children had no sequelae of RT at a median follow-up of 7 years (95% confidence interval, 66.4-90.0 months). The results of our study have shown that hyperfractionated RT is safe but provides no outcome benefit compared with other strategies of RT such as standard fractionated regimens.

The role of stereotactic radiotherapy in the management of ependymomas

Intracranial ependymomas in children have high rates of recurrence. Salvage therapy typically includes repeat resection, possibly chemotherapy, and re-irradiation. Stereotactic radiosurgery has been used for re-irradiation. It offers the theoretical advantages of delivering a high dose of radiation to a small target, providing a maximum dose to the tumor while avoiding surrounding critical brain structures and previously irradiated tissue. Few reports in the literature describe this technique, with mixed, but not often successful, outcomes.

Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children

Introduction: Intracranial ependymomas are the third most common histologic subtype of childhood brain tumor. Although ependymomas may occur at any age, about 50% are diagnosed in children younger than 5 years of age. In adults, most ependymomas are cortical, but 70% to 80% of the tumors that arise in children involve posterior fossa structures [1]. Ependymomas are often laterally placed, involving the brainstem and cerebellar peduncles, with contiguous involvement of multiple cranial nerves. The intramixture of the tumor with the cranial nerves and portions of the brainstem makes surgical resections diffi cult, and there is often a quandary as to whether to attempt a gross total resection, given the potential morbidity. This decision is made even more diffi cult because many studies suggest that patients who undergo total resections are more likely to have long-term disease control [1–3]. The histologic grading of ependymomas has been variably related to outcome, with most but not all studies demonstrating poorer survival for anaplastic tumors [1–3]. Most reports conclude that less than 5% of ependymomas are disseminated at the time of diagnosis. Thus, stratifi cation based on the extent of disease at the time of diagnosis is usually not particularly helpful. Similarly, robust prospective biologic predictors of outcome have yet to be identifi ed. Recent reports have demonstrated excellent 3to 5-year survival rates in children with totally resected ependymomas of the posterior fossa who receive postoperative radiotherapy to the primary tumor site [2]. Conformal radiotherapy techniques result in excellent disease control, with the caveat that many posterior fossa ependymomas extend to the lower brainstem or upper cervical cord, and these regions may need to be included in the radiation portal [2–4]. Somewhat reassuringly, neurocognitive outcome has been reported to be relatively unimpaired by the radiotherapy delivered, although this assumption must be considered somewhat preliminary [5]. The benefi t of chemotherapy for children with ependymomas is far from proven. Early studies suggested no benefi t from the addition of adjuvant chemotherapy when given during and after radiotherapy. Recently, other studies have suggested that chemotherapy given prior to radiation therapy may be of benefi t, especially in patients with anaplastic tumors. One of the rationales for the use of chemotherapy is to couple it with radiotherapy to improve survival. Another is to use chemotherapy to try to delay the need for radiotherapy, especially in patients thought to be most vulnerable to the deleterious long-term effects of radiotherapy—primarily children with large cortical lesions or younger children with lesions anywhere in the neuroaxis. There is no consensus on the age at which radiotherapy, including conformal radiotherapy, is safe. Some studies have used conformal radiotherapy in children as young as 1 year of age, and others have suggested the use of chemotherapy in order to delay radiation therapy until the child is at least 3 years old [2].

Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children: the UKCCSG/SIOP prospective study

Over half of childhood intracranial ependymomas occur in children younger than 5 years. As an adjuvant treatment, radiotherapy can be effective, but has the potential to damage the child's developing nervous system at a crucial time-with a resultant reduction in IQ and cognitive impairment, endocrinopathy, and risk of second malignancy. We aimed to assess the role of a primary chemotherapy strategy in avoiding or delaying radiotherapy in children younger than 3 years with intracranial ependymoma. Between December, 1992, and April, 2003, we enrolled 89 children with ependymoma who were aged 3 years or younger at diagnosis, of whom nine had metastatic disease on pre-operative imaging. After maximal surgical resection, children received alternating blocks of myelosuppressive and non-myelosuppressive chemotherapy every 14 days for an intended duration of 1 year. Radiotherapy was withheld unless local imaging (ie, from the child's treatment centre) showed progressive disease. 50 of the 80 patients with non-metastatic disease progressed, 34 of whom were irradiated for progression. The 5-year cumulative incidence of freedom from radiotherapy for the 80 non-metastatic patients was 42% (95% CI 32-53). With a median follow-up of 6 years (range 1.5-11.3), overall survival for the non-metastatic patients at 3 years was 79.3% (95% CI 68.5-86.8) and at 5 years 63.4% (51.2-73.4). The corresponding values for event-free survival were 47.6% (36.2-58.1) and 41.8% (30.7-52.6). There was no significant difference in event-free or overall survival between complete and incomplete surgical resection, nor did survival differ according to histological grade, age at diagnosis, or site of disease. In 47 of 59 (80%) patients who progressed, relapse resulted from local control only. The median time to progression for the 59 patients who progressed was 1.6 years (range 0.1-10.2 years). The median age at irradiation of the whole group was 3.6 years (range 1.5-11.9). For the 80 non-metastatic patients, the 23 who achieved the highest relative dose intensity of chemotherapy had the highest post-chemotherapy 5-year overall survival of 76% (95% CI 46.6-91.2), compared with 52% (33.3-68.1) for the 32 patients who achieved the lowest relative dose intensity of chemotherapy. This protocol avoided or delayed radiotherapy in a substantial proportion of children younger than 3 years without compromising survival. These results suggest, therefore, that primary chemotherapy strategies have an important role in the treatment of very young children with intracranial ependymoma.

Intracranial ependymomas in childhood: recurrence, reoperation, and outcome

Intracranial ependymomas (IE) in children are aggressive tumors, and total resection (TR) is considered to be the most powerful predictor of outcome. The data regarding recurrent IE (RIE), in particular the role of reoperation, are scarce in the literature. We studied 70 cases of IE in children operated on for IE since the advent of computed imaging. TR was achieved at initial surgery in 38 out of 70 cases. After a mean follow-up of 76.1 months, 33 out of 70 tumors had progressed. Eighteen were reoperated, achieving TR in 12 out of 18 cases. The morbidity associated with reoperation was minimal, especially compared with the first operation. After a mean follow-up of 74.7 months after reoperation, 10 out of 18 patients had died of tumor progression, 1 had a stable tumor, and 7 were tumor free. When TR was achieved, the overall survival rate was 7 out of 12. Total resection is the only curative treatment for RIE and is often possible, especially when the initial resection was total.

Intracranial ependymomas in children: A single institution experience

Intracranial ependymomas in children: A single institution experience

Intracranial ependymomas in children: A single institution experience

Intracranial ependymomas in children: A single institution experience

Recurrent intracranial ependymoma in children: salvage therapy with oral etoposide

Chronic oral VP-16 (etoposide) is a chemotherapy regimen with a wide application in oncology and documented efficacy against germ cell tumors, lymphomas, Kaposi’s sarcoma, and primary brain tumors. This study was performed to assess the toxicity and activity of chronic oral etoposide in the management of children with recurrent intracranial nondisseminated ependymoma. Twelve children (median age of 8 years) with recurrent ependymoma who were refractory to surgery, radiotherapy, and chemotherapy (carboplatinum or the combination of procarbazine, lomustine, and vincristine) were treated with chronic oral etoposide (50 mg/m 2/day). Treatment-related complications included the following: alopecia (10 children), diarrhea (6), weight loss (5), anemia (4), neutropenia (3), and thrombocytopenia (3). Three children required transfusion (two with packed red blood cells; two with platelets), and two children developed neutropenic fever. No treatment-related deaths occurred. Six children (50%) demonstrated either a radiographic response (two children, both with partial response) or stable disease (four children) with a median duration of response or stable disease of 7 months. In this small cohort of children with recurrent intracranial ependymoma, oral etoposide was well tolerated, produced modest toxicity, and had apparent activity.

Prognostic factors in intracranial ependymomas in children.

The occurrence of intracranial ependymomas in children is relatively infrequent, and their prognostic factors are still controversial, especially regarding histological composition. A retrospective study was conducted of 37 children treated during the last 20 years for intracranial ependymomas at the Hôpital de la Timone. Both univariate and multivariate statistical analyses were performed to assess the prognostic relevance of patient age and sex, extent of tumor removal, location of the tumor (supratentorial compared with infratentorial, median compared with lateral), tumor histological composition, and adjuvant therapies in affecting the 5-year progression-free survival (PFS) rate and overall survival (OS) rate. The following histopathological features, either alone or in combination, were analyzed: endothelial proliferation, necrosis, loss of differentiating structures (present compared with absent), the number of mitotic figures per 10 hpf, and cellularity (number of nuclei/5 hpf). In addition, immunohistochemical detection of Ki-67 antigen was performed and the Ki-67 labeling index (LI) evaluated in all cases. The 5-year OS and PFS rates were 45% and 25%, respectively (median follow up 34 months). Four patients died of disease without remission (median 163 days) and disease in 21 patients relapsed: 18 in situ and three both in situ and distantly. On univariate analysis total surgical resection and median infratentorial location were associated with a better outcome (p < 0.002) for both OS and PFS. Loss of differentiating structures was associated with poor prognosis (p < 0.008) and the combination of necrosis, endothelial proliferation, and mitotic index greater than 5 was also a negative predictive factor for both OS (p < 0.002) and PFS (p = 0.02). The PFS time was shorter in patients younger than 4 years of age and in patients in whom a Ki-67 LI greater than 1 was found (p = 0.03 and 0.006, respectively). Adjuvant radiotherapy and chemotherapy were not relevant to prognosis. Moreover, among the 15 patients in whom total excision was achieved, OS was better in those who did not receive adjuvant therapies. In contrast, adjuvant therapies significantly enhanced PFS time in patients in whom tumor excision was incomplete. This study and analysis of the literature further highlight that total tumor removal is the treatment of choice for ependymomas in children. Postoperative measurement of residual tumor is required, especially because a subgroup of patients might be treated by surgery alone. Median infratentorial ependymomas have to be distinguished from the lateral type. Appropriate and reproducible histological parameters and Ki-67 LI are of interest as predictors of outcome.

Comment: Intracranial ependymomas in children

Medical and Pediatric OncologyVolume 30, Issue 6 p. 330-330 Comment Comment: Intracranial ependymomas in children Roger Packer, Corresponding Author Roger Packer Department of Neurology, Children's National Medical Center, Washington, D.C.Department of Neurology, Children's National Medical Center, Washington, D.C.Search for more papers by this author Roger Packer, Corresponding Author Roger Packer Department of Neurology, Children's National Medical Center, Washington, D.C.Department of Neurology, Children's National Medical Center, Washington, D.C.Search for more papers by this author First published: 07 December 1998 https://doi.org/10.1002/(SICI)1096-911X(199806)30:6<330::AID-MPO4>3.0.CO;2-FAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Volume30, Issue6June 1998Pages 330-330 RelatedInformation

Intracranial ependymomas in children: A critical review of prognostic factors and a plea for cooperation

Intracranial ependymomas in children: A critical review of prognostic factors and a plea for cooperation

Comment: Intracranial ependymomas in children

Comment: Intracranial ependymomas in children

Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation.

Current controversies in pediatric intracranial ependymoma include histologic categorization and management. Most of our knowledge of this disease comes from single-institution reports. A literature search was done, covering the period 1976-1996. The aim of this review is to analyze the prognostic factors reported in the literature over the last 20 years. Forty-five series were reviewed, including more than 1,400 children. The largest series reported on 92 patients, and the accrual rate ranged from 0.32-12 patients per year. None of the prognostic factors reported achieved a consensus throughout the different series. Histology remains a major issue, and the range in the incidence of anaplastic ependymo mas (7-89%) highlights the difficulty in agreeing on a histological grading system. The role of surgery on the outcome seems to be determinant. Recent series based on homogeneous imaging-documented extents of resection strongly support the benefit of postoperative radiotherapy. The lack of a proven, effective chemotherapy regimen precludes its use except in prospective pilot studies. Limited information is available from single-institution reports in ependymoma. Only large national or international studies can provide enough information to allow a multivariate analysis of the prognostic factors, and thus lead to new therapeutic proposals.