Tag: radiation therapy

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Guidelines for the Treatment of Adults With WHO Grade II Diffuse Glioma: Update

Neurosurgery 98:984–991, 2026

These updated CNS evidence-based guidelines consolidate current literature on the management of WHO grade II diffuse gliomas, covering imaging, surgical strategies, neuropathology, radiotherapy, chemotherapy, and recurrence. They summarize evidence levels, provide topic-specific recommendations, and identify areas needing higher-quality research to inform clinical decisions.

The document details unchanged, updated, and new recommendations—ranging from MRI protocols and intraoperative mapping to molecular testing and adjuvant therapy—while describing methodology, conflict-of-interest procedures, and plans for periodic revision to ensure clinical relevance.

Scope: Updated evidence-based recommendations for adult WHO grade II diffuse glioma across imaging, surgery, neuropathology/molecular markers, radiotherapy, chemotherapy, and recurrence management.

Methods: Systematic search of MEDLINE/Embase/Cochrane covering Jan 1, 2013–Jan 31, 2020, with recommendations based on evidence rather than consensus.

Imaging (minimum MRI): Minimum evaluation includes T2-weighted and pre- and post-gadolinium T1-weighted MRI; serial imaging is used to detect new enhancement or significant growth suggesting higher-grade transformation.

Imaging (advanced/PET): Diffusion plus perfusion techniques (e.g., DSC/DCE/ASL) are suggested when standard MRI cannot distinguish grade II from higher-grade gliomas; amino-acid PET (FET or FDOPA) may be added when not evident on MRI to help with grading, prognosis (FET), or progression detection (FDOPA).

Surgery: When safe/feasible, resection (gross total or subtotal) is recommended over biopsy alone to reduce progression; extent of resection should be maximized when safely possible (including for IDH-mutant and IDH–wild-type tumors).

Pathology/molecular testing: Diagnosis relies on histopathology from representative tissue; IDH mutation testing is recommended for classification/prognosis, and 1p/19q loss-of-heterozygosity testing is recommended in oligodendroglial cases for prognosis/treatment planning.

Adjuvant therapy (high-risk): For high-risk grade II diffuse glioma (age >40 or <40 without gross total resection), adding chemotherapy to radiotherapy is recommended to improve overall survival (including procarbazine/PCV-based approaches) and is also recommended without decline in neurocognitive function.

Recurrence: At suspected recurrence, advanced imaging (MRS/perfusion/diffusion/PET) is suggested; temozolomide is suggested (often initial choice) and procarbazine may help (strongest evidence in oligodendroglioma); radiation is suggested if not previously given and reirradiation may be considered.

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Longitudinal brain volumetrics in glioma survivors

J Neurosurg 141:634–641, 2024

Radiation therapy (RT) is used selectively for patients with low-grade glioma (LGG) given the concerns for potential cognitive effects in survivors, but prior cognitive outcome studies among LGG survivors have had inconsistent findings. Translational studies that characterize changes in brain anatomy and physiology after treatment of LGG may help to both contextualize cognitive findings and improve the overall understanding of radiation effects in normal brain tissue. This study aimed to investigate the hypothesis that patients with LGG who are treated with RT will experience greater brain volume loss than those who do not receive RT.

METHODS This retrospective longitudinal study included all patients with WHO grade 2 glioma who received posttreatment surveillance MRI at the University of Alabama at Birmingham. Volumetric analysis of contralateral cortical white matter (WM), cortical gray matter (GM), and hippocampus was performed on all posttreatment T1-weighted MRI sequences using the SynthSeg script. The effect of clinical and treatment variables on brain volumes was assessed using two-level hierarchical linear models.

RESULTS The final study cohort consisted of 105 patients with 1974 time points analyzed. The median length of imaging follow-up was 4.6 years (range 0.36–18.9 years), and the median number of time points analyzed per patient was 12 (range 2–40). Resection was performed in 79 (75.2%) patients, RT was administered to 61 (58.1%) patients, and chemotherapy was administered to 66 (62.9%) patients. Age at diagnosis (β = −0.06, p < 0.001) and use of RT (β = −1.12, p = 0.002) were associated with the slope of the contralateral cortical GM volume model (i.e., change in GM over time). Age at diagnosis (β = −0.08, p < 0.001), midline involvement (β = 1.31, p = 0.006), and use of RT (β = −1.45, p = 0.001) were associated with slope of the contralateral cortical WM volume model. Age (β = −0.0027, p = 0.001), tumor resection (β = −0.069, p < 0.001), use of chemotherapy (β = −0.0597, p = 0.003), and use of RT (β = −0.0589, p < 0.001) were associated with the slope of the contralateral hippocampus volume model.

CONCLUSIONS This study demonstrated volume loss in contralateral brain structures among LGG survivors, and patients who received RT experienced greater volume loss than those who did not. The results of this study may help to provide context for cognitive outcome research in LGG survivors and inform the design of future strategies to preserve cognition.

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Predictors of Progression-Free Survival in Patients With Spinal Intramedullary Ependymoma: A Multicenter Retrospective Study by the Neurospinal Society of Japan

Neurosurgery 93:1046–1056, 2023

Ependymoma is the most common spinal intramedullary tumor. Although clinical outcomes have been described in the literature, most of the reports were based on limited numbers of cases or been confined to institutional experience. The objective of this study was to analyze more detailed characteristics of spinal intramedullary ependymoma (SIE) and provide clinical factors associated with progression-free survival (PFS).

METHODS: This retrospective observational multicenter study included consecutive patients with SIE in the cervical or thoracic spine treated surgically at a total of 58 institutions between 2009 and 2020. The results of pathological diagnosis at each institute were confirmed, and patients with myxopapillary ependymoma, subependymoma, or unverified histopathology were strictly excluded from this study. Outcome measures included surgical data, surgery-related complications, postoperative systemic adverse events, postoperative adjuvant treatment, postoperative functional condition, and presence of recurrence.

RESULTS: This study included 324 cases of World Health Organization grade II (96.4%) and 12 cases of World Health Organization grade III (3.6%). Gross total resection (GTR) was achieved in 76.5% of cases. Radiation therapy (RT) was applied after surgery in 16 cases (4.8%), all of which received local RT and 5 of which underwent chemotherapy in combination. Functional outcomes were significantly affected by preoperative neurological symptoms, tumor location, extent of tumor resection, and recurrence. Multivariate regression analysis suggested that limited extent of tumor resection or recurrence resulted in poor functional outcomes. Multiple comparisons among the groups undergoing GTR, subtotal resection and biopsy, or partial resection of the tumor showed that the probability of PFS differed significantly between GTR and other extents of resection.

CONCLUSION: When GTR can be safely obtained in the surgery for SIE, functional maintenance and longer PFScan be expected.

 

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Novel Postoperative Serum Biomarkers in Atypical Meningiomas: A Multicenter Study

Neurosurgery 93:599–610, 2023

There has been no known serum biomarker to predict the prognosis of atypical meningioma.

OBJECTIVE: To investigate the prognostic impact of serum biomarkers in patients newly diagnosed with resected intracranial atypical meningiomas.

METHODS: This study enrolled 523 patients with atypical meningioma who underwent surgical resection between 1998 and 2018 from 5 Asian institutions. Serum laboratory data within 1 week after surgery were obtained for analysis. Optimal cutoffs were calculated for each serum marker using the maxstat package of R.

RESULTS: Of 523 patients, 19.5% underwent subtotal resection and 29.8% were treated with adjuvant radiation therapy (ART). Among the 523 patients, 454 were included in the multivariate analysis for the progression/recurrence (P/R) rate excluding patients with incomplete histopathologic or laboratory data. On multivariate analysis, tumor size >5 cm, subtotal resection, and postoperative aspartate aminotransferase/alanine transaminase (De Ritis) ratio >2 were associated with higher P/R rates, whereas ART and postoperative platelet count >137 × 103 / μ L were associated with lower P/R rates. In the subgroup of patients treated with ART, tumor size >5 cm and postoperative neutrophil-to-lymphocyte ratio >21 were associated with higher P/R rates. By contrast, postoperative De Ritis ratio >2 remained an adverse prognosticator in patients not treated with ART.

CONCLUSION: Postoperative De Ritis ratio, platelet count, and neutrophil-to-lymphocyte ratio were revealed as a novel serum prognosticator in newly diagnosed atypical meningiomas. Additional studies are warranted to validate its clinical significance and biological background.

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Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on the Management of Progressive Glioblastoma in Adults: Update of the 2014 Guidelines

Neurosurgery 90:E112–E115, 2022

The Institute of Medicine best practice recommendation to review guidelines every 5 years is followed by the Congress of Neurological Surgeons Guidelines Committee. The aim of this work was to provide an updated literature review and evidence-based recommendations on the topic of diagnosis and treatment of patients with progressive glioblastoma (pGBM).

OBJECTIVE: To review the literature published since the last guidelines on pGBM dated 2014, with literature search ending in June 2012.

METHODS: PubMed, Embase, and Cochrane were searched for the period July 1, 2012, to March 31, 2019, using search terms and search strategies to identify pertinent abstracts. These were then screened using published exclusion/inclusion criteria to identify full-text review articles. Evidence tables were constructed using data derived from full-text reviews and recommendations made from the evidence derived.

RESULTS: From the total 8786 abstracts identified by the search, 237 full-text articles met inclusion/exclusion criteria and were included in this update. Two new level II recommendations derived from this work. For the diagnosis of patients with GBM, the use of diffusion-weighted images is recommended to be included in the magnetic resonance images with and without contrast used for surveillance to detect pGBM. For the treatment of patients with pGBM, repeat cytoreductive surgery is recommended to improve overall survival. An additional 21 level III recommendations were provided.

CONCLUSION: Recent published literature provides new recommendations for the diagnosis and treatment of pGBM. The Central Nervous System Guidelines Committee will continue to pursue timely updates to further improve the care of patients with diagnosis. https://www.cns.org/guidelines/browse-guidelines-detail/guidelines-management-ofprogressive-glioblastoma.

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Long-term outcomes of high-dose single-fraction radiosurgery for chordomas of the spine and sacrum

J Neurosurg Spine 32:79–88, 2020

The current treatment of chordomas is associated with significant morbidity, high rates of local recurrence, and the potential for metastases. Stereotactic radiosurgery (SRS) as a primary treatment could reduce the need for en bloc resection to achieve wide or marginal margins. Spinal SRS outcomes support the exploration of SRS’s role in the durable control of these conventionally radioresistant tumors. The goal of the study was to evaluate outcomes of patients with primary chordomas treated with spinal SRS alone or in combination with surgery.

METHODS Clinical records were reviewed for outcomes of patients with primary chordomas of the mobile spine and sacrum who underwent single-fraction SRS between 2006 and 2017. Radiographic local recurrence-free survival (LRFS), overall survival (OS), symptom response, and toxicity were assessed in relation to the extent of surgery.

RESULTS In total, 35 patients with de novo chordomas of the mobile spine (n = 17) and sacrum (n = 18) received SRS and had a median post-SRS follow-up duration of 38.8 months (range 2.0–122.9 months). The median planning target volume dose was a 24-Gy single fraction (range 18–24 Gy). Overall, 12 patients (34%) underwent definitive SRS and 23 patients (66%) underwent surgery and either neoadjuvant or postoperative adjuvant SRS. Definitive SRS was selectively used to treat both sacral (n = 7) and mobile spine (n = 5) chordomas. Surgical strategies for the mobile spine were either intralesional, gross-total resection (n = 5) or separation surgery (n = 7) and for the sacrum en bloc sacrectomy (n = 11). The 3- and 5-year LRFS rates were 86.2% and 80.5%, respectively. Among 32 patients (91%) receiving 24-Gy radiation doses, the 3- and 5-year LRFS rates were 96.3% and 89.9%, respectively. The 3- and 5-year OS rates were 90.0% and 84.3%, respectively. The symptom response rate to treatment was 88% for pain and radiculopathy. The extent or type of surgery was not associated with LRFS, OS, or symptom response rates (p > 0.05), but en bloc resection was associated with higher surgical toxicity, as measured using the Common Terminology Criteria for Adverse Events (version 5.0) classification tool, than epidural decompression and curettage/intralesional resection (p = 0.03). The long-term rate of toxicity ≥ grade 2 was 31%, including 20% grade 3 tissue necrosis, recurrent laryngeal nerve palsy, myelopathy, fracture, and secondary malignancy.

CONCLUSIONS High-dose spinal SRS offers the chance for durable radiological control and effective symptom relief with acceptable toxicity in patients with primary chordomas as either a definitive or adjuvant therapy.

 

 

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Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for the Treatment of Adults With Metastatic Brain Tumors: Executive Summary

Neurosurgery 84:550–552, 2019

The Congress of Neurological Surgeons systematic review and evidencebased clinical practice parameter guidelines for the treatment of adults with metastatic brain tumorswas first published in 2010. Because of the time elapsed since that publication, an update of this set of guidelines based on literature published since is now indicated.

OBJECTIVE: To establish the best evidence-based management of metastatic brain tumors over all commonly used diagnostic and treatment modalities in regularly encountered clinical situations.

METHODS: Literature searches regarding management of metastatic brain tumors with whole brain radiation therapy, surgery, stereotactic radiosurgery, chemotherapy, prophylactic anticonvulsants, steroids, instances of multiple brain metastases, and emerging and investigational therapies were carried out to answer questions designed by consensus of a multidisciplinary writing group.

RESULTS: Recommendations were created and their strength linked to the quality of the literature data available thus creating an evidence-based guideline. Importantly, shortcomings and biases to the literature data are brought out so as to provide guidance for future investigation and improvements in the management of patients with metastatic brain tumors.

CONCLUSION: This series of guidelines was constructed to assess the most current and clinically relevant evidence for management of metastatic brain tumors. They set a benchmark regarding the current evidence base for this management while also highlighting important key areas for future basic and clinical research, particularly on those topics for which no recommendations could be formulated.

The full guideline can be found here.

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Stereotactic Radiosurgery for Intracranial Ependymomas: An International Multicenter Study

Neurosurgery 84:227–234, 2019

Stereotactic radiosurgery (SRS) is a potentially important option for intracranial ependymoma patients.

OBJECTIVE: To analyze the outcomes of intracranial ependymoma patients who underwent SRS as a part of multimodality management.

METHODS: Seven centers participating in the International Gamma Knife Research Foundation identified 89 intracranial ependymoma patients who underwent SRS (113 tumors). The median patient age was 16.3 yr (2.9-80). All patients underwent previous surgical resection and radiation therapy (RT) of their ependymomas and 40 underwent previous chemotherapy. Grade 2 ependymomas were present in 42 patients (52 tumors) and grade 3 ependymomas in 48 patients (61 tumors). The median tumor volume was 2.2 cc (0.03-36.8) and the median margin dose was 15 Gy (9-24).

RESULTS: Forty-seven (53%) patients were alive and 42 (47%) patients died at the last follow-up. The overall survival after SRSwas 86% at 1 yr, 50% at 3 yr, and 44% at 5 yr. Smaller total tumor volume was associated with longer overall survival (P = .006). Twenty-two patients (grade 2: n = 9, grade 3: n = 13) developed additional recurrent ependymomas in the craniospinal axis. The progression-free survival after SRSwas 71%at 1 yr, 56% at 3 yr, and 48% at 5 yr. Adult age, female sex, and smaller tumor volume indicated significantly better progression-free survival. Symptomatic adverse radiation effects were seen in 7 patients (8%).

CONCLUSION: SRS provides another management option for residual or recurrent progressive intracranial ependymoma patients who have failed initial surgery and RT.

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Multimodality Treatment of Skull Base Chondrosarcomas

Neurosurgery 81:520–530, 2017

Limited data exist to guide the multimodality management of chondrosarcomas (CSAs) arising in the skull base.

OBJECTIVE : To determine the impact of histological subtype/grade on progression-free survival (PFS) and the indications for surgery, radiation, and chemotherapy based on histology.

METHODS: A retrospective review was performed of 37 patients (conventional type: 81%, mesenchymal: 16.2%, dedifferentiated: 2.7%) treated at The University of Texas M.D. Anderson Cancer Center. Of the conventional subtype, 23% were grade 1, 63% were grade 2, and 14% were grade 3. In addition to surgery, mesenchymal/dedifferentiated CSAs (18% of the cohort) underwent neoadjuvant chemotherapy and 48.6% of the overall cohort received adjuvant radiotherapy. Histological grade/subtype and treatment factors were assessed for impact on median PFS (primary outcome).

RESULTS: Conventional subtype vs mesenchymal/dedifferentiated was positively associated with median PFS (166 vs 24 months, P < .05). Increasing conventional grade inversely correlated with median PFS (P < .05). Gross total resection positively impacted PFS in conventionalCSAs (111.8 vs 42.9months, P=.201) and mesenchymal/dedifferentiated CSAs (58.2 vs 1.0 month, P < .05). Adjuvant radiotherapy significantly impacted PFS in conventional grades 2 and 3 (182 vs 79 months, P < .05) and a positive trend with mesenchymal/dedifferentiated CSAs (43.5 vs 22.0 months). Chemotherapy improved PFS for mesenchymal/dedifferentiated CSAs (50 vs 9 months, P = .089).

CONCLUSION: There is a potential need for histological subtype/grade specific treatment protocols. For conventional CSAs, surgery alone provides optimal results grade 1 CSAs, while resection with adjuvant radiotherapy yields the best outcomefor grade 2 and 3 CSAs. Improvements in PFS seen with neoadjuvant therapy in mesenchymal/dedifferentiated CSAs indicate a potential role for systemic therapies. Larger studies are necessary to confirm the proposed treatment protocols.

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Radiation Therapy for Residual or Recurrent Atypical Meningioma

Radiation Therapy for Residual or Recurrent Atypical Meningioma

Neurosurgery 79:23–32, 2016

Optimal use of stereotactic radiosurgery (SRS) vs external beam radiation therapy (EBRT) for treatment of residual/recurrent atypical meningioma is unclear.

OBJECTIVE: To analyze features associated with progression after radiation therapy.

METHODS: Fifty radiation-naive patients who received SRS or EBRT for residual and/or recurrent atypical meningioma were examined for predictors of progression using Cox regression and Kaplan-Meier analyses.

RESULTS: Thirty-two patients (64%) received adjuvant radiation after subtotal resection, 12 patients (24%) received salvage radiation after progression following subtotal resection, and 6 patients (12%) received salvage radiation after recurrence following gross total resection. Twenty-one patients (42%) received SRS (median 18 Gy), and 7 (33%) had tumor progression. Twenty-nine patients (58%) received EBRT (median 54 Gy), and 13 (45%) had tumor progression. Whereas tumor volume (P = .53), SRS vs EBRT (P = .45), and adjuvant vs salvage (P = .34) were not associated with progression after radiation therapy, spontaneous necrosis (hazard ratio [HR] = 82.3, P < .001), embolization necrosis (HR = 15.6, P = .03), and brain invasion (HR = 3.8, P = .008) predicted progression in univariate and multivariate analyses. Tumors treated with SRS/EBRT had 2- and 5-year actuarial locoregional control rates of 91%/88% and 71%/69%, respectively. Tumors with spontaneous necrosis, embolization necrosis, and no necrosis had 2- and 5-year locoregional control rates of 76%, 92%, and 100% and 36%, 73%, and 100%, respectively (P < .001).

CONCLUSION: This study suggests that necrosis may be a negative predictor of radiation response regardless of radiation timing or modality.

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High-dose proton-based radiation therapy in the management of spine chordomas

spine_chordoma

J Neurosurg Spine 23:788–797, 2015

Spinal chordomas can have high local recurrence rates after surgery with or without conventional dose radiation therapy (RT). Treatment outcomes and prognostic factors after high-dose proton-based RT with or without surgery were assessed.

Methods The authors conducted a retrospective review of 126 treated patients (127 lesions) categorized according to disease status (primary vs recurrent), resection (en bloc vs intralesional), margin status, and RT timing.

Results Seventy-one sacrococcygeal, 40 lumbar, and 16 thoracic chordomas were analyzed. Mean RT dose was 72.4 GyRBE (relative biological effectiveness). With median follow-up of 41 months, the 5-year overall survival (OS), local control (LC), locoregional control (LRC), and distant control (DC) for the entire cohort were 81%, 62%, 60%, and 77%, respectively. LC for primary chordoma was 68% versus 49% for recurrent lesions (p = 0.058). LC if treated with a component of preoperative RT was 72% versus 54% without this treatment (p = 0.113). Among primary tumors, LC and LRC were higher with preoperative RT, 85% (p = 0.019) and 79% (0.034), respectively, versus 56% and 56% if no preoperative RT was provided. Overall LC was significantly improved with en bloc versus intralesional resection (72% vs 55%, p = 0.016), as was LRC (70% vs 53%, p = 0.035). A trend was noted toward improved LC and LRC for R0/R1 margins and the absence of intralesional procedures.

Conclusions High-dose proton-based RT in the management of spinal chordomas can be effective treatment. In patients undergoing surgery, those with primary chordomas undergoing preoperative RT, en bloc resection, and postoperative RT boost have the highest rate of local tumor control; among 28 patients with primary chordomas who underwent preoperative RT and en bloc resection, no local recurrences were seen. Intralesional and incomplete resections are associated with higher local failure rates and are to be avoided.

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A clinical trial of bevacizumab, temozolomide, and radiation for newly diagnosed glioblastoma

J Neurosurg 116:341–345, 2012.DOI: 10.3171/2011.9.JNS11656

The presence of angiogenesis is a hallmark of glioblastoma (GBM). Vascular endothelial growth factor (VEGF), which drives angiogenesis, provides an additional target for conventional therapy. The authors conducted a prospective clinical trial to test the effectiveness of bevacizumab, an inhibitor of VEGF, in newly diagnosed GBM.

Methods. From 2006 through 2010, 51 eligible patients with newly diagnosed GBM were treated with involvedfield radiation therapy and concomitant temozolomide (75 mg/m2 daily for 42 days) along with bevacizumab (10 mg/ kg every 2 weeks), starting 29 days after surgery. This was followed by 6 cycles of adjuvant temozolomide therapy (150 mg/m2 on Days 1–7 of a 28-day cycle) with bevacizumab administered at 10 mg/kg on Days 8 and 22 of each 28-day cycle.

Results. The 6- and 12-month progression-free survival (PFS) rates were 85.1% and 51%, respectively. The 12- and 24-month overall survival (OS) rates were 85.1% and 42.5%, respectively. Grade III/IV toxicities were noted in 10 patients (19.6%). No treatment-related deaths were observed. Asymptomatic intracranial bleeding was noted in 5 patients.

Conclusions. The addition of bevacizumab to conventional therapy in newly diagnosed GBM appears to improve both PFS and OS in patients with newly diagnosed GBM, with acceptable morbidity. A shift toward diffuse relapse was noted in a significant number of patients. Ongoing Phase III clinical trials will show the true benefit of this antiangiogenic approach.

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Integrated Genomic Analysis Identifies Clinically Relevant Subtypes of Glioblastoma Characterized by Abnormalities in PDGFRA, IDH1, EGFR, and NF1

Cancer Cell 17, 98–110, January 19, 2010

The Cancer Genome Atlas Network recently cataloged recurrent genomic abnormalities in glioblastoma multiforme (GBM). We describe a robust gene expression-based molecular classification of GBM into Proneural, Neural, Classical, and Mesenchymal subtypes and integrate multidimensional genomic data to establish patterns of somatic mutations and DNA copy number. Aberrations and gene expression of EGFR, NF1, and PDGFRA/IDH1 each define the Classical, Mesenchymal, and Proneural subtypes, respectively. Gene signatures of normal brain cell types show a strong relationship between subtypes and different neural lineages. Additionally, response to aggressive therapy differs by subtype, with the greatest benefit in the Classical subtype and no benefit in the Proneural subtype. We provide a framework that unifies transcriptomic and genomic dimensions for GBM molecular stratification with important implications for future studies.

This work expands on previous glioblastoma classification studies by associating known subtypes with specific alterations in NF1 and PDGFRA/IDH1 and by identifying two additional subtypes, one of which is characterized by EGFR abnormalities and wild-type p53. In addition, the subtypes have specific differentiation characteristics that, combined with data from recent mouse studies, suggest a link to alternative cells of origin. Together, these data provide a framework for investigation of targeted therapies. Temozolomide and radiation, a common treatment for glioblastoma, has demonstrated a significant increase in survival. Our analysis illustrates that a survival advantage in heavily treated patients varies by subtype, with Classical or Mesenchymal subtypes having significantly delayed mortality that was not observed in the Proneural subtype


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Infratentorial ependymomas: prognostic factors and outcome analysis in a multi-center retrospective series of 106 adult patients

Acta Neurochirurgica (151)8: 947 – 960. 01/08/2009

This study was undertaken to analyze outcomes and to assess the prognostic impact of age, location, surgery, radiotherapy (RT), and histopathology in a series of adult infratentorial ependymomas. This was a retrospective study of a population of 106 adult patients with infratentorial ependymomas diagnosed between 1990 and 2004. A central pathological review of all cases was performed. Grading was according to the WHO and Marseille’s neograding classifications. The series consisted of 58 males (54.7%) and 48 females (45.3%) in the age range of 18-82 years. Using the WHO classification, 88 patients (83.0%) had grade II and 18 patients (17.0%) grade III ependymomas. Using the Marseille’s neograding system, 91 patients were low-grade and 15 high-grade. Gross total resection was achieved in 66 patients (62.3%). Thirty-seven patients (35.0%) received adjuvant RT. The 5- and 10-year overall survival rates for the entire cohort were 86.1% and 80.5%, respectively. On multivariate analysis, a preoperative Karnofski performance status score > 80, no recessus lateral extension and a low histological grade (Marseille’s grading) were associated with a longer overall survival. The 5- and 10-year progression-free survival rates for the entire cohort were 70.8% and 57.7%, respectively. On multivariate analysis, no recessus lateral extension, gross total resection and a low histological grade (Marseille’s grading) were associated with a longer progression-free survival. Adjuvant RT was significantly associated with a better overall and progression-free survival in incompletely resected WHO grade II ependymomas. This study highlights the key role of histology in the clinical outcome and the fact that gross total resection is a main prognostic factor and the treatment of choice for posterior fossa ependymomas. The use of adjuvant RT in patients with incompletely resected WHO grade II ependymomas appears beneficial, but its effect on high-grade tumors remains to be determined.

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