Tag: ependymoma

Posted on

Impact of Molecular Subgroups on Prognosis and Survival Outcomes in Posterior Fossa Ependymomas: A Retrospective Study of 412 Cases

Neurosurgery 95:651–659, 2024

Posterior fossa ependymomas (PFEs) are rare brain tumors classified as PF-EPN-A (PFA) and PF-EPN-B (PFB) subgroups. The study aimed to evaluate the prognosis and survival outcomes in PFEs, with a focus on the impact of molecular subgroups.

METHODS: A retrospective study was conducted on 412 patients with PFEs. Kaplan–Meier survival analyses were conducted to evaluate the overall survival (OS) and progression-free survival. Cox regression analyses were conducted to assess the prognostic factors. A nomogram was developed to predict the OS rates of PFEs.

RESULTS: The study revealed significant differences between PFA and PFB in patient and tumor characteristics. PFAs were associated with poorer OS (hazard ratios [HR] 3.252, 95% CI 1.777-5.950, P < .001) and progression-free survival (HR 4.144, 95% CI 2.869-5.985, P < .001). World Health Organization grade 3 was associated with poorer OS (HR 2.389, 95% CI 1.236-4.617, P = .010). As for treatment patterns, gross total resection followed by radiotherapy or the combination of radiotherapy and chemotherapy yielded the most favorable OS for PFA (P = .025 for both), whereas gross total resection followed by radiotherapy rather than observation showed improved OS for PFB (P = .046). The nomogram demonstrated a high degree of accuracy and discrimination capacity for the prediction of OS rates for up to 10 years. In addition, 6 cases of PFA (3.51%) with H3K27M mutations were identified.

CONCLUSION: PFAs demonstrate worse prognosis and survival outcomes compared with PFBs. Both PFAs and PFBs necessitate maximal resection followed by intensive adjuvant therapies in long-term effects.

Posted on

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.

 

Posted on

Perioperative outcomes and survival after surgery for intramedullary spinal cord tumors: a single-institution series of 302 patients

J Neurosurg Spine 37:252–262, 2022

Intramedullary spinal cord tumors (IMSCTs) are rare neoplasms whose treatment is often technically challenging. Given the low volume seen at most centers, perioperative outcomes have been reported infrequently. Here, the authors present the largest single-institution series of IMSCTs, focusing on the clinical presentation, histological makeup, perioperative outcomes, and long-term survival of surgically treated patients.

METHODS A cohort of patients operated on for primary IMSCTs at a comprehensive cancer center between June 2002 and May 2020 was retrospectively identified. Data on patient demographics, tumor histology, neuraxial location, baseline neurological status, functional deficits, and operative characteristics were collected. Perioperative outcomes of interest included length of stay, postoperative complications, readmission, reoperation, and discharge disposition. Data were compared across tumor histologies using the Kruskal-Wallis H test, chi-square test, and Fisher exact test. Pairwise comparisons were conducted using Tukey’s honest significant difference test, chi-square test, and Fisher exact test. Long-term survival was assessed across tumor categories and histological subtype using the log-rank test.

RESULTS Three hundred two patients were included in the study (mean age 34.9 ± 19 years, 77% white, 57% male). The most common tumors were ependymomas (47%), astrocytomas (31%), and hemangioblastomas (11%). Ependymomas and hemangioblastomas disproportionately localized to the cervical cord (54% and 59%, respectively), whereas astrocytomas were distributed almost equally between the cervical cord (36%) and thoracic cord (38%). Clinical presentation, extent of functional dependence, and postoperative 30-day outcomes were largely independent of underlying tumor pathology, although tumors of the thoracic cord had worse American Spinal Injury Association (ASIA) grades than cervical tumors. Rates of gross-total resection were lower for astrocytomas than for ependymomas (54% vs 84%, p < 0.01) and hemangioblastomas (54% vs 100%, p < 0.01). Additionally, 30-day readmission rates were significantly higher for astrocytomas than ependymomas (14% vs 6%, p = 0.02). Overall survival was significantly affected by the underlying pathology, with astrocytomas having poorer associated prognoses (40% at 15 years) than ependymomas (81%) and hemangioblastomas (66%; p < 0.01) and patients with high-grade ependymomas and astrocytomas having poorer longterm survival than those with low-grade lesions (p < 0.01).

CONCLUSIONS The neuraxial location of IMSCTs, extent of resection, and postoperative survival differed significantly across tumor pathologies. However, perioperative outcomes did not vary significantly across tumor cohorts, suggesting that operative details, rather than pathology, may have a stronger influence on the short-term clinical course, whereas pathology appears to have a stronger impact on long-term survival.

Posted on

Adult Posterior Fossa Anaplastic Ependymoma

World Neurosurg. (2022) 158:205-209

Ependymomas are rare central nervous system tumors. The current treatment strategy is gross total tumor removal. Whether adjuvant therapy will be beneficial is controversial. We retrospectively analyzed 3 cases of World Health Organization (WHO) grade III posterior fossa anaplastic ependymomas treated with different treatment modalities. We aimed to identify possible treatment options for infratentorial WHO grade III anaplastic ependymoma in adults.

METHODS: We performed a retrospective analysis of 3 patients diagnosed with infratentorial anaplastic ependymomas in our institution from 2016 to 2020. The demographic data were documented. This case series of 3 patients does not meet the Department of Health and Human Services definition of research and does not need Institutional Review Board approval. All patients’ informed consents have been obtained.

RESULTS: One patient underwent subtotal tumor resection combined with adjuvant radiotherapy and Gamma Knife radiosurgery while the other 2 patients underwent gross total tumor removal combined with Gamma Knife radiosurgery or adjuvant radiotherapy. Tumors recurred in the first patient 20 months later, while the other 2 patents did not develop recurrence. The modified Rankin scale scores of these patients were 1, 0, and 0. All patients are followed up with regular magnetic resonance imaging at our facility.

CONCLUSIONS: The strategy for treating WHO grade III anaplastic ependymomas is controversial, but gross total tumor resection remains the key element. Adjuvant stereotactic radiosurgery after tumor removal might be considered if radiotherapy is not an option. The role of chemotherapy is unclear, and the use of chemotherapy should be tailored to individual patients

Posted on

Pediatric Supratentorial Ependymoma: Surgical, Clinical, and Molecular Analysis

Neurosurgery 85:41–49, 2019

Pediatric supratentorial ependymomas (SEs) have distinct molecular and behavioral differences from their infratentorial counterparts.

OBJECTIVE: To present our experience with pediatric SEs over a 24-yr period.

METHODS: Clinical, operative, and radiographic information was abstracted retrospectively. Our primary outcomes were progression-free survival (PFS) and overall survival (OS). Detection of C11or f95-RELA rearrangement was performed using interphase fluorescence in situ hybridization (iFISH).

RESULTS: Seventy-three patients were identified (41 female, 32 male); median age was 6.7 yrs (range, 1 mo-18.8 yr); median follow-up was 8.3 yrs (range, 2.0-26.3). Fifty-eight (79.5%) of 73 patients underwent gross total resection (GTR); no patient with subtotal resection had greater than 1 cm3 of residual tumor; 42 patients (57.5%) experienced subsequent disease progression with 17 patients ultimately dying of their disease. Median PFS was 3.7 yrs. Molecular analysis was available for 51 patients (70%). On bivariate analysis, PFS and OS were not statistically affected by age, tumor grade, or extent of resection, although there was a clinically significant trend for the latter in favor of aggressive resection on PFS (P = .061). Children with RELA fusion had significantly higher PFS (P = .013) than those without, although there was no difference in OS when compared with those with no C11orf95-RELA fusion or C11orf95 gene rearrangement alone.

CONCLUSION: In our series, GTR may be associated with better PFS, but did not impact OS. Surprisingly, RELA fusion was not found to be a negative prognostic factor, raising the possibility that the deleterious effects may be overcome by aggressive resection.

Posted on

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.

Posted on

Circulating serum oncologic miRNA in pediatric juvenile pilocytic astrocytoma patients predicts mural nodule volume

Acta Neurochirurgica (2018) 160:1571–1581

Juvenile pilocytic astrocytomas represent the largest group of pediatric brain tumors. The ideal management for these tumors is early, total surgical resection. To detect and track treatment response, a screening tool is needed to identify patients for surgical evaluation and assess the quality of treatment. The identification of aberrant miRNA profiles in the sera of juvenile pilocytic astrocytoma patients could provide such a screening tool.

Methods The authors reviewed the serum profiles of 84 oncologically relevant miRNAs in pediatric juvenile pilocytic astrocytoma patients via qPCR screening.

Results miR-21, miR-15b, miR-23a, and miR-146b were significantly elevated in the sera of JPA patients as compared to nononcologic controls, oncologic controls, and post-JPA resection samples (p < 0.001, 0.022, 0.034, 0.044). miR-21 had the highest AUC on ROC analysis (AUC > 0.99, sensitivity 75%, specificity 100%). All four miRNAs also correlated well with tumor mural nodule size, though they only poorly correlated with total tumor size, including cystic components (Spearman’s R2: miR-21 91.7 vs 6.9%, miR-15b 86.3 vs 23.1%, miR-23a 85.8 vs 23.0%, miR-146b 59.8 vs 11.9%).

Conclusion In this small pilot study, pediatric juvenile pilocytic astrocytoma patients had significant elevations in serum miR-21, miR-15b, miR-23a, and miR-146b levels that do not appear to be driven by hydrocephalus or local distortion of the intracranial contents. These alterations correlate with solid tumor component volume and reverse with complete tumor resection, suggesting that this serum miRNA profile may delineate biomarkers for screening and tracking juvenile pilocytic astrocytoma patients. Additional studies, with a larger cohort, are needed to verify these results.

Posted on

Minimally Invasive Microsurgical Resection of Primary, Intradural Spinal Tumors is Feasible and Safe: A Consecutive Series of 83 Patients

Neurosurgery 82:365–371, 2018

To date, the traditional approach to intraspinal tumors has been open laminectomy or laminoplasty followed by microsurgical tumor resection. Recently, however, minimally invasive approaches have been attempted by some.

OBJECTIVE: To investigate the feasibility and safety of minimally invasive surgery (MIS) for primary intradural spinal tumors.

METHODS:Medical charts of 83 consecutive patients treated with MIS for intradural spinal tumors were reviewed. Patients were followed up during the study year, 2015, by either routine history/physical examination or by telephone consultation, with a focus on tumor status and surgery-related complications. RESULTS: Mean age at surgery was 53.7 yr and 52% were female. There were 49 schwannomas, 18 meningeomas, 10 ependymomas, 2 hemangioblastomas, 1 neurofibroma, 1 paraganglioma, 1 epidermoid cyst, and 1 hemangiopericytoma. The surgical mortality was 0%. In 87% of cases, gross total resection was achieved. The complication rate was 11%, including 2 cerebrospinal fluid leakages, 1 asymptomatic pseudomeningocele, 2 superficial surgical site infections, 1 sinus vein thrombosis, and 4 cases of neurological deterioration. There were no postoperative hematomas, and no cases of deep vein thrombosis or pulmonary embolism. Ninety-three percent of patients were ambulatory and able to work at the time of follow-up.

CONCLUSION: This study both demonstrates that it is feasible and safe to remove select, primary intradural spinal tumors using MIS, and augments the previous literature in favor of MIS for these tumors.

Posted on

Intramedullary ependymoma: long-term outcome after surgery

Acta Neurochirurgica (2018) 160:439–447

Overall (OS) and progression-free survival (PFS) of patients undergoing spinal ependymoma resection has been frequently reported. Contrarily, OS and PFS of purely intramedullary ependymomas have not been clearly determined yet.

Methods The data of 37 patients undergoing resection of an intramedullary ependymoma (IE) from January 2000 to December 2016 were analysed retrospectively.

Results The mean age was 46 years. The male:female ratio was 24:13. The median duration of symptoms was 12 months. Sixtytwo per cent of ependymomas were in the cervical, 24% in the thoracic, and 14% in the conus region in our series. The median volume was 1.3 ml. A syrinx was found in 49%and a cyst in 32%. GTR was achieved in 89%, STR in three (8%), and PR in one patient (3%). Median follow-up was 114 months. PFS was 87%, 82%, and 82%at 5, 10, and 15 years, respectively. OS was 97%, 88%, and 63% at 5, 10, and 15 years, respectively. There was a significant difference in PFS depending on the extent of resection and in OS depending on the pre-operative clinical status. There was no significant difference in OS and PFS regarding the other examined influencing factors.

Conclusion GTR resection was the most important factor influencing PFS. According to our results OS of IEs is much worse than that of spinal ependymomas. Our analysis confirms that patients with good pre-operative (McCormick grade 1 and 2) clinical status have significantly better OS than patients with McCormick grade 3 and higher.

Posted on

Supratentorial hemispheric ependymomas: an analysis of 109 adults for survival and prognostic factors

supratentorial hemispheric ependymomas

J Neurosurg 125:410–418, 2016

Survival rates and prognostic factors for supratentorial hemispheric ependymomas have not been determined. The authors therefore designed a retrospective study to determine progression-free survival (PFS), overall sur- vival (OS), and prognostic factors for hemispheric ependymomas.

Methods: The study population consisted of 8 patients from our institution and 101 patients from the literature with disaggregated survival information (n = 109). Patient age, sex, tumor side, tumor location, extent of resection (EOR), tumor grade, postoperative chemotherapy, radiation, time to recurrence, and survival were recorded. Kaplan-Meier survival analyses and Cox proportional hazard models were completed to determine survival rates and prognostic factors.

Results: Anaplastic histology/WHO Grade III tumors were identifed in 62% of cases and correlated with older age. Three-, 5-, and 10-year PFS rates were 57%, 51%, and 42%, respectively. Three-, 5-, and 10-year OS rates were 77%, 71%, and 58%, respectively. EOR and tumor grade were identifed on both Kaplan-Meier log-rank testing and univariate Cox proportional hazard models as prognostic for PFS and OS. Both EOR and tumor grade remained prognostic on multivariate analysis. Subtotal resection (STR) predicted a worse PFS (hazard ratio [HR] 4.764, p = 0.001) and OS (HR 4.216, p = 0.008). Subgroup survival analysis of patients with STR demonstrated a 5- and 10-year OS of 28% and 0%, respectively. WHO Grade III tumors also had worse PFS (HR 10.2, p = 0.004) and OS (HR 9.1, p = 0.035). Patients with WHO Grade III tumors demonstrated 5- and 10-year OS of 61% and 46%, respectively. Postoperative radiation was not prognostic for PFS or OS.

Conclusions: A high incidence of anaplastic histology was found in hemispheric ependymomas and was associated with older age. EOR and tumor grade were prognostic factors for PFS and OS on multivariate analysis. STR or WHO Grade III pathology, or both, predicted worse overall prognosis in patients with hemispheric ependymoma.

Posted on

Qualitative analysis of spinal intramedullary lesions using PET/CT

Qualitative analysis of spinal intramedullary lesions using PET-CT.1

J Neurosurg Spine 23:613–619, 2015

Although the usefulness of PET for brain lesions has been established, few reports have examined the use of PET for spinal intramedullary lesions. This study investigated the diagnostic utility of PET/CT for spinal intramedullary lesions.

Methods l-[methyl-11C]-methionine (MET)– or [18F]-fluorodeoxyglucose (FDG)–PET/CT was performed in 26 patients with spinal intramedullary lesions. The region of interest (ROI) within the spinal cord parenchyma was placed manually in the axial plane. Maximum pixel counts in the ROIs were normalized to the maximum standardized uptake value (SUVmax) using subject body weight. For FDG-PET the SUVmax was corrected for lean body mass (SULmax) to exclude any influence of the patient’s body shape. Each SUV was analyzed based on histopathological results after surgery. The diagnostic validity of the SUV was further compared with the tumor proliferation index using the MIB-1 monoclonal antibody (MIB-1 index).

Results A total of 16 patients underwent both FDG-PET and MET-PET, and the remaining 10 patients underwent either FDG-PET or MET-PET. Pathological diagnoses included high-grade malignancy such as glioblastoma multiforme, anaplastic astrocytoma, or anaplastic ependymoma in 5 patients; low-grade malignancy such as hemangioblastoma, diffuse astrocytoma, or ependymoma in 12 patients; and nonneoplastic lesion including cavernous malformation in 9 patients. Both FDG and MET accumulated significantly in high-grade malignancy, and the SULmax and SUVmax correlated with the tumor proliferation index. Therapeutic response after chemotherapy or radiation in high-grade malignancy was well monitored. However, a significant difference in SULmax and SUVmax for FDG-PET and MET-PET was not evident between low-grade malignancy and nonneoplastic lesions.

Conclusions Spinal PET/CT using FDG or MET for spinal intramedullary lesions appears useful and practical, particularly for tumors with high-grade malignancy. Differentiation of tumors with low-grade malignancy from nonneoplastic lesions may still prove difficult. Further technological refinement, including the selection of radiotracer or analysis evaluation methods, is needed.

Posted on

Predicting the “usefulness” of 5-ALA-derived tumor fluorescence for fluorescence-guided resections in pediatric brain tumors

5-ALA in pedidatric brain tumors

Acta Neurochir (2014) 156:2315–2324

Five-aminolevulinic acid (Gliolan, medac, Wedel, Germany, 5-ALA) is approved for fluorescence-guided resections of adult malignant gliomas. Case reports indicate that 5-ALA can be used for children, yet no prospective study has been conducted as of yet. As a basis for a study, we conducted a survey among certified European Gliolan users to collect data on their experiences with children.

Methods Information on patient characteristics, MRI characteristics of tumors, histology, fluorescence qualities, and outcomes were requested. Surgeons were further asked to indicate whether fluorescence was “useful”, i.e., leading to changes in surgical strategy or identification of residual tumor. Recursive partitioning analysis (RPA) was used for defining cohorts with high or low likelihoods for useful fluorescence.

Results Data on 78 patients <18 years of age were submitted by 20 centers. Fluorescence was found useful in 12 of 14 glioblastomas (85 %), four of five anaplastic astrocytomas (60 %), and eight of ten ependymomas grades II and III (80 %). Fluorescence was found inconsistently useful in PNETs (three of seven; 43 %), gangliogliomas (two of five; 40 %), medulloblastomas (two of eight, 25 %) and pilocytic astrocytomas (two of 13; 15 %). RPA of pre-operative factors showed tumors with supratentorial location, strong contrast enhancement and first operation to have a likelihood of useful fluorescence of 64.3 %, as opposed to infratentorial tumors with first surgery (23.1 %).

Conclusions Our survey demonstrates 5-ALA as being used in pediatric brain tumors. 5-ALA may be especially useful for contrast-enhancing supratentorial tumors. These data indicate controlled studies to be necessary and also provide a basis for planning such a study.

Posted on

Pathology of Spinal Ependymomas

Pathology of Spinal Ependymomas

Neurosurgery 73:247–255, 2013

Ependymomas constitute approximately 40% of primary intraspinal tumors. Current World Health Organization (WHO) grading may not correlate with observed progression-free survival (PFS).

OBJECTIVE: This retrospective study of prospectively collected data examines whether PFS is influenced by the histological grade or by the extent of resection. It also analyzes the usage and effectiveness of postoperative adjuvant radiotherapy.

METHODS: We reviewed 134 consecutive patients with ependymomas of all grades. Pathology slides were re-reviewed and the histological grades were confirmed by a single neuropathologist. Postoperative residual or recurrence was evaluated with follow-up magnetic resonance imaging.

RESULTS: There were 85 male and 49 female patients, ranging from 10 to 79 (median 41) years of age. Thirty patients had WHO grade I tumors, 101 had grade II tumors, and 3 had grade III tumors. Kaplan-Meier analysis of PFS demonstrated a mean duration of 6 years for grade I, 14.9 years for grade II, and 3.7 years for grade III (P , .001). In grade II ependymomas, mean PFS was 11.2 years with subtotal resection and 17.8 years with gross total resection (P, .01). PFS of patients who underwent subtotal resection was not significantly changed by adjuvant radiotherapy (P , .36).

CONCLUSION: Patients with grade II ependymoma have significantly longer PFS than patients with grade I ependymoma. The extent of resection did not affect PFS in grade I ependymoma but it did in grade II. Contrary to its higher grade, WHO grade II ependymoma carries a better prognosis than WHO grade I ependymoma.

Posted on

Long-term Outcome After Resection of Intraspinal Ependymomas: Report of 86 Consecutive Cases

Neurosurgery 67:1622–1631, 2010 DOI: 10.1227/NEU.0b013e3181f96d41

Objective: To evaluate progression-free survival, overall survival (OS) and long-term clinical outcome in a consecutive series of 86 patients with intraspinal ependymomas.

METHODS: Medical charts were retrospectively reviewed. Surviving patients voluntarily participated in a clinical history and physical examination that focused on neurological function and current tumor status.

RESULTS: Follow-up data are nearly 100% complete; mean follow-up time was 82 months. Eighty-five patients (99%) had surgery as a first-line treatment; 14 (17%) of these patients received adjuvant radiotherapy. Of the 85 patients who underwent primary surgery, gross total resection was performed in 60 patients (71%) and subtotal resection in 25 patients (29%). Ten-year progression-free survival rate was 75%; 5-year OS, 97%; and 10-year OS, 91%. Reduced preoperative neurological function and older age at diagnosis were significantly associated with increased risk of death. At follow-up, spontaneous regression of residual tumor after primary surgery may have occurred in 7 of 19 patients (37%). More than 75% of patients had neurological function compatible with an independent life at follow-up. Good preoperative neurological function was significantly associated with favorable outcome. It was not possible to evaluate the effect of radiotherapy on progression-free survival and OS.

CONCLUSION: Gross total resection remains the optimal treatment for patients with spinal ependymoma. Patients should be monitored with a clinical examination and magnetic resonance imaging at regular intervals up to 10 years after surgery.

Posted on

Primary Intramedullary Tumors of the Spinal Cord

SPINE Volume 34, Number 22S, pp S69 –S77, 2009

Study Design. Clinically based systematic review.

Objective. To define optimal clinical care for primary intramedullary spinal cord tumors using a systematic re- view with expert opinion.

Methods. Focused questions on the treatment of primary intramedullary spinal cord tumors were refined by a panel of spine oncology surgeons, medical and radiation oncologist. Keyword were searched through Medline database and pertinent abstracts and manuscripts obtained. The quality of literature was rated as high, moderate, low, or very low. Using the GRADE evidence based review system the proposed questions were answered using the literature review and expert opinion. These treatment recommendations were then rated as either strong or weak based on the quality of evidence and clinical expertise.

Results. The literature searches revealed low and very low quality evidence with no prospective or randomized studies. The MEDLINE search engine returned 9000 articles which was restricted to articles about human subjects and written in the English language. The subsequent search resulted in a return of: “spinal cord tumor” (5053), “ependymoma” (580), “astrocytoma” (420), and “glioma” (235) articles. Seventeen articles referenced timing of surgical intervention and symptomatology for intramedullary spinal cord tumors. One hundred fifty-eight chemotherapy and 183 radiation therapy articles for intramedullary spinal cord tumors were reviewed.

Conclusion. The most important factor in determining the IMSCT patient’s long-term neurologic and functional outcome after surgery is the patient’s preoperative neurologic status. However, this must be taken in the context of the underlying tumor histology. Therefore, resection is reserved for progressive neurologic decline and serial monitoring for asymptomatic individuals. Adjuvant therapy is an option for high grade astrocytomas (WHO grades 3–4).


Posted on

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.

Proudly powered by WordPress