Management of The Posterior Cerebral Fossa Ependymomas

Background Posterior fossa brain ependymomas are one of the most devastating forms of human illnesses which are more common in children. Brainstem compression, herniation and death are the risks with tumours in this critical location. Patients and Methods: A retrospective study including 50 patients with posterior fossa ependymoma were performed at the Neurosurgery Department in Ait IDDIR University Hospital between the period of January 2005 and December 2015 . In each case, diagnosis was made clinically and confirmed radiologically and histo-pathologically.. All patients received the adjuvant treatment Results Out of 50 patients, 30 (60%) patients were males and 20 (40%) were females. The mean age was 24 years (ranged 5 months –47 years) ; we identified 29 (58%) children and 21 (42%) adults,. Ventriculo-peritoneal shunts were placed in all our patients, the total tumor excision was done to 35% and the partial tumor excision was done to 65% patients. The most common complications were as follows: Shunt malfunction : 4% , operative cavity hematoma 6%, CSF fistula 4%, deterioration of Cerebellar syndrome 6%, Cerebellar Mutism 2%, Mixed nerves palsy 4% and early post operative deaths 10%. Tumor architecture was classified as classic (Grade II) in 35 (70%) cases and anaplastic (Grade III) in 13(26%) cases. Adjuvant treatment regimens following resection included radiation therapy only (72%) for 36 patients including children above and adults and chemotherapy only (36%) for 9 children below 4 years and 9 recurrent tumors. During follow-up period, recurrence occurred in 27% 11patients out of 41patients .Five patients died (10%). Conclusion The surgical treatment of posterior fossa tumours still represents a challenge for neurosurgeons, Radical surgery with preservation of vital structures is the treatment of choice in patients with Posterior Cerebral Fossa ependymomas.Our experience shows the accepted results, complications and surgical outcome in relation to previous clinical studies.

Download Full-text

Adult outcome after treatment of pediatric posterior fossa ependymoma: long-term follow-up of a single consecutive institutional series of 22 patients with more than 5 years of survival

Journal of Neurosurgery Pediatrics ◽

10.3171/2020.1.peds19700 ◽

2020 ◽

Vol 26 (1) ◽

pp. 22-26 ◽

Cited By ~ 1

Author(s):

Tryggve Lundar ◽

Bernt Johan Due-Tønnessen ◽

Radek Frič ◽

Petter Brandal ◽

Paulina Due-Tønnessen

Keyword(s):

Proton Beam ◽

Posterior Fossa ◽

Adjuvant Treatment ◽

Tumor Control ◽

Full Time ◽

Time Work ◽

Long Term Follow Up ◽

Long Term Survivors

OBJECTIVEEpendymoma is the third most common posterior fossa tumor in children; however, there is a lack of long-term follow-up data on outcomes after surgical treatment of posterior fossa ependymoma (PFE) in pediatric patients. Therefore, the authors sought to investigate the long-term outcomes of children treated for PFE at their institution.METHODSThe authors performed a retrospective analysis of outcome data from children who underwent treatment for PFE and survived for at least 5 years.RESULTSThe authors identified 22 children (median age at the time of surgery 3 years, range 0–18 years) who underwent primary tumor resection of PFE during the period from 1945 to 2014 and who had at least 5 years of observed survival. None of these 22 patients were lost to follow-up, and they represent the long-term survivors (38%) from a total of 58 pediatric PFE patients treated. Nine (26%) of the 34 children treated during the pre-MRI era (1945–1986) were long-term survivors, while the observed 5-year survival rate in the children treated during the MRI era (1987–2014) was 13 (54%) of 24 patients. The majority of patients (n = 16) received adjuvant radiotherapy, and 4 of these received proton-beam irradiation. Six children had either no adjuvant treatment (n = 3) or only chemotherapy as adjuvant treatment (n = 3). Fourteen patients were alive at the time of this report. According to MRI findings, all of these patients were tumor free except 1 patient (age 78 years) with a known residual tumor after 65 years of event-free survival.Repeat resections for residual or recurrent tumor were performed in 9 patients, mostly for local residual disease with progressive clinical symptoms; 4 patients underwent only 1 repeated resection, whereas 5 patients each had 3 or more resections within 15 years after their initial surgery. At further follow-up, 5 of the patients who underwent a second surgery were found to be dead from the disease with or without undergoing additional resections, which were performed from 6 to 13 years after the second procedure. The other 4 patients, however, were tumor free on the latest follow-up MRI, performed from 6 to 27 years after the last resection. Hence, repeated surgery appears to increase the chance of tumor control in some patients, along with modern (proton-beam) radiotherapy. Six of 8 patients with more than 20 years of survival are in a good clinical condition, 5 of them in full-time work and 1 in part-time work.CONCLUSIONSPediatric PFE occurs mostly in young children, and there is marked risk for local recurrence among 5-year survivors even after gross-total resection and postoperative radiotherapy. Repeated resections are therefore an important part of treatment and may lead to persistent tumor control. Even though the majority of children with PFE die from their tumor disease, some patients survive for more than 50 years with excellent functional outcome and working capacity.

Download Full-text

NCOG-30. ADJUVANT VERSUS SALVAGE MANAGEMENT FOR IDH-MUTANT LOW GRADE GLIOMA

Neuro-Oncology ◽

10.1093/neuonc/noaa215.568 ◽

2020 ◽

Vol 22 (Supplement_2) ◽

pp. ii135-ii135

Author(s):

Martin Tom ◽

Deborah Park ◽

Surabhi Tewari ◽

Wei Wei ◽

Samuel Chao ◽

...

Keyword(s):

Adjuvant Treatment ◽

Salvage Treatment ◽

Low Grade Glioma ◽

Postoperative Treatment ◽

Management Approach ◽

Low Grade ◽

Free Survival ◽

Grade Ii ◽

Postoperative Observation

Abstract PURPOSE Timing of postoperative treatment in low-grade glioma (LGG) remains controversial. We sought to evaluate outcomes between adjuvant versus salvage management among patients with IDH-mutant molecularly-defined LGG. METHODS We analyzed a single-institutional database of adults diagnosed with LGG (grade II) with an IDH-mutation and either 1p19q-codeletion (oligodendroglioma) or 1p19q-intact (astrocytoma). Cox multivariable analysis (MVA) accounting for age, sex, and extent-of-resection, was used to compare adjuvant versus salvage approaches on overall survival (OS), progression free survival (PFS), next-intervention free survival (NIFS, defined as intervention subsequent to either adjuvant or salvage treatment), and malignant-transformation free survival (MTFS). Adjuvant treatment was defined as immediate postoperative treatment with radiotherapy (RT) and/or temozolomide (TMZ) prior to progression. Salvage management was defined as postoperative observation followed by surgery or RT and/or TMZ at progression. RESULTS Of 162 patients with oligodendroglioma, median follow-up was 8.5 years (range, 0.03-25.7). Adjuvant treatment was given to 97 (59.9%) patients, with 65 (40.1%) undergoing a salvage approach. On MVA, adjuvant treatment was not associated with OS, PFS, NIFS, or MTFS (p > 0.05 each). Among 82 patients with astrocytoma, median follow-up was 6.1 years (range, 0.5–25.7), and adjuvant treatment was administered to 41 (50.0%) patients, while 41 (50.0%) received salvage management. On MVA, adjuvant treatment was associated with improved PFS (HR 0.42, 95% CI 0.24-0.73, p < 0.001) and NIFS (HR 0.35, 95% CI 0.18–0.65, p < 0.001), but was not associated with OS or MTFS (p > 0.05 each). CONCLUSIONS Among grade II, IDH-mutant oligodendrogliomas, initial postoperative observation followed by salvage treatment at progression may be appropriate, as immediate adjuvant therapy was not associated improved outcomes. However, a more individualized postoperative management approach is required for grade II, IDH-mutant astrocytomas, as adjuvant treatment was associated with improved PFS and NIFS, but not OS. Further validation and prospective studies are required.

Download Full-text

Multicentric Registry Study on Epidemiological and Biological Disease Profile as Well as Clinical Outcome in Patients with Low-Grade Gliomas: The LoG-Glio Project

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0039-1693650 ◽

2019 ◽

Vol 81 (01) ◽

pp. 048-057 ◽

Cited By ~ 1

Author(s):

Andrej Pala ◽

Minou Nadji-Ohl ◽

Katharina Faust ◽

Stefan Rückriegel ◽

Constantin Roder ◽

...

Keyword(s):

Surgical Management ◽

Adjuvant Treatment ◽

Low Grade ◽

Inclusion Criteria ◽

Wild Type ◽

Who Grade ◽

Low Grade Gliomas ◽

Grade Ii ◽

Multicenter Registry

Abstract Background World Health Organization (WHO) grade II low-grade gliomas (LGGs) in adults are rare, and patients' mean overall survival (OS) is relatively long. Epidemiological data on factors influencing tumor genesis and progression are scarce, and prospective data on surgical management are still lacking. Because of the molecular heterogeneity of LGG, a comprehensive molecular characterization is required for any clinical and epidemiological research. Further, a detailed radiologic assessment is needed as the only established objective criterion for progressive disease. Both radiologic and molecular assessments have to be standardized to produce comparable data. The aim of the registry is to improve the evidence for surgical management of LGG patients by establishing a multicenter registry with a strong surgical and clinical focus including mandatory biobanking. Methods The LoG-Glio project is a prospective national observational multicenter registry that began on November 1, 2015. Inclusion criteria encompass all patients > 18 years of age with a radiologic suspicion of LGG. Patients with severe neurologic or psychiatric disorders that may interfere with their informed consent or if there is no possibility for further follow-up are excluded. Diagnosis of glioblastoma WHO grade IV isocitrate dehydrogenase (IDH) wild type leads to a secondary exclusion of patients. In addition to demographic data, results of the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire, add-on for patients with brain tumors, and National Health Institute Stroke Scale before and after surgery and during regular follow-ups are collected. At each time point a detailed recording of surgical and adjuvant treatment is performed. Radiologic assessment involves three-dimensional (3D) acquisition of T1, fluid-attenuated inversion recovery, and T2 sequences. For the final evaluation, a central detailed neuropathologic and molecular assessment of tumor samples and a radiologic evaluation of imaging sets are part of the study protocol. Results We report the first 100 consecutively registered patients for LoG-Glio. Three patients dropped out due to loss of follow-up. Of the remaining recruited patients, 8 were classified as wait and scan; 89 had surgery. Using the inclusion criteria described previously, 70 patients had an IDH-mutated glioma, 10 had miscellaneous rare LGGs, and 8 patients had an IDH wild-type WHO grade II or III glioma. Conclusion The LoG-Glio registry has been successfully implemented. Applied selection criteria result in an appropriately balanced patient cohort. Short-term outcome data on epidemiology as well as the influence of current surgical techniques and adjuvant treatment on patient outcomes are expected. In the long run, the aim of the registry is to validate the new molecular-based WHO classification and the influence of the extent of resection on progression-free survival and OS. The registry provides an open platform for future research projects benefiting patients with LGG.

Download Full-text

EPEN-38. EZH2 INHIBITORY PROTEIN (EZHIP/Cxorf67) EXPRESSION IS HIGHLY CONCORDANT WITH H3K27me3 LOSS AND IS A PROMISING SURROGATE MARKER FOR POSTERIOR FOSSA TYPE A EPENDYMOMAS

Neuro-Oncology ◽

10.1093/neuonc/noaa222.173 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii315-iii315

Author(s):

Aruna Nambirajan ◽

Madhu Rajeshwari ◽

Meher Boorgula ◽

Ramesh Doddamani ◽

Manmohan Singh ◽

...

Keyword(s):

Posterior Fossa ◽

In Situ Hybridisation ◽

Surrogate Marker ◽

Fluorescence In Situ Hybridisation ◽

Who Grade ◽

Tenascin C ◽

Grade Ii ◽

1Q Gain

Abstract BACKGROUND Gene expression and DNA methylation have identified 2 distinct clinicopathological subgroups among the WHO Grade II/III posterior fossa (PF) ependymomas (EPN), of which the PF-A molecular subgroup associates with poor outcome. OBJECTIVE To analyse the utility of immunohistochemistry for H3K27me3, Tenascin C, EZHIP (Cxorf67), EZH2 and fluorescence-in-situ-hybridisation for chromosome 1q21 locus gain in the prognostic stratification of PF-EPNs. METHODS All PF Grade II/III tumors were retrieved (2009–2019). Immunohistochemistry for H3K27me3, H3K27M-mutation-specific antibody, EZH2, EZHIP, Tenascin-C and fluorescence in-situ hybridisation for 1q21 locus was performed and compared with outcome. RESULTS 71 PF-EPNs were included. H3K27me3 loss (PF-A) was seen in 65% (46/71) of cases, of which majority were positive for EZHIP (73%, 24/33) and Tenascin C (65%, 28/43). Minority showed chromosome 1q gain (19%, 8/42). An EZHIP negative PF-A tumor was immunopositive for H3K27M-mutant staining, while all others were negative. PF-A EPNs occurred at a median age of 4.5 years (range 1–53), were predominantly grade III (Grade III:II – 1.6:1), and 50% (10/20) of patients on follow-up experienced tumor progression. EPNs with retained H3K27me3 (PF-B) did not show EZHIP expression (0/20) or 1q gain; however, tenascin C expression was seen in 47% (8/25) of them. They occurred predominantly in adults, showed Grade II preponderance and only 2/11 patients on follow-up experienced progression. EZH2 expression did not correlate with H3K27me3 loss but positively correlated with EZHIP expression (p=0.015). CONCLUSION H3K27me3 is a reliable surrogate for prognostic classification of PF-EPNs. EZHIP expression is highly concordant with H3K27me3 loss and is a valuable adjunct.

Download Full-text

Dispersed Bone Spicules as a Cause of Postoperative Headache after Retrosigmoid Vestibular Schwannoma Surgery: A Myth?

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0041-1741112 ◽

2021 ◽

Author(s):

Yin Ren ◽

Marin A. McDonald ◽

Paul Manning ◽

Bridget V. MacDonald ◽

Marc S. Schwartz ◽

...

Keyword(s):

Posterior Fossa ◽

Vestibular Schwannoma ◽

Case Series ◽

Tumor Diameter ◽

Retrospective Case ◽

Meningeal Irritation ◽

Brainstem Compression ◽

Postoperative Headache ◽

Petrous Temporal Bone

Abstract Objectives Dispersion of bone dust in the posterior fossa during retrosigmoid craniectomy for vestibular schwannoma (VS) resection could be a source of meningeal irritation and lead to development of persistent postoperative headaches (POH). We aim to determine risk factors, including whether the presence of bone spicules that influence POH after retrosigmoid VS resection. Design Present study is a retrospective case series. Setting The study was conducted at a tertiary skull-base referral center. Participants Adult patients undergoing VS resection via a retrosigmoid approach between November 2017 and February 2020 were included for this study. Main Outcome Measures Development of POH lasting ≥ 3 months is the primary outcome of this study. Results Of 64 patients undergoing surgery, 49 had complete data (mean age, 49 years; 53% female). Mean follow-up time was 2.4 years. At latest follow up, 16 (33%) had no headaches, 14 (29%) experienced headaches lasting <3 months, 19 (39%) reported POH lasting ≥3 months. Twenty-seven (55%) patients had posterior fossa bone spicules detectable on postoperative computed tomography (CT). Age, gender, body mass index, length of stay, tumor diameter, size of craniectomy, the presence of bone spicules, or the amount of posterior petrous temporal bone removed from drilling did not differ significantly between patients with POH and those without. On multivariate logistic regression, patients with POH were less likely to have preoperative brainstem compression by the tumor (odds ratio [OR] = 0.21, p = 0.028) and more likely to have higher opioid requirements during hospitalization (OR = 1.023, p = 0.045). Conclusion The presence of bone spicules in the posterior fossa on postoperative CT did not contribute to headaches following retrosigmoid craniectomy approach for VS resection.

Download Full-text

Traumatic posterior fossa extradural hematoma

Romanian Neurosurgery ◽

10.33962/roneuro-2019-030 ◽

2019 ◽

pp. 160-165

Author(s):

Vibhu Shankar Parashar ◽

Vivek Kumar Kankane ◽

Gaurav Jaiswal ◽

Tarun Kumar Gupta

Keyword(s):

Early Detection ◽

Posterior Fossa ◽

Obstructive Hydrocephalus ◽

Postoperative Outcome ◽

Extradural Hematoma ◽

Tonsillar Herniation ◽

Medical College ◽

Brainstem Compression ◽

Noncontrast Ct

Background. Extradural hematoma of posterior fossa (PFEDH) is less common and there are not many articles about PFEDH. These patients can deteriorate very rapidly due to compression over brainstem. Thus, early identification and immediate intervention can save the lives of these patients.Objective. This study aims to conduct a comprehensive analysis of patients with PFEDH and evaluate the postoperative outcome which may be of help to make further preventive strategies.Methods and Materials. The study included 16 patients admitted with traumatic PFEDH from July 2016 to July 2018 at R.N.T. Medical College & M.B. Groups of Hospital Udaipur, southwestern Rajasthan, India. We have retrospectively reviewed the data. Analysed factors were gender, age, Glasgow Coma Scale (GCS), Noncontrast CT scan findings, associated brain injury, type of intervention, Glasgow Outcome Scale (GOS). GOS was assessed at discharge, at 3 months and 6 months follow-up.Results. Out of a total of 16 patients, 11 were male and 5 were female with age ranging from 05-46 years. 12 patients had GCS 13 -15 at admission and only one of them had GCS < 8. 15 patients underwent surgical intervention. At 6 months follow-up, 12 patients had good recovery GOS is 5.Conclusion. Early detection and immediate evacuation of PFEDHs should be done if causing fourth ventricle, basal cistern or brain stem compression. It may be rapidly fatal due to the expansion of hematoma leading to brainstem compression, tonsillar herniation, and/or obstructive hydrocephalus. Early detection and immediate evacuation lead to a better outcome in these patients.

Download Full-text