Papilledema and Spinal Ependymoma: When Eyes are Windows to the Spinal Cord

Object. The authors evaluated an alternative method to avoid postoperative posterior tethering of the spinal cord following resection of spinal ependymomas. Methods. Twenty-five patients with spinal ependymoma underwent surgery between 1978 and 2002. There were 16 male and nine female patients whose ages at the time of surgery ranged from 14 to 64 years (mean 41.8 years). The follow-up period ranged from 6 to 279 months (mean 112.4 months). In the initial 17 patients (Group A), the procedure to prevent arachnoidal adhesion consisted of the layer-to-layer closure of three meninges and laminoplasty. In the subsequently treated eight patients (Group B), the authors performed an alternative technique that included pial suturing, dural closure with Gore-Tex membrane—assisted patch grafting, and expansive laminoplasty. In Group A, postoperative adhesion was radiologically detected in eight cases (47%), and delayed neurological deterioration secondary to posterior tethering of the cord was found in five cases. In Group B, there was no evidence of adhesive posterior tethering or delayed neurological deterioration. A significant intergroup statistical difference was demonstrated for radiologically documented posterior tethering (p < 0.05, Fisher exact test). Moreover, patients with radiologically demonstrated posterior tethering suffered a significant delayed neurological functional deterioration (p < 0.01, Fisher exact test). Conclusions. This new technique for closure of the surgical wound is effective in preventing of postoperative posterior spinal cord tethering after excision of spinal ependymoma.

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EPCO-01. MOLECULAR PROFILING OF SPINAL CORD EPENDYMOMA

Neuro-Oncology ◽

10.1093/neuonc/noab196.000 ◽

2021 ◽

Vol 23 (Supplement_6) ◽

pp. vi1-vi1

Author(s):

Erika Yamazawa ◽

Shota Tanaka ◽

Genta Nagae ◽

Takayoshi Umeda ◽

Taijun Hana ◽

...

Keyword(s):

Spinal Cord ◽

Dna Methylation ◽

Methylation Status ◽

Neurofibromatosis Type ◽

Methylation Pattern ◽

Who Grade ◽

Spinal Ependymoma ◽

Fresh Frozen ◽

Spinal Cord Ependymoma ◽

The Difference

Abstract BACKGROUND Ependymomas are currently classified into 9 subgroups by DNA methylation profiles. Although spinal cord ependymoma (SP-EPN) is distinct from other tumors, diversity within SP-EPN is still unclear. Here, we used transcriptomic and epigenomic profiles to investigate the diversity among Japanese SP-EPN cases. MATERIALS AND METHODS We analyzed 57 SP-EPN patients (32 males and 25 females, aged from 18 to 78 years, median: 52), including two cases of neurofibromatosis type 2, five cases of grade 3 (WHO grade). We obtained transcriptome (RNA-seq) and DNA methylation (Infinium Methylation EPIC array) data from fresh frozen specimens of SP-EPN resected at the University of Tokyo Hospital and our collaborative groups. RESULTS Three cases had a previous intracranial ependymoma operation. Hierarchical clustering of the DNA methylation data showed that these three cases of intracranial origin as a different cluster from spinal origin. The 45 grade 2 spinal ependymoma showed a relatively homogenous methylation pattern. However, the methylation status of HOX gene cluster regions is compatible with the segment of origin, which reflects the cells of origins are derived after the determination of segment identity. RNA sequencing of 57 cases revealed two subgroups within grade 2. Gene ontology analysis of differentially expressed genes suggested the difference in metabolic state such as rRNA translation and mitochondrial respiration between the two expression subgroups. CONCLUSION Epigenetic analysis indicated the accurate body segment origin of SP-EPN. We observed that metabolic states could divide grade 2 spinal cord ependymoma into 2 subgroups and will present the relationship to clinicopathological information.

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Cytologic and histologic features of spinal cord ependymoma in a young dog: a case report

Veterinární Medicína ◽

10.17221/86/2009-vetmed ◽

2010 ◽

Vol 55 (No. 1) ◽

pp. 35-38 ◽

Cited By ~ 2

Author(s):

A. Sfacteria ◽

F. Macrì ◽

L. Perillo ◽

G. Rapisarda ◽

G. Lanteri ◽

...

Keyword(s):

Spinal Cord ◽

Case Report ◽

Contrast Medium ◽

Histological Examination ◽

Neoplastic Cells ◽

Spinal Ependymoma ◽

Eosinophilic Cytoplasm ◽

Cytoplasmic Processes ◽

Positive Results ◽

Fibrillar Network

A case of intramedullary ependymoma in a young dog is reported. A two year old dog was presented with paralysis of the forelimbs. At myelographic examination, an intramedullary pattern, blocking the progression of contrast medium, was observed. At necropsy, a 3 × 2 cm white-greyish mass was found extending from the 3<sup>rd</sup> to 5<sup>th</sup> lumbar levels. At cytological and histological examination, the mass was highly cellular and was comprised of ovoid cells with indistinct borders, elongated eosinophilic cytoplasm and round to oval vesicular nuclei. Cytoplasmic processes formed a fibrillar network wherein true rosettes and many pseudorosettes around a fibrovascular stroma were observed. Immunohistochemistry for vimentin and GFAP gave strong positive results in the neoplastic cells, especially around pseudorosettes and confirmed the diagnosis of intramedullary spinal ependymoma.

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The response of spinal cord ependymomas to bevacizumab in patients with neurofibromatosis Type 2

Journal of Neurosurgery Spine ◽

10.3171/2016.8.spine16589 ◽

2017 ◽

Vol 26 (4) ◽

pp. 474-482 ◽

Cited By ~ 10

Author(s):

Katrina A. Morris ◽

Shazia K. Afridi ◽

D. Gareth Evans ◽

Anke E. Hensiek ◽

Martin G. McCabe ◽

...

Keyword(s):

Spinal Cord ◽

Neurofibromatosis Type ◽

Neurofibromatosis Type 2 ◽

Response To Treatment ◽

Spinal Ependymoma ◽

Bevacizumab Treatment ◽

Radiological Changes ◽

And Behavior ◽

Serial Images

OBJECTIVE People with neurofibromatosis Type 2 (NF2) have a genetic predisposition to nervous system tumors. NF2-associated schwannomas stabilize or decrease in size in over half of the patients while they are receiving bevacizumab. NF2 patients treated with bevacizumab for rapidly growing schwannoma were retrospectively reviewed with regard to ependymoma prevalence and response to treatment. METHODS The records of 95 NF2 patients receiving bevacizumab were retrospectively reviewed with regard to spinal ependymoma prevalence and behavior. The maximum longitudinal extent (MLE) of the ependymoma and associated intratumoral or juxtatumoral cysts were measured on serial images. Neurological changes and patient function were reviewed and correlated with radiological changes. RESULTS Forty-one of 95 patients were found to have ependymomas (median age 26 years; range 11–53 years). Thirty-two patients with a total of 71 ependymomas had scans appropriate for serial assessment with a mean follow-up of 24 months (range 3–57 months). Ependymomas without cystic components showed minimal change in MLE. Twelve patients had ependymomas with cystic components or syringes. In these patients, reductions in MLE were observed, particularly due to decreases in the cystic components of the ependymoma. Clinical improvement was seen in 7 patients, who all had cystic ependymomas. CONCLUSIONS Bevacizumab treatment in NF2 patients with spinal cord ependymomas results in a decrease in the size of intratumoral and juxtatumoral cysts as well as adjacent-cord syringes and a decrease in cord edema. This may provide clinical benefit in some patients, although the changes do not meet the current criteria for radiological tumor response.

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Treatment of pediatric Grade II spinal ependymomas: a population-based study

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.9.peds1473 ◽

2015 ◽

Vol 15 (3) ◽

pp. 243-249 ◽

Cited By ~ 16

Author(s):

Yimo Lin ◽

Andrew Jea ◽

Stephanie C. Melkonian ◽

Sandi Lam

Keyword(s):

Spinal Cord ◽

Overall Survival ◽

Radiation Therapy ◽

Pediatric Patients ◽

Treatment Patterns ◽

Gross Total Resection ◽

Subtotal Resection ◽

Total Resection ◽

Spinal Ependymoma ◽

Grade Ii

OBJECT Grade II spinal cord ependymomas occurring in pediatric patients are exceptionally rare neoplasms. In this paper the authors use a national cancer database to determine patient demographics, treatment patterns, and associated outcomes of this cohort. METHODS The Surveillance Epidemiology and End Results (SEER) database was used to analyze subjects younger than 18 years with histologically confirmed diagnoses of Grade II spinal cord ependymoma from the years 1973 to 2008. Descriptive data on the demographic characteristics of this cohort and the associated treatment patterns are shown. The Kaplan-Meier method was used to estimate overall survival at 1, 2, 5, and 10 years. RESULTS This cohort comprised 64 pediatric subjects with Grade II spinal ependymoma. The median age was 13 years, nearly half of the patients were male, and most were white (84%). The median follow-up was 9.2 years. Overall survival at 5 and 10 years was 86% and 83%, respectively. Gross-total resection was achieved in 57% of subjects, and radiation therapy was administered to 36%. Radiation therapy was administered to 78% of subjects after subtotal resection but only to 19% of patients after gross-total resection; this difference was significant (p < 0.001). In a multivariate regression model analyzing sex, age at diagnosis, year of diagnosis, radiotherapy, and extent of resection, female sex was found to be an independent predictor of decreased mortality (HR 0.15 [95% CI 0.02–0.94], p = 0.04). CONCLUSIONS These data show long-term outcomes for pediatric patients with Grade II spinal ependymoma. Radiotherapy was more likely to be administered in cases of subtotal resection than in cases of gross-total resection. Female sex is associated with decreased mortality, while other demographic or treatment modalities are not.

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PATH-20. INVESTIGATION OF CLINICALLY AGGRESSIVE SPINAL CORD EPENDYMOMA THROUGH METHYLATION ANALYSIS

Neuro-Oncology ◽

10.1093/neuonc/noz175.616 ◽

2019 ◽

Vol 21 (Supplement_6) ◽

pp. vi147-vi147

Author(s):

Nicole Briceno ◽

Zied Abdullaev ◽

Elizabeth Vera ◽

Francine Blumental De Abreu ◽

Martha Quezado ◽

...

Keyword(s):

Spinal Cord ◽

Progression Free Survival ◽

Research Network ◽

Mycn Amplification ◽

Low Grade ◽

Methylation Analysis ◽

Clinical Criteria ◽

Spinal Ependymoma ◽

The Poor ◽

High Level

Abstract Spinal ependymoma is a rare, often low-grade tumor, for which the clinical course and prognosis has been poorly defined. Classification has relied on histological criteria, as there are few defining molecular mutations, causing controversy in diagnosis and grading. DNA methylation analysis with the brain tumor classifier was used on tumor tissue from 37 patients identified from the Neuro-Oncology Branch Natural History Study and selected Collaborative Ependymoma Research Network (CERN) Tissue Repository. These were histologically diagnosed with non-myxopapillary spinal ependymoma (92% grade II and 8% grade III), with 12 designated “poor performers” (50% male, median age 30 years) defined by a progression free survival of less than two years (median 7 months) with at least one recurrence. Alternatively, the 25 “good performers” (56% male, median age 43 years) had no progression with a follow-up time greater than 5 years (median 84 months). Methylation classification matched to “spinal ependymoma” in 33% of the poor performer cases while 8% matched myxopapillary or RELA-fusion ependymoma and 50% were no-match. For the good performers, 52% agreed with histological diagnosis, 28% matched myxopapillary and 20% were no-match. Interestingly, we noted high-level MYCN amplification by copy number analysis in two (17%) cases, neither of which matched to a defined methylation class, and both of which were in the poor performers group. Overall, methylation results reveal complex biology in conventional spinal cord ependymomas, most evident in an increase of no-match cases in the poor performers group. Of importance, the no-match cases contain evidence of a potentially new diagnostic entity characterized by both a poor prognosis and MYCN amplification. For further elucidation of this entity, a larger cohort of patients with expanded clinical criteria has been identified for further testing.

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Spinal Cord Ependymoma – Surgical Management and Outcome

Journal of Neurosciences in Rural Practice ◽

10.4103/jnrp.jnrp_267_18 ◽

2019 ◽

Vol 10 (02) ◽

pp. 316-320 ◽

Cited By ~ 1

Author(s):

Wail Mohammed ◽

Michael Farrell ◽

Ciaran Bolger

Keyword(s):

Spinal Cord ◽

Extent Of Resection ◽

Subtotal Resection ◽

Spinal Level ◽

Total Resection ◽

Spinal Ependymoma ◽

Slow Progression ◽

Primary Neoplasm ◽

Surgical Difficulty

ABSTRACT Background: Ependymoma is a common primary neoplasm of the spinal cord and filum terminale. Patients with spinal ependymoma usually experience gradual symptoms due to slow progression of the tumor; thus, early diagnosis can be challenging to make. Objective: The objective of this study was to report 5 years’ experience in treating spinal intramedullary ependymomas and to illustrate the advantage of aggressive complete resection whenever possible. Patients and Methods: Retrospective medical notes of all patients with spinal ependymoma treated surgically over a 5-year period between January 2003 and January 2008 were recorded. Clinical presentation, spinal level, extent of resection, and complications were recorded. A prolonged follow-up was documented. Results: There were 20 patients – 11 males, and nine females –included in this study. Their median age was 48 years (range 3–75 years). In 18 patients, total gross resection was achieved. Subtotal resection was only possible in one patient due to surgical difficulty. One patient underwent biopsy and referred for further surgery and subsequently had total resection. Conclusions: Radical total resection is achievable in spinal ependymomas, with minimal resultant morbidity.

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Complications associated with the treatment for spinal ependymomas

Neurosurgical FOCUS ◽

10.3171/2011.7.focus11158 ◽

2011 ◽

Vol 31 (4) ◽

pp. E13 ◽

Cited By ~ 35

Author(s):

Daniel T. Nagasawa ◽

Zachary A. Smith ◽

Nicole Cremer ◽

Christina Fong ◽

Daniel C. Lu ◽

...

Keyword(s):

Spinal Cord ◽

Pediatric Population ◽

Dorsal Column ◽

Neurological Deficits ◽

Sensory Loss ◽

Wound Complications ◽

Spinal Instability ◽

Spinal Ependymoma ◽

Technological Advances ◽

Increased Risk

Spinal cord ependymomas are rare neoplasms, comprising approximately 5% of all CNS tumors and 15% of all spinal cord tumors. Although surgery was once reserved for diagnosis alone, the evolution of surgical practices has elevated resection to the treatment of choice for these lesions. While technological advances continue to improve the capacity for gross-total resections and thus decrease the risk of recurrence, ependymoma spinal surgery still contains a variety of potential complications. The presence of neurological deficits and deterioration are not uncommonly associated with spinal cord ependymoma surgery, including sensory loss, dorsal column dysfunction, dysesthetic syndrome, and bowel and bladder dysfunction, particularly in the immediate postoperative period. Surgical treatment may also lead to wound complications and CSF leaks, with increased risk when radiotherapy has been involved. Radiation therapy may also predispose patients to radiation myelopathy and ultimately result in neurological damage. Additionally, resections of spinal ependymomas have been associated with postoperative spinal instability and deformities, particularly in the pediatric population. Despite the advances in microsurgical techniques and intraoperative cord monitoring modalities, there remain a number of serious complications related to the treatment of spinal ependymoma tumors. Identification and acknowledgment of these potential problems may assist in their prevention, early detection, and increased quality of life for patients afflicted with this disease.

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PATH-33. EPIGENOMIC ANALYSIS OF SPINAL EPENDYMOMA

Neuro-Oncology ◽

10.1093/neuonc/noaa215.714 ◽

2020 ◽

Vol 22 (Supplement_2) ◽

pp. ii171-ii171

Author(s):

Erika Yamazawa ◽

Shota Tanaka ◽

Nagae Genta ◽

Meguro Hiroko ◽

Takayoshi Umeda ◽

...

Keyword(s):

Spinal Cord ◽

Dna Methylation ◽

Profile Analysis ◽

Neurofibromatosis Type ◽

Methylation Data ◽

Who Grade ◽

Spinal Ependymoma ◽

Dna Methylation Profile ◽

Spinal Cord Ependymoma ◽

The University

Abstract BACKGROUND Ependymomas commonly occur in the fourth ventricle and the spinal cord. Gross total resection, age and WHO grade are known prognostic factors. Ependymomas are currently classified into 9 distinct subgroups by DNA methylation profile analysis. Spinal cord ependymoma is distinct from other subgroups. To investigate heterogeneity within spinal cord ependymoma, we examined DNA methylation profiles. MATERIALS AND METHODS We used Infinium MethylationEPIC array (illumina) to obtain DNA methylation data from frozen specimens of spinal ependymoma resected at the University of Tokyo, Osaka City University, and Tokyo Metropolitan Neurological Hospital. Japan Pediatric Molecular Neuro-Oncology Group provided methylation data for 11 reported cases. Cluster analysis was performed using Cluster3.0. RESULTS We analyzed 34 patients, 21 male and 13 female, aged from 18 to 76 years (median 50.5 years), including 2 cases with neurofibromatosis type 2. WHO grade was grade_3 in 2 cases and grade_2 in others. Clustering of the DNA methylation data showed that WHO grade_3 cases tended to be classified into a subgroup distinct from other cases. CONCLUSION This is the largest DNA methylation profiling study on spinal cord ependymoma to date. The study may suggest a new subgroup correlated with higher WHO grade.

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Prognosis by tumor location in adults with spinal ependymomas

Journal of Neurosurgery Spine ◽

10.3171/2012.12.spine12591 ◽

2013 ◽

Vol 18 (3) ◽

pp. 226-235 ◽

Cited By ~ 34

Author(s):

Michael C. Oh ◽

Joseph M. Kim ◽

Gurvinder Kaur ◽

Michael Safaee ◽

Matthew Z. Sun ◽

...

Keyword(s):

Spinal Cord ◽

Cox Regression ◽

Statistical Significance ◽

Cauda Equina ◽

Tumor Location ◽

Spinal Cord Tumors ◽

Prognostic Information ◽

Who Grade ◽

Spinal Ependymoma ◽

Spinal Axis

Object Ependymomas are primary central nervous system tumors that occur more frequently in the spines of adults than they do there in children. Previous studies consist mainly of retrospective single-institutional experiences or case studies. In this study, a comprehensive literature review was performed on reported cases of spinal ependymoma treated with resection to determine whether tumor location along the spinal axis conveys important prognostic information. Methods A PubMed search was performed to identify all papers that included data on patients with spinal ependymoma. Only cases involving adult patients who underwent ependymoma resection with a clearly reported tumor location were included for analysis. Tumor locations were separated into 6 groups: cervicomedullary, cervical, cervicothoracic, thoracic, thoracolumbar, and conus + cauda equina. Kaplan-Meier survival and Cox regression analyses were performed to determine the effect of tumor location on progression-free survival (PFS) and overall survival (OS). Results A total of 447 patients who underwent resection of spinal ependymomas with clearly indicated location of tumor were identified. The most common locations of spinal ependymomas were the cervical (32.0%) and conus + cauda equina (26.8%) regions. The thoracolumbar and cervicomedullary regions had the fewest tumors (accounting for, respectively, 5.1% and 3.4% of the total number of cases). The conus + cauda equina and thoracolumbar regions had the highest percentage of WHO Grade I tumors, while tumors located above these regions consisted of mostly WHO Grade II tumors. Despite the tendency for benign grades in the lower spinal regions, PFS for patients with spinal ependymomas in the lower 3 regions (thoracic, thoracolumbar, conus + cauda equina) was significantly shorter (p < 0.001) than for those with tumors in the upper regions (cervicomedullary, cervical, cervicothoracic), but the difference in OS did not achieve statistical significance (p = 0.131). Conclusions Spinal ependymomas along different regions of spinal axis have different characteristics and clinical behaviors. Tumor grade, extent of resection, and PFS varied by tumor location (upper vs lower spinal regions), while OS did not. Recurrence rates were higher for the lower spinal cord tumors, despite a greater prevalence of lower WHO grade lesions, compared with upper spinal cord tumors, suggesting that tumor location along the spinal axis is an important prognostic factor.

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