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Myxopapillary ependymoma (MPE) is a unique slow-growing benign (WHO grade 1) subtype of spinal cord ependymoma arising predominantly in the filum terminale. Despite its benign nature, it occasionally disseminates through the cerebrospinal fluid and metastasizes to distant sites. Here, we report an extremely rare case of MPE with interval CSF seeding and metachronous metastasis in a 47 -year-old female presented as a gradually increasing low back pain for three months with bilateral radiculopathy down to the knees. Magnetic resonance imaging (MRI) showed an intradural extramedullary spinal mass of iso-intense signal to the cord on T1 weighted-images (WIs), heterogeneous, predominantly hyperintense signal on T2WIs with homogenous enhancement after contrast administration. L2 laminectomy with gross total resection (GTR) was performed, and histopathological results confirmed the diagnosis of MPE. Adjuvant radiotherapy was administered, followed by series of MRI scans. 28 months after GTR, Lumbar MRI showed multiple tiny enhancing nodules in the cauda equina. 44 months follow-up whole spine MRI revealed multiple intradural extramedullary nodules throughout the entire spine. The largest one measures about 1.5cm opposite to T3 -T4 intervertebral disc space. The patient underwent T3 and T4 laminectomy and GTR under general anesthesia using microsurgical techniques, and the histopathological result came with the diagnosis of MPE.

We report a novel group of clinically aggressive spinal cord ependymomas characterized by Grade III histology, MYCN amplification, an absence of NF2 alterations or other recurrent pathogenic mutations, and a unique methylation classifier profile. Seven cases were found to have MYCN amplification in the course of routine mutational profiling of 552 patients with central nervous system tumors between December 2016 and July of 2019 and an eighth patient was identified from an unrelated set of cases. Methylation array analysis revealed that none of the 8 cases clustered with any of the nine previously described ependymoma methylation subgroups, and 7 of 8 formed their own tight unique cluster. Histologically all cases showed grade III features, and all demonstrated aggressive clinical behavior. These findings are presented in the context of data from three other studies describing similar cases. Therefore, a combined total of 27 MYCN amplified spinal cord ependymoma cases have now been reported in the literature, warranting their consideration as a distinctive subtype of spinal cord ependymoma (SP-EPN-MYCN) with their unique molecular characteristics and aggressive clinical behavior.

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.

PURPOSE Pediatric Spinal cord ependymoma (SCE) is rare, and the management is often heterogeneous across centers. We evaluated the impact of clinical, pathologic, and treatment-related factors on outcomes in a multi-institutional, international cohort. METHODS SCE patients age RESULTS 125 patients were identified, 18 (14.4%) with metastases. Initial surgery was GTR, and STR in 44, 56% of patients respectively. Histology was grade 1, 2, and 3 in 55, 17.7 and 23.2% respectively. 55 patients with initial GTR were observed (52.7%) or irradiated (43.6%); 60 patients had STR and were observed (40%) or irradiated (60%). The 7-year event-free (EFS) and overall survival (OS) was 60% (95% CI 51.5–71.4) and 79% (95% CI 71.1–87.8) respectively. STR and metastasis increased the hazard for death [HR 1.87, 95% CI 1.02–3.57, p=0.05 (vs. GTR)] and [HR 2.28, 95% CI 1.1–5.2, p=0.048 (vs. localized)] respectively. Across 43 failures, local failure predominated (48.8%). Distant and combined failure occurred in 30.2 and 13.9% respectively. Adjuvant RT offered a 20% absolute improvement (vs. observation) in EFS at 5 years regardless of extent of resection. RPA identified thoracic (vs. non-thoracic), grade (1 & 3 vs. 2), STR (vs. GTR) and metastases as determinants of inferior EFS. CONCLUSIONS Tumor and treatment-related factors are predictive of EFS. OS is favorable despite diverse schema and frequent distant failures.

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.

Patients with neurofibromatosis 1 (NF1) are predisposed to develop central nervous system tumors, due to the loss of neurofibromin, an inactivator of proto-oncogene Ras. However, to our knowledge, only three cases of ependymomas with NF1 have been reported in the literature. The authors present a case of NF1 patient with a spinal cord ependymoma. She was referred for about half a year history of increasing numbness that progressed from her fingers to her entire body above the bellybutton. Magnetic resonance imaging revealed a relative-demarcated, heterogeneously enhanced mass lesion accompanied by perifocal edema in C5-7 level, a left-sided T11 spinous process heterogeneously enhanced mass in soft tissue, intervertebral disk hernia in L2-5 level, and widespread punctum enhancing lesion in her scalp and in T11-L5 level. The patient underwent C5-7 laminectomies and total excision of the tumor under operative microscope, and intraoperative ultrasonography and physiological monitoring were used during the surgery. Histopathologically, her tumor was found to be a ependymoma without malignant features (grade II in the World Health Organization classification). Therefore, no adjuvant therapy was applied. Following the operation, the patient showed an uneventful clinical recovery with no evidence of tumor recurrence after one year of follow-up.

Spinal ependymomas are rare tumours, with total resection favoured where possible. Several case series assessing the outcome following neurosurgical treatment for spinal ependymoma advocate the usage of adjuvant radiotherapy in cases of subtotal resection, or in unencapsulated tumours. We assessed the outcome of 61 consecutive cases of spinal ependymoma in a single centre over a 20year period using a variety of outcome measures. Sex distribution was equal, with a mean age at surgery of 43.6years (range 5-76years). Overall, most tumours occurred in the lumbosacral region (70.5%), with fewer in the thoracic (27.9%) and cervical regions (18.0%). Myxopapillary features were seen in 41.0% of tumours, and were more common when occurring in the lumbar region (51.2%). Gross total resection was achieved in 52.5%, subtotal resection in 37.7% and biopsy alone in 9.8% of patients and 31.1% received adjuvant radiotherapy. Two-thirds of patients achieved an excellent post-operative neurological outcome (Frankel grade E). Tumour recurrence was rare. Gross total resection and good preoperative neurological condition were most strongly predictive of good outcome. Post-operative radiotherapy did not seem to confer survival benefit in this case series, even in cases of incomplete resection, leading us to question its utility for all cases of spinal cord ependymoma.

Relatamos a associação impar entre esclerose múltipla (EM), tumor medular e tumor intracraniano em uma paciente de 63 anos de idade e com EM há dez anos com evolução em surtos de remissão e exacerbação. Havia melhora dos sintomas com o uso de corticosteróides. Em 1997 apresentou paraparesia crural e do membro superior direito, de instalação progressiva e que não respondeu à corticoterapia. A ressonância magnética da coluna cervical evidenciou tumor intramedular, que se revelou um ependimoma, e a do crânio, a presença de meningioma parietal à esquerda. Ressaltamos a associação incomum entre tumores do sistema nervoso central e EM e enfatizamos a necessidade de investigação clínica e por imagem diante de uma manifestação ou evolução clínica incomum no curso da doença.

Background. Ependymoma is the most common glial tumor of the adult spinal cord. Current consensus recommends surgical resection with gross total resection (GTR) whenever possible. We performed a comprehensive review of the literature to evaluate whether adjuvant radiotherapy after subtotal resection (STR) has any benefit. Methods. A PubMed search was performed to identify adult patients with spinal cord ependymoma who underwent surgical resection. Only patients who had clearly defined extent of resection with or without adjuvant radiotherapy were included for analysis. Kaplan-Meier and multivariate Cox regression survival analyses were performed to determine the effects of adjuvant radiotherapy on progression-free survival (PFS) and overall survival (OS). Results. A total of 348 patients underwent surgical resection of spinal cord ependymomas, where GTR was obtained in 77.0% (268/348) of patients. Among those who received STR, 58.8% (47/80) received adjuvant radiotherapy. PFS was significantly prolonged among those who received adjuvant radiotherapy after STR (log rank; P , .001). This prolonged PFS with adjuvant radiotherapy remained significant in multivariate Cox regression analysis (STR versus STR + RT group; hazard ratio (HR) ¼ 2.26, P ¼ .047). By contrast, improved OS was only associated with GTR (GTR versus STR + RT group; HR ¼ 0.07, P ¼ .001) and benign ependymomas (HR ¼ 0.16, P ¼ .001). Conclusions. Surgery remains the mainstay treatment for spinal cord ependymomas, where GTR provides optimal outcomes with longest PFS and OS. Adjuvant radiotherapy prolongs PFS after STR significantly, and OS is improved by GTR and benign tumor grade only.

Objective : To evaluate the clinical results of gross total resection in the surgical approach to spinal ependymoma. Methods : Between June 1995 and May 2009, 13 males and 8 females (mean age 34) diagnosed with intramedullary or extramedullary spinal ependymoma were surgically treated at our centre. The neurological and functional state of each patient were evaluated according to the modified McCormick scale. Results : The average follow-up duration was 54 months (ranging from 12 to 168 months). The locations of the lesions were : thoracic region (4, 19%), lumbar region (7, 34%), cervical region (4, 19%), cervicothoracic region (3, 14%) and conus medullaris (3, 14%). Four patients (19%) had deterioration of neurological function in the early postoperative period. The neurological function of three patients was completely recovered at the 6th postoperative month, while that of another patient was recovered at the 14th month. In the last assessment of neurological function, 20 patients (95%) were assessed as McCormick grade 1. No perioperative complications developed in any of our patients. In one patient’s 24-month assessment, tumour recurrence was observed. Re-operation was not performed and the patient was taken under observation. Conclusion : Two determinants of good clinical results after spinal ependymoma surgery are a gross total resection of the tumour and a good neurological condition before the operation. Although neurological deficits in the early postoperative period can develop as a result of gross total tumour resection, significant improvement is observed six months after the operation. 10.3340/jkns.2010.47.2.102

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 rd to 5 th 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.

Ependymomas account for 2-6% of all central nervous system neoplasms. They develop from the ependymal cells that line the ventricular cavities of the brain and the central canal of the spinal cord, as well as from ependymal clusters in the filum terminale. Giant cell ependymoma (GCE) is a rare subtype, with few cases reported, mostly in the brain. We describe the case of a cervical spinal cord ependymoma with pleomorphic giant cells and focal calcifications occurring in a 25-year-old woman. Magnetic resonance imaging revealed a large multicystic and partially enhancing intramedullary tumour extending from C2 to C5. Intraoperative analysis of frozen section tissue fragments suggested a malignant tumour; however, an obvious cleavage plane was present around most of the mass, and a macroscopically complete tumour removal could be achieved. Subsequently, paraffin sections and immunohistochemical investigations revealed unequivocal evidence of a GCE with focal calcifications. This case, the second giant-cell ependymoma to be described in the spinal cord and the first with focal calcifications, highlights the features of GCE and the discrepancy between the worrisome histological appearance, the surgical findings and the clinical relatively good prognosis.

The aim of this study was to determine predictors of functional outcome and survival in a retrospective cohort of spinal cord ependymomas treated at a single institution. Twenty-six patients who underwent treatment of spinal cord ependymoma at a single institution were retrospectively analysed. The clinicopathological features were reviewed and correlated with functional outcome (measured using the Frankel grade), recurrence (clinical or radiological), progression-free survival (PFS) and overall survival (OS). Seventy-nine percent of patients with complete excision had maintained or improved functional outcome, compared to 75% in the incomplete resection plus radiotherapy group. Patients with a good pre-operative Frankel grade tended to maintain their functional status, though this did not reach statistical significance (Fisher’s Exact test, P = 0.090). Univariate analysis revealed that longer symptom duration prior to treatment was associated with poorer functional outcome (P = 0.006). Extent of resection and the use of adjuvant radiotherapy did not influence PFS or OS; however, early diagnosis and treatment are paramount in the management of spinal ependymoma if a good functional outcome is to be achieved.