Spinal Myxopapillary Ependymoma Research Articles

Pretemporal transcavernous transtentorial approach for left pontine glioma.

A 39-year-old man, who had a history of spinal myxopapillary ependymoma with cerebrospinal seeding status post twice operations and radiation therapy, presented with aggravating headaches, diplopia, dysphagia, and unsteady gait for 2 weeks. The brain MRI revealed a parenchymal lesion at the left aspect of the pons, about 2.8 × 2.3 × 3.2 cm3. The patient underwent a pretemporal transcavernous transtentorial approach for tumor removal. The pathological report showed an anaplastic astrocytoma. In this approach, a wider surgical corridor was obtained by opening the Meckel’s cave and cutting the tentorium, via which a safe entry point into the pons could be determined with neuromonitoring. In the authors’ opinion, this approach is safe and effective in selected ventrolateral pontine gliomas.The video can be found here: https://youtu.be/sUt-9QFGgCI.

Clinical characteristics and surgical outcomes of spinal myxopapillary ependymomas.

Spinal myxopapillary ependymoma (SME) is a rare pathological variant of ependymoma, which most commonly occurs in the cauda equina and filum terminale of the spinal cord. Although SME is considered as a benign entity, histologically corresponding to WHO grade I, local recurrence and metastasis have been reported in many cases. The purpose of this large-scale, single-center study was to investigate the clinical characteristics and surgical outcomes of SME. A total of 34 consecutive patients diagnosed with SME were enrolled in this retrospective study. All patients underwent magnetic resonance imaging (MRI) and were treated with surgical resection. Individual clinical data were collected, and surgical outcomes were evaluated during the follow-up period. There were 21 males and 13 females, with an average age of 29.97years. Clinical symptoms included back pain (82.4%), weakness (44.1%) and numbness (20.6%) in extremities, and sphincter dysfunction (26.5%). The tumor locations included lumbar segments (52.9%), thoracolumbar segments (23.5%), lumbosacral segments (17.6%), and thoraco-lumbo-sacral segments (5.9%). On MRI, all SMEs appeared hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging, with remarkable enhancement. Gross total resection was achieved in 18 patients, and subtotal resection (STR) was achieved in 16 patients. After an average follow-up period of 41.5months, recurrence was noted in 5 patients (5 of 16) who underwent STR. SMEs have some characteristic features on MRI which can facilitate the preoperative diagnosis. Complete surgical resection is the best treatment of choice with a favorable outcome. In cases of incomplete resection, postoperative radiotherapy may be an effective alternative.

F41CHILDHOOD PHYSICAL AND PSYCHOLOGICAL MALTREATMENT MODIFIES THE RISK OF OBESITY ON DEPRESSION

Spinal myxopapillary ependymoma (sMPE) is an uncommon primary spinal neoplasm infiltrating the spinal cord, conus medullaris (CM), and nerve roots. It is associated with low resection and high recurrence rates. The purpose of this literature review is to evaluate the exact impact of the involvement of the CM and the role played by gross total resection (GTR) on overall survival (OS).The English literature was systematically investigated using MEDLINE, the NIH Library, PubMed, and Google Scholar search engines with relevant queries. Case series reporting details concerning OS, GTR, and CM involvement rate were included, with a differential statistical weight given by the number of patients enrolled. A final cohort of 1602 clinical records was analyzed according to the 3 selected end point variables.The average age was 36.44 ± 3.41 years, and the CM was involved in 28.4% ± 28.2% of cases. The average GTR rate was 53.94% ± 22.20%. Five- and 10-year OS rates were respectively available in 1170 and 1167 cases, with an average 5- and 10-year OS rate of 94.99% ± 3.87% and 92.31% ± 5.73%. By means of analyses performed both on aggregated and disaggregated data a strong positive statistical connection between GTR and increased OS was demonstrated despite the real clinical advantage could range as low as around 1% of increased OS rate.Given the indolent sMPE behavior, it is difficult to evaluate the exact impact of GTR and CM involvement on OS; however, GTR could be associated with a limited survival advantage, whereas CM involvement could be associated with a survival disadvantage.

Differentiation of spinal myxopapillary ependymomas from schwannomas by contrast-enhanced MRI

BackgroundSpinal myxopapillary ependymomas (MPEs) and schwannomas (SCHs) are both frequently located at the conus and caudal regions. The differentiation between MPEs and SCHs before surgery is crucial. Signal patterns of MPEs and SCHs on T2-weighted (T2W) magnetic resonance imaging (MRI) are diverse. Gadolinium (Gd+)-enhanced areas in MRI scans areas are areas where tumor cells are abundant. Therefore, investigating these enhanced areas should facilitate the differentiation. This study aimed to evaluate Gd+ areas in MRI scans in spinal MPEs and SCHs. Furthermore, the relation between MRI pattern and pathological features was investigated. MethodsIn total, 41 patients with spinal MPEs (n = 7) or SCHs (n = 34) around the conus medullaris were included. SCHs were classified per the Gd+ area of the tumor on T1-weighted (T1W) contrast images, into Gd+ areas with T2W hyperintensity (Gd+/T2high) (n = 4, 11.8%) or Gd+ areas with T2W isointensity (Gd+/T2iso) (n = 30, 88.2%). Four patients with Gd+/T2iso were selected randomly for comparison. In three patient groups, T2 values at the Gd+ area and tumor cell density as measured by HE stains (cell density) were compared. ResultsT2 values at the Gd+ area was higher in patients with MPE than in those with SCH with Gd+/T2high, and significantly higher than that in patients with SCH with Gd+/T2iso. Cell density was significantly lower in patients with MPEs than for those with SCHs with Gd+/T2high, and those with SCHs with Gd+/T2iso. ConclusionThe evaluation of the Gd+ area proved useful because it excludes cysts or necrotic portions. If the Gd+ area is hyperintense on T2WI, MPEs can be suspected. If the Gd+ area is isointense on T2WI, SCHs can be suspected. There were several exceptional cases of SCH with a Gd+ area that was hyperintense on T2WI. This could be explained by pathological findings showing low cell density, as is typical for MPEs.

DNA methylation-based classification of ependymomas in adulthood: implications for diagnosis and treatment

Ependymal tumors are glial tumors that commonly manifest in children and young adults. Their classification has remained entirely morphological until recently, and surgery and radiotherapy are the main treatment options, especially in adults. Here we sought to correlate DNA methylation profiles with clinical and pathological characteristics in the prospective cohort of the German Glioma Network. Tumors from 122 adult patients with myxopapillary ependymoma, ependymoma, anaplastic ependymoma, subependymoma, or RELA fusion-positive ependymoma classified according to the World Health Organization (WHO) 2016 were subjected to DNA methylation profiling using the Illumina HumanMethylation450 BeadChip platform. Molecular data were correlated with histologic features and clinical characteristics. At a median follow-up of 86.7 months, only 22 patients experienced progression (18.0%) and 13 patients (10.7%) died. Each tumor could be assigned to one of the previously defined molecular ependymoma subgroups. All histologic subependymomas corresponded to subependymoma (SE) DNA methylation subgroups, but the reverse was not true: 19 histologic ependymomas (WHO grade II) were allocated to molecular SE groups. Similarly, all histological myxopapillary ependymomas were assigned to the molecularly defined spinal myxopapillary ependymoma (SP-MPE) class, but this molecular subgroup additionally included 15 WHO grade II ependymomas by histology. Overall, WHO grade II ependymomas distributed into 7 molecular subgroups. Most adult patients with ependymoma show a favorable prognosis. Molecular classification may provide diagnostic and prognostic information beyond histology and facilitate patient stratification in future clinical trials. The prognostic significance of a subependymoma or myxopapillary ependymoma DNA methylation phenotype without corresponding histology requires further study. 1. Ependymoma diagnosed in adult patients most often shows a good prognosis. 2. Molecular classification can support diagnostic and prognostic information beyond histology.

Metastases of spinal myxopapillary ependymoma: unique characteristics and clinical management.

OBJECTIVE Myxopapillary ependymoma is a benign WHO Grade I tumor most commonly located in the conus-cauda equina region of the spine. Although this tumor is defined by an overall excellent survival, dissemination throughout the whole neuraxis occurs frequently. The current study evaluated the clinical characteristics and significance of myxopapillary ependymoma metastases. METHODS Patients who underwent surgery from 2005 to 2015 for treatment of spinal myxopapillary ependymoma were included in the study. Charts were reviewed for primary tumor symptoms and initial treatment, local recurrence, response to salvage therapy, and presence and behavior of distant metastases. RESULTS Nineteen patients with spinal myxopapillary ependymoma were included in the study (52.6% female). The median age at first diagnosis was 32 years old (range 9-58 years old), and 26.3% were ≤ 18 years old. The median follow-up of all included patients was 48 months (range 6-456 months). Of the primary tumors, 84.2% were located in the lower thoracic or upper lumbar spine, spanning 1-3 levels in 94.7%. All patients underwent surgery for initial treatment: in 78.9% a gross-total resection (GTR) was achieved, with adjuvant radiation therapy (RT) in 20%. Of the 21.2% who underwent a subtotal resection (STR), 75% underwent postoperative RT. Tumor progression was noted in 26.3% of patients after a median 36-month follow-up (range 12-240 months). In 57.9% of patients, distant metastases were found, of which 36.4% were present at initial diagnosis. Further metastases occurred within a median of 20 months (range 2-360 months). Following a diagnosis of metastatic tumor, 72.7% did not show progression and no symptoms were observed during a median follow-up of 36 months (range 6-216 months). Metastases occurred in all parts of the neuroaxis, but were principally localized in the thoracic and sacral spine in 38.9% and 33.3%, respectively; the brain was involved in 11.1%. In 54.5%, more than 1 level was affected. Overall survival was 100% with an excellent clinical and neurological outcome in 78.9% of cases. CONCLUSIONS Metastatic dissemination within the CNS can be observed in many patients with myxopapillary ependymoma. GTR of the primary tumor should be the primary treatment goal, and additional RT is recommended after STR. For distant metastases of myxopapillary ependymoma without clinical manifestation, close clinical and MRI follow-up represents a sufficient strategy because most of the metastases remain asymptomatic and do not show progression over time. Additional resection or irradiation as salvage therapy would be recommended if metastases become symptomatic.

Abstract LB-224: A novel model of pediatric spinal ependymoma using conditionally reprogrammed cells from a primary tumor demonstrates aberrant expression of HMGA, HOX, MYC and other Wnt target genes

Abstract Pediatric spinal ependymomas are rare tumors of the central nervous system with limited treatment options. Successful outcomes depend primarily upon the extent of surgical resection, although significant impairment is common, particularly in children with developing nervous systems. Because these tumors are uncommon, there are no established tumor models nor are there many studies to define underlying molecular lesions. Here, we describe an innovative spinal ependymoma (SE) tumor model based on the recent discovery that primary carcinoma cells can be cultured indefinitely ex vivo on fibroblast feeder cells in the presence of a rho kinase inhibitor; these cells are denoted conditionally reprogrammed cells (CRCs). In prior studies, CRCs form spheres in vitro, replicate tumor pathology as xenografts in vivo, and predict drug responses to the primary tumor. We therefore adopted this strategy to generate a cell line from a 12 year-old girl with a spinal myxopapillary ependymoma. The cell line and primary tumor are of human origin and identical with respect to short tandem repeat (STR) analysis. Using targeted gene expression analysis, we discovered that genes encoding the of High Mobility Group Protein AT-hook 1 (HMGA1), High Mobility Group Protein AT-hook 2 (HMGA2), cMYC, HOXB13, and HOXA10 proteins are all up-regulated compared to their expression in spinal cord tissue from a normal individual. In primary pediatric tumors, HMGA1 gene expression was also higher in spinal ependymomas as compared to supratentorial or posterior fossa ependymomas. There was also a significant positive correlation between HMGA1 and HOX genes, including HOXA10, HOXD13, HOXD1, and HOXB7 in primary tumors. In addition, HMGA1 and cMYC, together with other Wnt target genes, were positively correlated in primary spinal ependymoma. Pathway analysis showed that HMGA1 expression correlated with the following pathways: 1) metabolic pathways, 2) osteoclast differentiation, 3) MAPK signaling, and, 4) Neurotropin signaling. Studies are underway to generate additional cell lines from primary tumor samples and to elucidate the mechanisms that account for up-regulation in HMGA1, HOX and Wnt genes. We will harness this information to identify novel therapeutic targets and agents for children and young adults with spinal ependymoma. Citation Format: Li Z. Luo, Ewa Krawczyk, Anbarasu Lourdusamy, Lisa C. Storer, Lingling Xian, Kenneth J. Cohen, Richard Schlegel, Richard Grundy, Linda Resar. A novel model of pediatric spinal ependymoma using conditionally reprogrammed cells from a primary tumor demonstrates aberrant expression of HMGA, HOX, MYC and other Wnt target genes [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2017; 2017 Apr 1-5; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2017;77(13 Suppl):Abstract nr LB-224. doi:10.1158/1538-7445.AM2017-LB-224

RARE-03. EPENDYMOMAS ARISING OUTSIDE OF THE CENTRAL NERVOUS SYSTEM: A CASE SERIES

Extracranial and extraspinal ependymomas are extremely rare tumors mostly published in the literature as case reports. Our institutional database was screened for patients with extra CNS ependymomas over a 25 year period. Eight patients with extra CNS ependymoma were identified. Five cases originated in the sacrum or subcutaneous sacral area, and the other 3 cases originating in the breast, lung and adnexa of the uterus. By histology, most cases were myxopapilary ependymomas (5 cases), 2 cases were grade 2 ependymoma and 1 case was an anaplastic ependymoma. Metastases occurred in 6 cases and 3 patients died due to progressive disease. Most cases required surgery, radiation and chemotherapy. Extra CNS ependymomas are very rare tumors that tend to metastasize, even though most cases are histologically classified as low grade ependymomas, suggesting that the tumor biology does not correlate with conventional histologic findings. Future plans include molecular phenotyping to determine if these rare cancers are molecular similar to the recent findings from infratentorial, supratentorial ependymomas or the metabolic profile of classic spinal myxopapillary ependymoma.

Primary spinal myxopapillary ependymoma in the pediatric population: a study from the Surveillance, Epidemiology, and End Results (SEER) database.

Primary spinal myxopapillary ependymomas (MPE) in children are extremely rare. We examined the patient demographics, treatment modalities, and the associated outcomes of children with MPE using the Surveillance, Epidemiology, and End Results (SEER) national cancer database to gain a better understanding of these tumors. The SEER database (1973-2012) was used to analyze patients 21years of age and younger with histologically confirmed MPE localized to the spinal cord or cauda equina. We analyzed patient demographics, extent of surgical resection, and radiation treatment. Overall survival was calculated using the Kaplan-Meier method. Statistical significance was defined as p < 0.05, with all data analyzed in IBM SPSS Statistics 21. 122 pediatric patients met inclusion criteria. The median age was 16years (range 0-21) with 63 % male and 87 % Caucasian. The mean follow-up time was 4.5years (95 % CI 3.93-5.07). Overall survival at 5 and 10years was 97 and 95 %, respectively. We found 37 % underwent gross-total resection (GTR), 36 % subtotal resection (STR), and 27 % biopsy only. Patients who received GTR alone (n = 37) had a statistically significant increase in overall survival compared to those who received STR plus adjuvant radiation (n = 20) (Χ2 = 5.9, p < 0.05). To our knowledge, this is the largest survival analysis of pediatric patients with MPE. Overall survival is excellent at the 5 and 10-year time points; however, GTR should be the goal of treatment when possible. For patients with MPE, future studies should focus on longer follow-up, the role of radiation, and the therapeutic approach at tumor recurrence.

Spinal myxopapillary ependymoma in an adult male presenting with recurrent acute low back pain: a case report.

BackgroundSpinal intramedullary ependymomas are very rare and occur more commonly in the cervical and upper thoracic regions. These neoplasms tend to manifest in young adulthood, and patients typically present with mild clinical symptoms without objective evidence of neurologic deficits. The mean duration of symptoms is 40 months until the lesion is diagnosed.Case PresentationA 48-year-old male police officer was referred to a chiropractic clinic by a general practitioner for the evaluation of recurrent acute low back pain (LBP). Although the first episode of LBP was resolved, the clinical examination during the second episode revealed subtle changes that warranted referral to magnetic resonance imaging (MRI). The MRI revealed a spinal myxopapillary ependymoma.ConclusionBecause the primary symptoms of spinal intramedullary ependymomas can mimic ordinary LBP presentations, in particular lumbar intervertebral disc herniations, clinicians need to be sensitive to subtle changes in the clinical presentation of LBP patients. Prompt referral to advanced medical imaging such as MRI and early neurosurgical intervention is key to achieve best possible outcomes for patients with spinal intramedullary ependymomas.

Concomitant Double Tumors of Myxopapillary Ependymoma Presented at Cauda Equina-Filum Terminale in Adult Patient.

A 32-year-old man presented with gradually increasing bilateral buttock pain. He had intermittent claudication. Multiple, homogenously enhanced intradural extramedullary lesions at L2-L3 and L5-S1 levels were observed on magnetic resonance imaging. The tumors were debulked and were removed in piecemeal pattern until they had completely been resected. Histopathological examination of the surgical specimens confirmed that both tumors were myxopapillary ependymomas (MPE). MPE presenting as concomitant double tumor at conus-cauda-filum level are very rare. This kind of presentation could not be directly considered as dissemination, since both tumors were in the site of classical origin of MPE. Ten cases of double spinal MPEs have been reported to date. Including the present case, analysis of the 11 patients revealed some facts. There is a male predominance, which is opposite to the ependymomas that are commonly observed in females. Median age at presentation is 15 years. Most pronounced symptom is low back pain that sometimes radiates to lower extremities. Surgical approach was aimed in all tumors, which could be succeeded in all tumors except one. Adjuvant radiation therapy was applied in 5 patients. No recurrences have been reported after surgery or surgery + radiotherapy regimens.

Multiple spinal myxopapillary ependymomas presented with back pain

Multiple spinal myxopapillary ependymomas presented with back pain

Clinical treatments of lumbosacral spinal myxopapillary ependymoma

Objective To explore the disease characteristics and clinical treatment of lumbosacral spinal myxopapillary ependymoma. Methods The data of 50 patients with lumbosacral spinal myxopapillary ependymoma in our department from Jan. 2008 to Nov. 2013 were analyzed respectively. All the patients were treated by microsurgery. The patients without total removal and younger than 18-year-old were treated with adjunctive radiotherapy. Results Total removal was achieved in 43 cases and subtotal in 7. 3 subtotal removal cases were treated with adjunctive radiotherapy. There was no died patient. The mean follow-up period was 17.3 months. The neurological function was improved in 37 cases(74%), without change in 9(18%), and deteriorated in 4(8%). Four patients developed tumor local recurrence and two developed tumor disseminated recurrence.The patients who were treated with adjunctive radiotherapy had no recurrence. Conclusions Total removal was the most effective treatment for lumbosacral spinal myxopapillary ependymoma. The patients without total removal and younger than 18-year-old might need adjunctive radiotherapy and could get excellent or good clinical effects. Key words: Spinal cord neoplasms; Myxopapillary Ependymoma; Microsurgery; Radiotherapy

Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network.

Spinal myxopapillary ependymomas (MPEs) are slowly growing ependymal gliomas with preferential manifestation in young adults. The aim of this study was to assess the outcome of patients with MPE treated with surgery, radiotherapy (RT), and/or chemotherapy. The medical records of 183 MPE patients (male: 59%) treated at the MD Anderson Cancer Center and 11 institutions from the Rare Cancer Network were retrospectively reviewed. Mean patient' age at diagnosis was 35.5 ± 15.8 years. Ninety-seven (53.0%) patients underwent surgery without RT, and 86 (47.0%) were treated with surgery and/or RT. Median RT dose was 50.4 Gy. Median follow-up was 83.9 months. Fifteen (8.2%) patients died, 7 of unrelated cause. The estimated 10-year overall survival was 92.4% (95% CI: 87.7-97.1). Treatment failure was observed in 58 (31.7%) patients. Local failure, distant spinal relapse, and brain failure were observed in 49 (26.8%), 17 (9.3%), and 11 (6.0%) patients, respectively. The estimated 10-year progression-free survival was 61.2% (95% CI: 52.8-69.6). Age (<36 vs ≥36 y), treatment modality (surgery alone vs surgery and RT), and extent of surgery were prognostic factors for local control and progression-free survival on univariate and multivariate analysis. In this series, treatment failure of MPE occurred in approximately one third of patients. The observed recurrence pattern of primary spinal MPE was mainly local, but a substantial number of patients failed nonlocally. Younger patients and those not treated initially with adjuvant RT or not undergoing gross total resection were significantly more likely to present with tumor recurrence/progression.

Pediatric spinal ependymomas: an unpredictable and puzzling disease. Long-term follow-up of a single consecutive institutional series of ten patients.

MethodsTen consecutive children (0–18 years) who underwent primary tumor resection for a spinal ependymoma between 1980 and 2011 were included in this retrospective study. Gross motor function and activities of daily life were scored according to the Barthel Index.ConclusionThree out of six pediatric patients treated for spinal myxopapillary ependymoma are disease-free after 11 to 33 years of follow-up. The other three have progressive disease, after relapses occurring after 4.5, 7, and 20 years, respectively. One out of two patients with grade II ependymoma had progressive disease from 10 years after initial surgery but is in full-time work in spite of widespread metastatic disease after 32 years. One of the two children with grade III tumor died from progressive disease 17 years from primary diagnosis, while the last one is tumor-free after 19 years. The quality of life is good for three of the four patients with widespread disease, and they are managed conservatively aiming at symptomatic treatment intervention if necessary. We strongly advocate lifelong follow-up for children treated for spinal ependymomas.

Outcomes after surgery and radiotherapy for spinal myxopapillary ependymoma: update of the MD Anderson Cancer Center experience.

The role of radiotherapy after surgery for myxopapillary ependymoma (MPE) is unclear. To review long-term outcomes after surgery, with or without radiation, for spinal MPE. Fifty-one patients with spinal MPE treated from 1968 to 2007 were included. Associations between clinical variables and overall survival (OS), progression-free survival (PFS), and local control (LC) were tested with Cox regression analysis. The median age at diagnosis was 35 years (range, 8-63 years). Twenty patients (39%) had surgery alone, 30 (59%) had surgery plus radiotherapy (RT), and 1 (2%) had RT only. At a median follow-up of 11 years (range, 0.2-37 years), 10-year OS, PFS, and LC for the entire group were 93%, 63%, and 67%, respectively. Nineteen patients (37%) had disease recurrence, and the recurrence was mostly local (79%). Twenty-eight of 50 patients who had surgery (56%) had gross total resection; 10-year LC was 56% after surgery vs 92% after surgery and RT (log-rank P = .14); the median time of LC was 10.5 years for patients receiving gross total resection plus RT, and 4.75 years for gross total resection only (P = .03). Among 16 patients with subtotal resection and follow-up data, 10-year LC was 0% after surgery vs 65% for surgery plus RT (log-rank P = .008). On multivariate analyses adjusting for resection type, age older that 35 years at diagnosis and receipt of adjuvant radiation were associated with improved PFS (hazard ratio [HR]: 0.14, P = .003 and HR: 0.45, P = .009) and LC (HR: 0.22, P = .02 and HR: 0.45, P = .009). Postoperative radiotherapy after resection of MPE was associated with improved PFS and LC.

Differentiation of Spinal Schwannomas and Myxopapillary Ependymomas

Retrospective clinical study. The objective of this study is to evaluate magnetic resonance imaging and pathologic features of spinal schwannomas (SCHs) and myxopapillary ependymomas (MPEs) with focus on differentiating 2 disease entities. Few studies have reported on the differentiation of SCHs and MPEs. Fifty-three patients were retrospectively reviewed with histologically confirmed spinal SCHs (41 patients) or MPEs (12 patients) of the cauda equina and/or conus medullaris. We evaluated neurological deterioration after surgery in association with the intraoperative findings of the tumor, as well as with the preoperative magnetic resonance images and postoperative histologic findings. Patients in the SCH group had a greater mean age at surgery and a greater mean disease duration. In the SCH group, all 24 tumors that were homogeneously hyperintense on the T2-weighted (T2W) images showed rim enhancement on the postcontrast T1-weighted (T1W) images. Moreover, all 14 of the SCHs with homogeneous enhancement on the postcontrast T1W images were isointense on the T2W images. However, in the MPE group, all 8 of the tumors that were homogeneously hyperintense on T2W images showed homogeneous enhancement on their postcontrast T1W images. It is very important to differentiate SCHs and MPEs before surgery, because there are reported cases of dissemination of MPEs through cerebrospinal fluid throughout the neuraxis; the tumor must be removed en block to prevent this. Although MPEs and SCHs may have similar imaging characteristics, detailed examination of the magnetic resonance T2W image and postcontrast T1W image facilitates their differentiation.

Cumulative survival analysis of patients with spinal myxopapillary ependymomas in the first 2 decades of life

Reports of myxopapillary ependymomas (MPEs) of the spinal cord in pediatric patients are scarce. In the literature, various authors have shared their experiences with small groups of patients, which makes it difficult to create a consensus regarding the treatment approach for spinal MPEs in young patients. The aim of this study was to perform a survival analysis of patients in the first 2 decades of life whose cases were selected from the published studies, and to examine the influence of various factors on outcomes. A comprehensive search of studies published in English was performed on PubMed. Patients whose age was ≤ 20 years were included for integrative analysis. Information about age, treatment characteristics, critical events (progression, recurrence, and death), time to critical events, and follow-up duration was recorded. The degree of association of the various factors with the survival outcome was calculated by using Kaplan-Meier estimator and Cox proportional hazard model techniques. A total of 95 patients were included in the analysis. The overall rate of recurrence (RR) was 34.7% (n = 33), with a median time to recurrence of 36 months (range 2-100 months). Progression-free survival (PFS) and overall survival rates at 5 years were 73.7% and 98.9%, respectively. Addition of radiotherapy (RT) following resection significantly improved PFS (log-rank test, p = 0.008). In patients who underwent subtotal resection (STR), administering RT (STR + RT) improved outcome with the lowest failure rates (10.3%), superior to patients who underwent gross-total resection (GTR) alone (RR 43.1%; log-rank test, p < 0.001). Addition of RT to patients who underwent GTR was not beneficial (log-rank test, p = 0.628). In patients who had disseminated tumor at presentation, adjuvant RT controlled the disease effectively. High-dose RT (≥ 50 Gy) did not change PFS (log-rank test, p = 0.710). Routine inclusion of RT in the treatment protocol for spinal MPEs in young patients should be considered. Complete resection is always the goal of tumor resection. However, when complete resection does not seem to be possible in complex lesions, RT should be used as an adjunct to avoid aggressive resection and to minimize inadvertent injury to the surrounding neural tissues. High-dose RT (≥ 50 Gy) did not provide additional survival benefits, although this association needs to be evaluated by prospective studies.

Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children

Myxopapillary ependymomas (MPEs) are rare WHO Grade I tumors found in the conus medullaris, cauda equina, and filum terminale. Treatment generally consists of resection with or without adjuvant radiotherapy. Evidence-based guidelines for surgical management are lacking due to the rarity of this tumor. An English-language PubMed search was performed using the key words "myxopapillary" and "ependymoma." Reports describing fewer than 3 patients or those lacking data on the extent of resection or radiotherapy were excluded. A total of 28 articles describing 475 patients met the authors' inclusion criteria. Patients were grouped by extent of resection and whether or not they underwent adjuvant radiotherapy. Differences in recurrence rates were assessed by chi-square test. The overall recurrence rate was 15.5% in patients treated by gross-total resection (GTR) and 32.6% in patients treated by subtotal resection (STR), irrespective of whether they underwent adjuvant therapy (p < 0.001). Regardless of the extent of resection, adjuvant radiotherapy was not associated with a decrease in recurrence rates. The overall recurrence rate was 15.6% in patients who underwent GTR and radiotherapy compared with 15.9% in patients who underwent GTR alone (p = 0.58), and it was 29.3% in patients who underwent STR and radiotherapy compared with 35.1% in those who underwent STR alone (p = 0.53). The difference between recurrence rates for patients who underwent GTR alone versus STR and radiotherapy was statistically significant (p = 0.02). Subgroup analysis demonstrated significantly higher recurrence rates in pediatric patients compared with adults (40.5% vs 23.4%, respectively; p = 0.02). Even in the setting of GTR alone, recurrence rates were higher in pediatric patients (65% vs 7.6%; p < 0.001). Gross-total resection alone is associated with decreased recurrence rates compared with STR with or without radiotherapy. The authors' results suggest that treatment goals should include attempted GTR whenever possible. The observation that children benefitted from radiation therapy to a greater extent than did adults suggests that biological differences between tumors in these patient populations warrants more rigorous scientific studies.

The nature of double concomitant myxopapillary ependymoma: report of a case

Myxopapillary ependymomas are almost exclusively seen at the conus medullaris/filum terminale/cauda equina region, usually as solitary space-occupying lesions. The authors report the case of a 14-year-old boy with double concomitant myxopapillary ependymoma, proximal and caudal on the filum terminale in which a totally gross removal was achieved in two stages. This presentation is rare and, so far, we have known just three similar cases that were previously reported in children. The true nature of these lesions is controversial, and while some argue that they are related to metastatic seeding, others consider them independent lesions developing synchronously. A review on dissemination of spinal myxopapillary ependymomas was done.