Spinal Myxopapillary Ependymoma Research Articles

Characteristics of the Preoperative and Surgical Findings in Patients With Bladder Dysfunction After Surgery for Spinal Myxopapillary Ependymoma.

Purpose: To investigate the association between postoperative bladder dysfunction and preoperative/surgical findings on spinal myxopapillary ependymoma (MPE). Methods: The study included eight patients (five males and three females) with an average age of 56.2 years (range: 21-76 years) who underwent tumor resection between 2011 and 2021. The patient history, magnetic resonance imaging findings, intraoperative findings, surgical methods, and postoperative bladder dysfunction were evaluated. The bladder dysfunction was categorized as mild (frequent urination or retarded micturition) and severe (urinary retention or incontinence). Results: The mean postoperative follow-up was 97.3 (42-160) months. Gross total resection (GTR) was performed in six cases, in three cases by en block resection and three cases by piece-by-piece resection. In GTR cases, an en block resection case with intraoperative adhesion to conus and preoperative normal bladder function presented with postoperative severe bladder dysfunction up to one year. A piece-by-piece resection case with intraoperative adhesion to cauda equina and preoperative mild bladder dysfunctiondeteriorated to severe dysfunction postoperatively. Subtotal resection (STR) was performed in two cases, which did not show postoperative bladder dysfunction. There was no recurrence of tumor in the all cases. Conclusions:Surgeons should have in mind that in the case with intraoperative adhesion to conus or cauda equina, performing GTR may lead to deteriorated bladder function postoperatively. Careful detachment and gradual intraoperative neuromonitor are necessary to achieve GTR.

SPIN-02 AN INTRAMEDULLARY MYXOPAPILLARY EPENDYMOMA: A RARE OCCURANCE IN THE CERVICO-THORACIC SPINE

Abstract BACKGROUND Spinal myxopapillary ependymomas are a rare variant of spinal ependymoma that are considered to occur exclusively in the conus medullaris and filum terminale. They account for about 13% of all spinal ependymomas and have a slight male predominance. Case presentation: We report a case of 38-year-old male, Muganda, that presented at the Neurosurgery department Mulago National Referral Hospital with progressive paresthesia and paraparesis, of the upper limbs and chronic neck pain for 2years. MRI showed an isointense lesion on TIWI extending from C4 to T2 levels and exhibited heterogenous enhancement on gadolinium administration. The lesion was hyperintense on T2WI with a syrinx above the tumor. A radiological diagnosis of an intramedullary neoplastic process was made with differentials of Ependymoma, Schwanomma, and Astrocytoma. The patient underwent posterior laminectomy, and the tumor was completely excised. He is currently under follow up by the Neurosurgery team. CONCLUSION This case underscores the rarity of intramedullary myxopapillary ependymomas highlighting diagnostic challenges and the critical role of tailored surgical interventions in managing rare spinal neoplasms.

Outcomes and Pattern of Care for Spinal Myxopapillary Ependymoma in the Modern Era-A Population-Based Observational Study.

(1) Background: Myxopapillary ependymoma (MPE) is a rare tumor of the spine, typically slow-growing and low-grade. Optimal management strategies remain unclear due to limited evidence given the low incidence of the disease. (2) Methods: We analyzed data from 1197 patients with spinal MPE from the Surveillance, Epidemiology, and End Results (SEER) database (2000-2020). Patient demographics, treatment modalities, and survival outcomes were examined using statistical analyses. (3) Results: Most patients were White (89.9%) with a median age at diagnosis of 42 years. Surgical resection was performed in 95% of cases. The estimated 10-year overall survival was 91.4%. Younger age (hazard ratio (HR) = 1.09, p < 0.001) and receipt of surgery (HR = 0.43, p = 0.007) were associated with improved survival. Surprisingly, male sex was associated with worse survival (HR = 1.86, p = 0.008) and a younger age at diagnosis compared to females. (4) Conclusions: This study, the largest of its kind, underscores the importance of surgical resection in managing spinal MPE. The unexpected association between male sex and worse survival warrants further investigation into potential sex-specific pathophysiological factors influencing prognosis. Despite limitations, our findings contribute valuable insights for guiding clinical management strategies for spinal MPE.

Development of a novel prediction model for differential diagnosis between spinal myxopapillary ependymoma and schwannoma

Spinal myxopapillary ependymoma (MPE) and schwannoma represent clinically distinct intradural extramedullary tumors, albeit with shared and overlapping magnetic resonance imaging (MRI) characteristics. We aimed to identify significant MRI features that can differentiate between MPE and schwannoma and develop a novel prediction model using these features. In this study, 77 patients with MPE (n = 24) or schwannoma (n = 53) who underwent preoperative MRI and surgical removal between January 2012 and December 2022 were included. MRI features, including intratumoral T2 dark signals, subarachnoid hemorrhage (SAH), leptomeningeal seeding, and enhancement patterns, were analyzed. Logistic regression analysis was conducted to distinguish between MPE and schwannomas based on MRI parameters, and a prediction model was developed using significant MRI parameters. The model was validated internally using a stratified tenfold cross-validation. The area under the curve (AUC) was calculated based on the receiver operating characteristic curve analysis. MPEs had a significantly larger mean size (p = 0.0035), higher frequency of intratumoral T2 dark signals (p = 0.0021), associated SAH (p = 0.0377), and leptomeningeal seeding (p = 0.0377). Focal and diffuse heterogeneous enhancement patterns were significantly more common in MPEs (p = 0.0049 and 0.0038, respectively). Multivariable analyses showed that intratumoral T2 dark signal (p = 0.0439) and focal (p = 0.0029) and diffuse enhancement patterns (p = 0.0398) were independent factors. The prediction model showed an AUC of 0.9204 (95% CI 0.8532–0.9876) and the average AUC for internal validation was 0.9210 (95% CI 0.9160–0.9270). MRI provides useful data for differentiating spinal MPEs from schwannomas. The prediction model developed based on the MRI features demonstrated excellent discriminatory performance.

Ependymoma in WHO Classification of Tumours 5th Edition

Ependymoma is a rare central nervous system tumor that occurs in all age groups and is one of the most common childhood malignant brain tumors. Until the 3rd edition of the WHO Classification of Brain Tumours, ependymoma was classified based solely on histologic findings, but advances in molecular analysis led to the incorporation of molecular information into the diagnosis of ependymoma for the first time in the 2016 WHO Classification, 4th edition. However, the low correlation between tumor grade and survival prognosis remained a problem. Unlike other malignant brain tumors, ependymoma is genetically and epigenetically characterized with few identified point mutations. Methylation profiling was found to classify ependymoma into distinct subgroups, and the 2021 WHO Classification, 5th Edition, adopted new classifications by histologic findings, genetic alterations, methylation patterns, and anatomic location. This new classification includes 10 diagnostic entities: 1)supratentorial subependymoma; 2)supratentorial ependymoma, ZFTA-fusion positive; 3)supratentorial ependymoma, YAP1-fusion positive; 4)posterior fossa subependymoma; 5)posterior fossa group A ependymoma; 6)posterior fossa group B ependymoma; 7)spinal subependymoma; 8)spinal ependymoma; 9)spinal myxopapillary ependymoma; and 10)spinal ependymoma, MYCN-amplified. The previously accepted term "anaplastic ependymoma" was dropped. As with other CNS tumors, a new classification was introduced based on an integrated diagnosis that incorporates histologic and molecular diagnosis. In this article, we summarize the molecular classification of ependymoma in the WHO Classification of Brain Tumours, 5th edition.

Surgical outcomes and risk factors for recurrence of myxopapillary ependymoma: a single-center experience.

Myxopapillary ependymomas (MPEs) are low-grade, well-circumscribed tumors that often involve the conus medullaris, cauda equina, or filum terminale. They account for up to 5% of all tumors of the spine and 13% of spinal ependymomas, with a peak incidence between 30 and 50 years of age. Because of the rarity of MPEs, their clinical course and optimal management strategy are not well defined, and long-term outcomes remain difficult to predict. The objective of this study was to review long-term clinical outcomes of spinal MPEs and identify factors that may predict tumor resectability and recurrence. Pathologically confirmed cases of MPE at the authors' institution were identified and medical records were reviewed. Demographics, clinical presentation, imaging characteristics, surgical technique, follow-up, and outcome data were noted. Two groups of patients-those who underwent gross-total resection (GTR) and those who underwent subtotal resection (STR)-were compared using the Mann-Whitney U-test for continuous and ordinal variables and the Fisher exact test for categorical variables. Differences were considered statistically significant at p ≤ 0.05. Twenty-eight patients were identified, with a median age of 43 years at the index surgery. The median postoperative follow-up duration was 107 months (range 5-372 months). All patients presented with pain. Other common presenting symptoms were weakness (25.0%), sphincter disturbance (21.4%), and numbness (14.3%). GTR was achieved in 19 patients (68%) and STR in 9 (32%). Preoperative weakness and involvement of the sacral spinal canal were more common in the STR group. Tumors were larger and spanned more spinal levels in the STR group compared with the GTR cohort. Postoperative modified McCormick Scale grades were significantly higher in the STR cohort compared with the GTR group (p = 0.00175). Seven of the 9 STR patients (77.8%) underwent reoperation for recurrence at a median of 32 months from the index operation, while no patients required reoperation after GTR, for an overall reoperation rate of 25%. Findings of this study emphasize the importance of tumor size and location-particularly involvement of the sacral canal-in determining resectability. Reoperation for recurrence was necessary in 78% of patients with subtotally resected tumors; none of the patients who underwent GTR required reoperation. Most patients had stable neurological status postoperatively.

Clinical management and prognosis of spinal myxopapillary ependymoma: a single-institution cohort of 72 patients.

Myxopapillary ependymoma (MPE) was classified as grade 2 tumor in the 2021 World Health Organization central nervous system classification because of its high recurrence probability. This study aimed to investigate predictive factors and management of tumor recurrence. Seventy-two patients with spinal MPE underwent initial surgical treatment at our hospital between 2011 and 2021. Kaplan-Meier curves and Cox regression were used to analyze the correlation between clinical variables and progression-free survival (PFS). The median age at diagnosis was 33.5years (range 8-60years). Twenty-one patients (29.2%) had preoperative spinal drop metastases. Gross total resection (GTR) was performed in 37 patients (51.4%). The median follow-up was 7.2years, and the follow-up rate was 88.9% (64 of 72 cases). Twelve of the 64 patients (18.9%) relapsed, and preoperative drop metastasis occurred in 7 patients (58.3%). The estimated 5-year and 10-year PFS rates were 82% and 77%, respectively. Univariate analysis showed that GTR was associated with improved PFS (hazard ratio [HR] 0.149, p = 0.014), while preoperative drop metastasis (HR 3.648, p = 0.027) and tumor involvement sacrococcygeal region (HR 7.563, p = 0.003) were associated with tumor recurrence. Adjuvant radiotherapy (RT) was significantly associated with improved PFS in patients with preoperative drop metastasis (p = 0.039). Complete surgical resection under the premise of protecting neurological function is an important factor in reducing spinal MPE recurrence. Adjuvant RT is recommended when the tumor invades the capsule with preoperative drop metastasis or adhesion to the nerve and cannot reach GTR.

EPEN-19. Impact of molecular classification on prognosis in children and adolescents with spinal ependymoma: Results from the HIT-MED database

Abstract PURPOSE: Ependymomas of the spinal cord are rare among children, and individual risks of disease progression are difficult to predict. This study aims at evaluating the prognostic impact of DNA methylation-based classification in children with spinal ependymoma. METHODS: Eighty-two patients with spinal ependymoma &amp;lt;22 years registered in the HIT-MED database between 1992 and 2021 were included. Clinical, radiological, and histopathological data were collected retrospectively. DNA methylation profiles of 46 tumors were classified according to the Heidelberg Brain Tumor Classifier. RESULTS: Spinal myxopapillary ependymoma (SP-MPE, n=27) was the most common methylation group followed by spinal ependymoma (SP-EPN, n=15). Two cases belonged to MYCN-amplified subgroup, one had no match, and one was re-classified as anaplastic pilocytic astrocytoma (the latter excluded from final analysis). WHO grade I and III ependymomas (according to the WHO 2016 classification) classified predominantly as SP-MPE, whereas grade II ependymomas clustered into SP-MPE and SP-EPN. 6/15 patients with SP-EPN (40%) suffered from Neurofibromatosis type 2. Among patients with SP-MPE, 23 underwent gross-total and four a subtotal resection (GTR/STR). Relapses of SP-MPE were more common following STR (5-year progression-free survival (5y-PFS) [STR] 25.0% [95% confidence interval: 0.0-68.4], [GTR] 75.0% [53.4-96.6], p=0.003). In the SP-EPN group, 2/8 patients relapsed after STR (5y-PFS 64.3% [22,3-100]) and 0/7 after GTR (n.s.). WHO I° ependymoma had significantly inferior PFS than II° and III° ependymoma (5y-PFS [I°] 39.0% [5.8-62.2], [II°] 82.4% [67.8-97.0], [III°] 50.5% [18.9-82.1], p=0.009). However, PFS did not significantly differ between SP-MPE and SP-EPN (5y-PFS 65.9% [44.9-86.9], 76.9% [46.3-100], respectively). CONCLUSION: Spinal ependymomas of WHO grade I go along with relatively poor PFS in our cohort, while DNA methylation profiling does not segregate patients into distinct risk groups. Still, larger cohorts and further investigations of methylation class heterogeneity in pediatric spinal ependymomas are needed to complete the basis for future clinical decision-making.

Adjunctive treatment of myxopapillary ependymoma.

Myxopapillary ependymoma are rare tumors and optimal therapeutic strategy is remained controversial. The main treatments for myxopapillary ependymoma tumors include surgery and radiotherapy. Hence, the present study aimed to review adjuvant treatment of myxopapillary ependymoma, focusing on spinal myxopapillary ependymoma. The information sources of all articles were the English authoritative databases including PubMed, Web of science, Scopus, Science direct and Google scholar. In this review study, the keywords including adjuvant, treatment, myxopapillary and ependymoma were selected from MeSH medical library. Related articles were published from 2000 to 2020. Given radiation tolerance in the spinal cord is 10-15% lower than that of the brain, it also should be noted that with increased dose and scope of therapeutic field, the corresponding risks are increased, as well. Also, chemotherapy has never been used as the primary treatment approach. Radiotherapy’s value is considered while involving with sensitive areas where chemotherapy is also recommended. Gross total resection is the preferred primary treatment. But the role of adjuvant radiotherapy is debated in different tumor and patient scenarios and no standard treatment strategy had been defined yet. The bottom line is that as long as cellular and molecular methods or gene therapy can be used in the treatment of myxopapillary ependymoma, all the studies confirm that the best treatment method is still wide surgical resection as much as possible.

Clinical characteristics and long-term surgical outcome of spinal myxopapillary ependymoma: a French cohort of 101 patients.

Myxopapillary ependymoma (MPE) is the most frequent tumor affecting the medullary conus. The surgical therapeutic management is still debated and only few studies have focused on the postoperative clinical outcome of patients. This study aimed to demonstrate long-term postoperative outcome and to assess the predictive factors of recurrence as well as the clinical evolution of these patients. From 1984 to 2019, in four French centers, 101 adult patients diagnosed with MPE were retrospectively included. Median age at surgery was 39years. Median tumor size was 50mm and lesions were multifocal in 13% of patients. All patients benefited from surgery and one patient received postoperative radiotherapy. Gross total resection was obtained in 75% of cases. Sixteen percent of patients presented recurrence after a median follow-up of 70months. Progression free survival at 5 and 10years were respectively estimated at 83% and 79%. After multivariable analysis, sacral localization, and subtotal resection were shown to be independently associated with tumor recurrence. 85% of the patients had a favorable evolution concerning pain. 12% of the patients presented a postoperative deterioration of sphincter function and 4% of motor function. Surgery alone is an acceptable option for MPE patients. Patients with sacral location or incomplete resection are at high risk of recurrence and should be carefully monitored.

Clinical Features, Treatments, and Prognostic Factors of Spinal Myxopapillary Ependymoma

To investigate the clinical characteristics and factors affecting the prognosis of myxopapillary ependymoma (MPE). We retrospectively analyzed the clinical data of 24 patients diagnosed with MPE who were surgically treated from January 2010 to January 2020 in the Department of Neurosurgery at Tongji Hospital (Tongji Medical School, Huazhong University of Science and Technology). Among the 24 included patients, there were 13 male and 11 female patients. The ages of the included patients ranged from 15 to 59 years old, with an average age of 35.2 years old. The Preoperative McCormick grade included 20 cases (83.3%) that were grade II and 4 cases (16.7%) that were grade III. The follow-up times ranged from 6 months to 10 years, with an average of 50.9 months. The Postoperative McCormick grade included 7 cases (29.2%) that were grade I, 4 cases (16.7%) that were grade II, 12 cases (50%) that were grade III and 1 case (4.2%) that was grade IV. The 1-year, 2-year, and 10-year recurrence rate was 8.3%, 29.2%, 41.7%, respectively. The 1-year, 2-year, and 10-year survival rate was 100%, 100%, 95.8% respectively. χ2 test revealed a significant difference between the degree of surgical resection (P= 0.012 < 0.05). The Kaplan-Meier method found that the degree of tumor resection (P= 0.031 < 0.05) was related to progression-free survival. The Cox analysis revealed there was no significant independent prognostic factors. Our findings suggest that the degree of surgical resection was a key factor that affected the prognosis and neurologic function of the included patients with MPE.

Integrative molecular characterization of pediatric spinal ependymoma: the UK Children’s Cancer and Leukaemia Group study

BackgroundPediatric spinal ependymomas (SP-EPNs) are rare primary central nervous system tumors with heterogeneous clinical course. Considering that ependymomas in children are biologically distinct from their adult counterparts, this study aimed to define the molecular landscape of SP-EPNs in children.MethodsIn this retrospective study, we have collected tumor samples from 27 SP-EPN patients younger than 18 years and carried out the histological review, DNA methylation, and gene expression profiling.ResultsUnsupervised analyses with methylation profiles revealed 2 subgroups where all grade I tumors (n = 11) were in Group 1, but the grade II/III tumors split into 2 groups (n = 7 in Group 1 and n = 9 in Group 2). The Heidelberg classifier assigned Group 1 tumors as spinal myxopapillary ependymomas (SP-MPEs), 5 Group 2 tumors as SP-EPNs, and failed to classify 4 Group 2 tumors. Copy numbers derived from DNA methylation arrays revealed subgroup-specific genetic alterations and showed that SP-EPN tumors lack MYCN amplification. Gene expression profiling revealed distinct transcriptomic signatures, including overexpression of genes involved in oxidative phosphorylation in SP-MPEs that were validated by Western blot analysis. We discovered widespread decreases in DNA methylation at enhancer regions that are associated with the expression of oncogenic signaling pathways in SP-MPEs. Furthermore, transcription factor motifs for master regulators, including HNF1B, PAX3, and ZIC3, were significantly overrepresented in probes specific to distal regulatory regions in SP-MPEs.ConclusionOur findings show substantial heterogeneity in pediatric SP-EPN and uncover novel enhancers and transcriptional pathways specific to the SP-MPE subgroup, providing a foundation for future therapeutic strategies.

EPEN-10. SPINAL MYXOPAPILLARY EPENDYMOMA AND METHYLATION-PROFILING: THE MD ANDERSON CANCER CENTER (MDACC) EXPERIENCE

INTRODUCTIONSpinal myxopapillary ependymoma (MPE) is a rare histological variant of ependymoma, classified as WHO grade I tumor. Further interrogation of the molecular and clinical profile is warranted, to better understand the biology and clinical phenotype. We summarize our institutional experience with spinal MPE including methylation-profiling.METHODSA retrospective analysis of charts during the period of 2001 to 2019 of histologically proven MPE was done. We performed methylation profiling for 12 patients by Infinium MethylationEPIC Kit.RESULTS26 patients with spinal MPE were identified, median age of diagnosis was 34.2 years with a range of 11 to 59.9 years. Ten patients were below 30 years of age, lumbar spine location was commonest and 6 had leptomeningeal spread at diagnosis. All the patients underwent surgery and 11 received radiation following surgery. Eight patients below the age of 30 received radiation due to residual disease or metastases. Methylation profiling revealed 11,752 CpGs differentially methylated between the younger and older patients (p < 0.05), however only one CpG cg22496254 associated with gene NCAPG/DCAF16 (role in promoting mitosis) was detectable with FDR < 0.25 that overly methylated in the younger age group. This is a new finding in MPE.CONCLUSIONSSpinal MPE is a rare spinal tumor. Our study though limited by numbers, showed younger patients had aggressive phenotype, most requiring radiation. Methylation profiling reaffirmed this finding and trend in the younger patients. Prospective studies in a larger cohort of patients with methylation profiling are needed to identify prognostic variables and new targets for treatment.

Outcomes following limited-volume proton therapy for multifocal spinal myxopapillary ependymoma.

Spinal myxopapillary ependymoma (MPE) often presents with a multifocal distribution, complicating attempts at resection. There remains no standard approach to irradiating these patients. We report disease control and toxicity in pediatric patients with multifocal spinal MPE treated with limited-volume proton therapy. Twelve patients (≤21 years old) with multifocal spinal MPE were treated between 2009 and 2018 with limited-volume brain-sparing proton therapy. Median age was 13.5 years (range, 7-21). Radiotherapy was given as adjuvant therapy after primary surgery in five patients (42%) and for recurrence in seven (58%). No patient received prior radiation. Eleven patients (92%) had evidence of gross disease at radiotherapy. Eleven patients received 54 GyRBE; one received 50.4 GyRBE. Treatment toxicity was graded per the CTCAEv4.0. We estimated disease control and survival using the Kaplan-Meier product-limit method. The median follow-up was 3.6 years (range, 1.8-10.6). The five-year actuarial rates of local control, progression-free survival, and overall survival were 100%, 92%, and 100%, respectively. One patient experienced an out-of-field recurrence in the spine superior to the irradiated region. No patients developed in-field recurrences. Following surgery and irradiation, one patient developed grade three spinal kyphosis and one patient developed grade 2 unilateral L5 neuropathy. 54 GyRBE to a limited volume appears effective for disseminated spinal MPE in both the primary and salvage settings, sparing children the toxicity of full craniospinal irradiation. Compared with historical reports, this approach using proton therapy improves the therapeutic ratio, resulting in minimal side effects and high rates of disease control.

Overexpression of Long Noncoding RNA HOTAIR Is a Unique Epigenetic Characteristic of Myxopapillary Ependymoma.

Ependymomas are a heterogeneous group of central nervous system tumors. Despite the recent advances, there are no specific biomarkers for ependymomas. In this study, we explored the role of homeobox (HOX) genes and long noncoding RNA (LncRNA) HOTAIR in ependymomas along the neural axis. Bioinformatics analysis was performed on publicly available gene expression data. Quantitative RT-PCR was used to determine the mRNA expression level among different groups of ependymomas. RNA in situ hybridization (ISH) with probes specific to HOTAIR was performed on tumor tissue microarray (TMA) constructed with 19 ependymomas formalin-fixed paraffin-embedded tissue. Gene expression analysis revealed higher expression of posterior HOX genes and HOTAIR in myxopapillary ependymoma (MPE), in comparison to other spinal and intracranial ependymoma. qRT-PCR confirmed the high HOXD10 expression in spinal MPEs. There was a significant upregulation of HOTAIR expression in spinal MPE and elevated HOTAIR expressions were further confirmed by RNA ISH on the TMA. Intriguingly, HOXD10 and HOTAIR expressions were not elevated in nonependymoma spinal tumors. Our collective results suggest an important role for the lncRNA HOTAIR and posterior HOX genes in the tumorigenesis of spinal MPE. HOTAIR may also serve as a potential diagnostic marker for spinal MPE.

Image report: Extensive disseminated thoracolumbosacral myxopapillary ependymoma.

Background: Myxopapillary ependymoma occurs more frequently in adults, but is found in the first two decades of life in around 8–20% of patients. Tumors are usually benign with low likelihood for dissemination.Case Description: We describe a case of a 13-year-old boy who presented with progressive kyphosis and bilateral weakness of the lower limbs. MRI shows a thoracolumbosacral intradural tumor with invasion of sacral neural foramina and dissemination to the cervicothoracic region. The patient received T10-L5 laminectomy with subtotal tumor resection. Pathological examination revealed myxopapillary ependymoma. After surgical resection, the patient underwent physical therapy with whole spinal radiotherapy for disease control.Conclusion: Spinal myxopapillary ependymomas are usually benign and slow-growing tumors. This case illustrates an extensive and disseminated myxopapillary ependymoma.

Spinal Seeding Metastasis of Myxopapillary Ependymoma: Report of Three Pediatric Patients and a Brief Literature Review

Objective: Myxopapillary ependymomas (MPEs) in children are rarely reported low-grade glial tumors; however, MPEs sometimes possess malignant characteristics such as spinal seeding/drop metastasis (SSM). We aimed to present 3 pediatric MPE cases that experienced SSM at 2 neurosurgical centers. Materials and Methods: We retrospectively reviewed the medical records of 38 primary spinal MPE cases who underwent surgery at 2 neurosurgical centers spanning 16 years, from 2004 to 2019. All pediatric cases (patient age <18 years) who were diagnosed with MPE and re-presented with SSM were selected as the core sample for this study. Relevant literature was briefly reviewed. Results: Three pediatric MPE cases (2 females and 1 male) experienced SSM. The mean age at first presentation was 12.0 ± 1.0 years. The mean preoperative course was 2.9 ± 1.2 months. The predominant location was the lumbar spine in 2 tumors (both originated from terminal filum [TF]). Two tumors were located intradural intramedullary. Gross-total resection was achieved in 2 patients. No patient had neurofibromatosis type 2. No adjuvant treatment was given after the first surgery. The mean period between the first diagnosis and diagnosis of SSM was 44.0 ± 31.5 months. The location of SSM in all patients was the sacral spine (1 patient experienced distant metastasis in her brain besides her sacral metastasis). The mean follow-up was 68.3 ± 53.7 months. Conclusions: We found a statistically significant relationship between SSM in pediatric MPEs and the intramedullary location, TF origin, and number of affected segments. Close clinical and radiological follow-up is essential for pediatric MPE patients.

Analyzing the role of adjuvant or salvage radiotherapy for spinal myxopapillary ependymomas.

The authors sought to describe the long-term recurrence patterns, prognostic factors, and effect of adjuvant or salvage radiotherapy (RT) on treatment outcomes for patients with spinal myxopapillary ependymoma (MPE). The authors reviewed a tertiary institution IRB-approved database and collected data regarding patient, tumor, and treatment characteristics for all patients treated consecutively from 1974 to 2015 for histologically confirmed spinal MPE. Key outcomes included relapse-free survival (RFS), postrecurrence RFS, failure patterns, and influence of timing of RT on recurrence patterns. Cox proportional hazards regression and Kaplan-Meier analyses were utilized. Of the 59 patients included in the study, the median age at initial surgery was 34 years (range 12-74 years), 30 patients (51%) were female, and the most common presenting symptom was pain (n = 52, 88%). Extent of resection at diagnosis was gross-total resection (GTR) in 39 patients (66%), subtotal resection (STR) in 15 (25%), and unknown in 5 patients (9%). After surgery, 10 patients (17%) underwent adjuvant RT (5/39 GTR [13%] and 5/15 STR [33%] patients). Median follow-up was 6.2 years (range 0.1-35.3 years). Overall, 20 patients (34%) experienced recurrence (local, n = 15; distant, n = 5). The median RFS was 11.2 years (95% CI 77 to not reached), and the 5- and 10-year RFS rates were 72.3% (95% CI 59.4-86.3) and 54.0% (95% CI, 36.4-71.6), respectively.STR was associated with a higher risk of recurrence (HR 6.45, 95% CI 2.15-19.23, p < 0.001) than GTR, and the median RFS after GTR was 17.2 years versus 5.5 years after STR. Adjuvant RT was not associated with improved RFS, regardless of whether it was delivered after GTR or STR. Of the 20 patients with recurrence, 12 (60%) underwent salvage treatment with surgery alone (GTR, n = 6), 4 (20%) with RT alone, and 4 (20%) with surgery and RT. Compared to salvage surgery alone, salvage RT, with or without surgery, was associated with a significantly longer postrecurrence RFS (median 9.5 years vs 1.6 years; log-rank, p = 0.006). At initial diagnosis of spinal MPE, GTR is key to long-term RFS, with no benefit to immediate adjuvant RT observed in this series. RT at the time of recurrence, however, is associated with a significantly longer time to second disease recurrence. Surveillance imaging of the entire neuraxis remains crucial, as distant failure is not uncommon in this patient population.

National trends in management of adult myxopapillary ependymomas

Myxopapillary ependymomas (MPE) are WHO Grade I ependymomas that annually occur in 0.05–0.08 per 100,000 people. Surgical resection is the recommended first line therapy. Due to the rarity of the disease, there is a relatively poor understanding of the use of radiotherapy (RT) in managing this disease. The National Cancer Database (NCDB) was analyzed for patterns of care foradult MPE diagnosed between 2002 and 2016. Of 753 qualifying cases, the majority of patients underwent resection (n = 617, 81.9%). A relatively small portion received RT (n = 103, 13.3%) with most receiving RT post-operatively (n = 98, 95.1%). The likelihood of patients to undergo resection and RT was associated with patient age at diagnosis (p = 0.002), tumor size (p < 0.001), and race (p = 0.017). Chemotherapy was not widely utilized (0.27% of patients). One limitation of our analysis is that there was no data on progression free survival (PFS), an important outcome given the high survival rate in this disease. Surgery remains the primary means to manage adult MPE. For spinal MPE, it is understood that gross total resection (GTR) should be attempted whenever possible as GTR has been associated with improved PFS in several studies. The impact of RT on overall survival (OS) is indeterminate given the 1.6% death rate in the cohort. Analyses of the impact of RT on PFS in a larger database would be beneficial for determining an algorithm for post-operative and definitive RT in this disease entity.

Long-Term Surgical Resection Outcomes of Pediatric Myxopapillary Ependymoma: Experience of Two Centers and Brief Literature Review

Myxopapillary ependymoma (MPE) is a rarely reported lesion of the spinal cord in the pediatric population. Based on their low mitotic index and slow-growth, MPEs are classified as low-grade glial tumors. However, MPEs have malignant characteristics, such as distant neural axis and extraneural axis dissemination. We present the long-term surgical resection outcomes of 5 consecutive pediatric MPE cases and the results of a literature search for reported cases of pediatric spinal MPE. We retrospectively reviewed the medical records of 38 primary spinal MPE cases who underwent surgery at 2 neurosurgical centers over a 16-year period from January 2004 to January 2019. All pediatric cases (age <18 years; n= 5) who were diagnosed with MPE composed the core sample for this study. This series comprised 5 patients (3 females and 2 males), with a mean age at first presentation of 13.6 ± 2.3 years. The mean preoperative course was 8.2 ± 9.3 months. The predominant location was the lumbar spine, for 4 tumors. Gross total resection (GTR) was achieved in 4 patients. All patients were diagnosed histopathologically as MPE, World Health Organization grade I. No adjuvant treatment was provided after the first surgery. Three patients experienced spinal drop metastasis. The mean interval between the first diagnosis and diagnosis of neural dissemination was 44.0 ± 31.5 months. The location of neural dissemination in all patients was the sacral spine; 1 patient experienced distant metastasis in the brain along with her sacral metastasis. The mean duration of follow-up was 75.0 ± 37.6 months. Even with GTR, pediatric MPE has a high propensity for neural axis dissemination. We recommend close clinical and radiologic follow-up for pediatric patients with MPE.