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Myxopapillary ependymomas (MPEs) are rare spinal cord tumors with low rates of metastasis outside of the neuraxis. Gross total resection of MPEs can significantly improve progression-free survival; however, adjunctive treatment remains unstandardized. A 29-year-old female with a history of spina bifida occulta surgical correction and lower back pain presented with dyspnea and tachycardia. A large pulmonary artery mass was discovered consistent with pulmonary thromboembolism. It was subsequently determined to be an intravascular metastasis secondary to sacral MPE. Standardization of MPE treatment and clinical suspicion of spinal neoplasm in the setting of chronic back pain with undetermined origin are of value. [ABSTRACT FROM AUTHOR]

Ependymomas are rare nervous system tumors that can arise anywhere in the neuraxis. While having a high propensity for leptomeningeal dissemination, retrograde dissemination (from the spine to the CNS) remains infrequent. We describe the case of a 31-year-old female who presented with hydrocephalus secondary to an intracranial leptomeningeal metastasis of a giant spinal ependymoma with mixed (classic and myxopapillary) histopathologic features, successfully treated with surgical resection and radiotherapy of the entire neuraxis. This case highlights the importance of including ependymomas in the differential diagnosis for lesions in atypical extra-axial locations, of systematically obtaining imaging of the entire neuraxis when suspecting it, and of considering retrograde dissemination when both intracranial and spinal lesions are present.

Simple Summary: Myxopapillary ependymoma (MPE) is a rare tumor that typically grows in the lower spine. It is usually not aggressive and is most effectively treated with surgery. We studied data from over a thousand patients with spinal MPE from 2000 to 2020. Surgery was the main treatment for almost all patients. We found that younger age and having surgery were linked to better survival. Surprisingly, being male was associated with worse survival, and they tended to be diagnosed at a younger age than females. Our study, the largest of its kind, provides important information for treating spinal MPE. (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. [ABSTRACT FROM AUTHOR]

Myxopapillary ependymoma, a rare variant of ependymoma, commonly occurs in the conus medullaris or filum terminale. The rarity of these tumors can make their diagnosis and treatment challenging. This case report presents an atypical occurrence of myxopapillary ependymoma within the sacrum in a 68-year-old patient presented with a 3-month history of persistent left-sided low back pain radiating to the legs and fecal dysfunction. The patient underwent a sacral laminectomy and subtotal excision of the tumor, followed by adjuvant radiotherapy with favorable outcomes.This report highlights the significance of tailored approaches for unconventional tumor locations emphasizes the potential benefits of multimodal treatment strategies and provides insights from a comprehensive literature review on similar cases.

Ependymoma is a central nervous system (CNS) tumor that arises from the ependymal cells of the brain's ventricles and spinal cord. The histopathology of ependymomas is indistinguishable regardless of the site of origin, and the prognosis varies. Recent studies have revealed that the development site and prognosis reflect the genetic background. In this study, we used genome-wide DNA methylation array analysis to investigate the epigenetic background of ependymomas from different locations treated at our hospital. Four cases of posterior fossa ependymomas and 11 cases of spinal ependymomas were analyzed. DNA methylation profiling using the DKFZ methylation classifier showed that the methylation diagnoses of the 2 cases differed from the histopathological diagnoses, and 2 cases could not be classified. Tumor that spread from the brain to the spinal cord was molecularly distinguishable from other primary spinal tumors. Although adding DNA methylation classification to conventional diagnostic methods may be helpful, the diagnosis in some cases remains undetermined. This may affect decision-making regarding treatment strategies and follow-up. Further investigations are required to improve the diagnostic accuracy of these tumors. [ABSTRACT FROM AUTHOR]

In the evolving landscape of ependymoma classification, which integrates histological, molecular, and anatomical context, we detail a rare case divergent from the usual histopathological spectrum. We present the case of a 37‐year‐old man with symptomatic spinal cord compression at the L3–L4 level. Neuroradiological evaluation revealed an intradural, encapsulated mass. Histologically, the tumor displayed atypical features: bizarre pleomorphic giant cells, intranuclear inclusions, mitotic activity, and a profusion of eosinophilic cytoplasm with hyalinized vessels, deviating from the characteristic perivascular pseudorosettes or myxopapillary patterns. Immunohistochemical staining bolstered this divergence, marking the tumor cells positive for glial fibrillary acidic protein and epithelial membrane antigen with a characteristic ring‐like pattern, and CD99 but negative for Olig‐2. These markers, alongside methylation profiling, facilitated its classification as a myxopapillary ependymoma (MPE), despite the atypical histologic features. This profile underscores the necessity of a multifaceted diagnostic process, especially when histological presentation is uncommon, confirming the critical role of immunohistochemistry and molecular diagnostics in classifying morphologically ambiguous ependymomas and exemplifying the histological diversity within MPEs. [ABSTRACT FROM AUTHOR]

Objective: Rare disease. Background: Myxopapillary ependymoma is a rare type of slow-growing tumor that mainly occurs in the spinal cord, particularly in the region of the conus medullaris and the cauda equina. It originates from the ependymal glial cells found in the filum terminale. Case Report: We present a clinical case of a 44-year-old male patient who presented with symptoms of non-specific pain in the lower back persisting for the past 2 years. He did not report any specific neurological deficits or radicular symptoms. Unenhanced MRI of the lumbar spine showed a giant intradural, extramedullary, heterogenous, expansive tumor at the level L1-S4 with erosion of the sacral bone and invasion of presacral tissue. Based on its characteristic localization and growth pattern, suspicion arose for myxopapillary ependymoma. Biopsy confirmed the initial diagnosis. Partial resection of the tumor with laminectomy and laminoplasty was deemed necessary. Preoperative neural axis MRI showed contrast-enhancing lesions in the cerebellum and the cervical and thoracic spine; therefore, adjuvant radiation therapy was administered. Following the surgery, the patient experienced intermittent episodes of neurological deficits and required physiotherapy. Control MRI a year after the operation showed tumor growth and more metastases along the neural axis. Conclusions: Complete surgical excision of the tumor is the preferred treatment approach, but there is a risk of recurrence even after total excision, so radiotherapy is recommended to minimize the risk of recurrence. Prior to surgery, it is essential to conduct MRI/PET/CT of the head and spine to assess the possibility of metastases. [ABSTRACT FROM AUTHOR]

Myxopapillary ependymoma (MPE) is an uncommon variant of ependymoma, almost exclusively seen in conus medullaris or filum terminale. MPE can be diagnostically challenging, especially when arising extra‐axially. Here we report 5 cases of superficial soft tissue/cutaneous MPE, identified across three tertiary institutions. All patients were female and three of them (3/5, 60%) were children (median age 11 years, range 6–58 years). The tumors presented as slow‐growing masses of the sacrococcygeal subcutaneous soft tissues, occasionally identified after minor trauma and clinically favored to be pilonidal sinuses. Imaging showed no neuraxis connection. Macroscopically, tumors were well‐circumscribed, lobulated, and solid and microscopically they exhibited typical histopathology of MPE, at least focally. Two of the tumors (2/5, 40%) showed predominantly solid or trabecular architecture with greater cellular pleomorphism, scattered giant cells, and increased mitotic activity. All tumors (5/5, 100%) showed strong diffuse immunohistochemical expression of GFAP. One tumor clustered at the category "ependymoma, myxopapillary" by methylome analysis. Two patients (2/5, 40%) had local recurrence at 8 and 30 months after the initial surgery. No patients developed metastases during the follow‐up period (median 60 months, range 6–116 months). Since a subset of extra‐axial MPEs behaves more aggressively, timely and accurate diagnosis is of paramount importance. [ABSTRACT FROM AUTHOR]

Background: Patients affected by Von Hippel-Lindau (VHL) are prone to develop central nervous system neoplasms such as hemangioblastomas (HBs). Myxopapillary ependymoma (MPE) is not commonly associated with VHL disease. Case Description: We present the first case of a VHL patient affected by simultaneous silent cauda equina MPE and a symptomatic conus medullaris HB. The patient was first operated for systemic tumors and followed for asymptomatic HBs. Simple surveillance was maintained until neurological symptoms appeared. Regular followup demonstrated objective growth of the cystic conus medullaris tumor while the cauda equina lesion remained stable. Surgery was performed to avoid further neurological worsening. Histopathological examination showed conus medullaris HB and a nearby cauda equina MPE. Conclusion: Simultaneous spinal HBs and isolated MPE may exceptionally occur in VHL patients. [ABSTRACT FROM AUTHOR]

Purpose Distinguishing intradural extramedullary (IDEM) spinal ependymoma from myxopapillary ependymoma is challenging due to the location of IDEM spinal ependymoma. This study aimed to investigate the utility of clinical and MR imaging features for differentiating between IDEM spinal and myxopapillary ependymomas. Materials and Methods We compared tumor size, longitudinal/axial location, enhancement degree/ pattern, tumor margin, signal intensity (SI) of the tumor on T2-weighted images and T1-weighted image (T1WI), increased cerebrospinal fluid (CSF) SI caudal to the tumor on T1WI, and CSF dissemination of pathologically confirmed 12 IDEM spinal and 10 myxopapillary ependymomas. Furthermore, classification and regression tree (CART) was performed to identify the clinical and MR features for differentiating between IDEM spinal and myxopapillary ependymomas. Results Patients with IDEM spinal ependymomas were older than those with myxopapillary ependymomas (48 years vs. 29.5 years, p < 0.05). A high SI of the tumor on T1W1 was more frequently observed in IDEM spinal ependymomas than in myxopapillary ependymomas (p = 0.02). Conversely, myxopapillary ependymomas show CSF dissemination. Increased CSF SI caudal to the tumor on T1WI was observed more frequently in myxopapillary ependymomas than in IDEM spinal ependymomas (p < 0.05). Dissemination to the CSF space and increased CSF SI caudal to the tumor on T1WI were the most important variables in CART analysis. Conclusion Clinical and radiological variables may help differentiate between IDEM spinal and myxopapillary ependymomas.

Myxopapillary ependymomas (MPEs) are well-circumscribed tumors arising mainly from the caudal neuraxis, i.e., conus medullaris (CM) and filum terminale (FT), commonly seen in adults with median age at presentation of 39 years. 1 Owing to its partially aggressive clinical behavior involving cerebrospinal fluid dissemination and local recurrence, MPE is classified as grade 2 in the fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System. 2 Gross total resection without capsular violation is key, with subtotal resection being directly associated with local recurrence. 3 The FT has an intradural and extradural component. The intradural FT extends from the inferior tip of the CM to the coccyx. 4 The intradural FT–CM junction is not demarcated, but rather a zone of transition, with neural tissue being incrementally replaced by fibrous tissue of filum, gradually converging to a pure non-neural FT. 5 In intramedullary FT MPE in close proximity to the lower end of conus, achieving gross total resection presents a great challenge. Neuromonitoring is crucial to ensure preservation of vital CM functions. We present the case of a 33-year-old man with focal nocturnal back pain of 6 months' duration followed by bilateral lower limb deep boring pain. He had no neurological deficits. Preoperative magnetic resonance imaging revealed a T2 hyperintense, heterogeneous contrast-enhancing intradural extramedullary mass at L1. Video 1 highlights step-by-step en bloc excision of the FT MPE with technical nuances, including intraoperative neurophysiological monitoring. Triggered electromyography (EMG) was used to positively map the eloquent CM and identify the intradural FT–conus interface at the superior pole of the tumor, which was then carefully dissected under continuous bulbocavernosus reflex monitoring. Similarly, we confirmed non-neural intradural FT at the lower pole by negative mapping and resected the lesion en bloc with an adequate stump for clear margins. Free-run EMG monitored all the rootlets that adhered to or were in close proximity to the lesion, ensuring their integrity and an uneventful postoperative recovery. Figure 1 depicts the anatomical orientation of the lesion with surrounding neural structures. Histopathology confirmed MPE. En bloc resection with preservation of neurological function remains the mainstay of treatment for FT ependymoma. Understanding the transitional intradural FT–CM interface is essential, often precluding a clear filum stump superiorly while resecting MPE. Intraoperative neurophysiological monitoring is an indispensable adjunct to ensure safe en bloc resection. It is also theoretically possible to use tibial and pudendal sensory evoked potentials (SEPs) in this surgical procedure. However, the clinical utility of SEPs is limited in FT surgery compared with triggered EMG or transcranial motor evoked potentials because conventional SEPs from posterior tibial nerve of the lower extremity do not cover all the root levels at risk, and the change in SEPs cannot be immediately recognized (as SEPs are averaged responses, and there is always a time lag). We did not use pudendal SEPs in this study because SEPs may give information only on the sensory sacral pathway. 6 Dermatomal SEPs may be helpful, but again, they provide only sensory information. Instead, we used triggered EMG for mapping the nerve roots and transcranial motor evoked potentials to monitor the motor tracts. Further, we used the bulbocavernosus reflex, an alternative and more precise technique to monitor both motor and sensory nervous pathways at the sacral root level. Moreover, SEPs are more difficult to monitor in very young children and are less relevant in guiding the surgical strategy. Thus, we used both mapping (triggered EMG) and monitoring (transcranial motor evoked potentials and bulbocavernosus reflex) techniques, which can preserve sensory and motor sacral roots in this surgical procedure. [ABSTRACT FROM AUTHOR]

Purpose: 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. Methods: 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). Results: The median age at diagnosis was 33.5 years (range 8–60 years). 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 7.2 years, 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). Conclusion: 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. [ABSTRACT FROM AUTHOR]

Ependymal tumors arise from the ependymal cell remnants of the cerebral ventricles, the central canal of the spinal cord, or the filum terminale or conus medullaris, although most pediatric supratentorial ependymomas do not exhibit clear communication or abutment of the ventricles. In this article, we discuss the classification, imaging characteristics, and clinical settings of these tumors. The WHO 2021 classification system has categorized ependymal tumors based on histopathologic and molecular features and location, in which they are grouped as supratentorial, posterior fossa (PF), and spinal. The supratentorial tumors are defined by either the ZFTA (formerly RELA) fusion or the YAP1 fusion. Posterior fossa tumors are divided into group A and group B based on methylation. On imaging, supratentorial and infratentorial ependymomas may arise from the ventricles and commonly contain calcifications and cystic components, with variable hemorrhage and heterogeneous enhancement. Spinal ependymomas are defined by MYCN amplification. These tumors are less commonly calcified and may present with the “cap sign,” with T2 hypointensity due to hemosiderin deposition. Myxopapillary ependymoma and subependymoma remain tumor subtypes, with no change related to molecular classification as this does not provide additional clinical utility. Myxopapillary ependymomas are intradural and extramedullary tumors at the filum terminale and/or conus medullaris and may also present the cap sign. Subependymomas are homogeneous when small and may be heterogeneous and contain calcifications when larger. These tumors typically do not demonstrate enhancement. Clinical presentation and prognosis vary depending on tumor location and type. Knowledge of the updated WHO classification of the central nervous system in conjunction with imaging features is critical for accurate diagnosis and treatment.

Ependymomas are commonly classified as low-grade tumors, although they may harbor a malignant behavior characterized by distant neural dissemination and spinal drop metastasis. Extra-CNS ependymoma metastases are extremely rare and only few cases have been reported in the lung, lymph nodes, pleura, mediastinum, liver, bone, and diaphragmatic, abdominal, and pelvic muscles. A review of the literature yielded 14 other case reports metastasizing outside the central nervous system, but to our knowledge, no studies describe metastasis in the paravertebral muscles. Herein, we report the case of a 39-year-old patient with a paraspinal muscles metastasis from a myxopapillary ependymoma. The neoplasm was surgically excised and histologically and molecularly analyzed. Both the analyses were consistent with the diagnosis of muscle metastases of myxopapillary ependymoma. The here-presented case report is first case in the literature of a paraspinal muscles metastasis of myxopapillary ependymoma. [ABSTRACT FROM AUTHOR]

Background: Myxopapillary ependymomas (MPEs) possess leptomeningeal, neural axis dissemination, seeding metastases, and extra-neural spread which are malignant characteristics, even they classified low-grade lesions as WHO grade II. Managing such lesions remains an arguable neurosurgical challenge. The study aimed to discuss the management of MPEs by evaluating the long-term surgical outcomes of consecutively treated MPEs in different 3 neurosurgical centers. Methods: Medical records of all diagnosed patients with spinal tumors at our institutions were reviewed retrospectively. This study included all consecutive MPE who underwent surgical intervention in our institutions in different periods between February 2004 and December 2020. Results: A total of 44 patients with MPE were treated surgically in three institutions. 28 (63.6%) patients were males. Six patients were pediatric patients (≤18 years). The mean age was 36.2 years. The preoperative course was 19.2 months. The most common symptom was leg pain, observed in 35 (79.5%) patients. Gross-total resection was performed in 35 (79.5%) patients. 39 (88.7%) patients had good functional outcomes with an average follow-up period of 106.2 months. The progression was observed in 5 (11.4%) patients. Extending >2 segments, unclear boundaries, bone-erosions were associated with poor prognosis and progression. Laminectomy and surgical complications were associated with poor functional outcomes. En bloc resection without violation of lesions' integrities reduced the progression. Conclusions: Radiological, intraoperative, and surgical factors can affect the functional outcomes and the progression of MPEs. Some precautions in the surgical interventions particularly in MPEs with defined radiological features can improve functional outcomes and reduce the progression risk. [ABSTRACT FROM AUTHOR]

Study Design: Retrospective Study., Objective: Myxopapillary ependymomas (MPEs) are a unique subgroup of spinal ependymomas originating from the filum terminale's ependymal glia. The 2021 WHO classification reclassified all MPEs as grade 2, recognizing their higher recurrence risk. Due to their rarity, our objective with this study is to understand MPEs' clinical course and optimal management through a large retrospective cohort analysis., Methods: From the years 2000 to 2020, patients with MPEs were identified from the Surveillance, Epidemiology, and End Results (SEER) database. Univariate and multivariable Cox proportional hazard models were run to identify variables that had a significant impact on the primary endpoint of overall survival (OS). A predictive nomogram was built to predict 5-year and 10-year survival probability., Results: This retrospective cohort includes 1373 patients. Patients 65 years or older at diagnosis had a poorer OS ( P < 0.001). Most patients received subtotal resection. Only 320 patients (23%) received gross total resection (GTR). Patients that received GTR had the best OS when compared against all other modalities of treatment ( P < 0.05). Receiving radiotherapy did not affect OS in patients with MPE ( P = 0.2). Nomogram includes patient age and treatment modalities, demonstrating acceptable accuracy in estimating the survival probability at 5-year and 10-year intervals, with a C-index of 0.80 (95% CI of 0.71 to 0.90)., Conclusion: This study highlights the survival benefit of GTR in the treatment of patients with MPE. The role of adjuvant radiotherapy remains unclear as it did not seem to improve OS. The nomogram stratifies the risk of survival in patients with MPE based on age and treatment modality., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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 dysfunction deteriorated 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., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Ethics Committee of Hamamatsu University School of Medicine issued approval 19-146. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Yamada et al.)

Myxopapillary ependymoma (MPE) is a rare, slow-growing tumor that commonly arises in the lumbosacral region of the spinal cord, within the filum terminale and cauda equina. The frequent presentation of MPE is back, sacral, or leg pain. The tumor's size, site, and extension usually influence these symptoms. MPE is usually evaluated using magnetic resonance imaging (MRI) because of its superior soft tissue contrast. The best treatment modality is total surgical resection, which improves the long-term survival rate, with follow-up imaging recommended to ensure total resolution. Here, we present the case of a 29-year-old male who presented with symptoms suggestive of severe neurological impairment. An MRI scan revealed an intradural lesion arising from the cauda equina with peripheral and intrathecal haemorrhage, consistent with MPE. He was managed with laminectomy and microsurgical resection of the tumor, which achieved total resection. Postoperative follow-up found gradual improvement in his symptoms, and routine surveillance imaging confirmed the complete resolution of the tumor., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Al-Banna et al.)

Purpose of Review: To review state of art and relevant advances in the molecular genetics and management of ependymomas of children and adults. Recent Findings: Ependymomas may occur either in the brain or in the spinal cord. Compared with intracranial ependymomas, spinal ependymomas are less frequent and exhibit a better prognosis. The new WHO classification of CNS tumors of 2021 has subdivided ependymomas into different histomolecular subgroups with different outcome. The majority of studies have shown a major impact of extent of resection; thus, a complete resection must be performed, whenever possible, at first surgery or at reoperation. Conformal radiotherapy is recommended for grade 3 or incompletely resected grade II tumors. Proton therapy is increasingly employed especially in children to reduce the risk of neurocognitive and endocrine sequelae. Craniospinal irradiation is reserved for metastatic disease. Chemotherapy is not useful as primary treatment and is commonly employed as salvage treatment for patients failing surgery and radiotherapy. Summary: Standard treatments are still the mainstay of treatment: the discovery of new druggable pathways will hopefully increase the therapeutic armamentarium in the near future. [ABSTRACT FROM AUTHOR]

Teaching Point: Myxopapillary ependymoma presenting as a highly destructive lesion in the sacrum is rare but should be included in the differential diagnosis.

Teaching Point: Myxopapillary ependymoma presenting as a highly destructive lesion in the sacrum is rare but should be included in the differential diagnosis. [ABSTRACT FROM AUTHOR]

SOX10 is important in nonneoplastic oligodendroglial development, but mRNA transcripts and protein expression are identified in a wider variety of CNS glial neoplasms than oligodendrogliomas. We previously demonstrated high levels of SOX10 mRNA and protein in pilocytic astrocytomas (PAs) but not ependymomas (EPNs). We now extend these studies to investigate subsets of these 2 tumors that affect infants, pilomyxoid astrocytomas (PMAs) and infant (

Myxopapillary ependymomas (MPEs) with anaplastic features are rarely reported, with only 21 cases identified to date, and long-term recurrence is rarely presented. A case series is presented to expand understanding of this disease by describing 3 unique cases, including 2 that arose from MPE after a prolonged clinical course. A literature review was performed, and 3 cases of MPE with anaplastic features from our institution were included. Patient 1 was a 13-year-old boy who presented with an avidly enhancing intradural lumbar mass. On gross total resection, the tumor was found to be a solid mass with areas of myxopapillary architecture and MIB-1 (Ki-67) index of 12%. Patient 2 was a woman who initially presented at age 22 with a lumbosacral tumor that was treated with surgery and radiation. A recurrent tumor was resected at age 24. At age 50, the patient presented with a large heterogeneous exophytic mass in the sacrum extending into the presacral space and Ki-67 index of 8%. This was treated with complete resection. Patient 3 was a man who initially presented at age 35 with a lower thoracic, upper lumbar mass at L2 extending into the sacrum. Following resection and radiation, a metastatic focus followed an indolent course until causing pain at the age of 48. Ki-67 index was 16%. The presented cases of MPE with anaplastic features make a total of 24 cases on record in the medical literature and demonstrate 2 examples of late recurrence. [ABSTRACT FROM AUTHOR]

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A 6-year-old girl presented with a 1-week history of progressive upper and lower extremity weakness and bilateral upper extremity dysesthesia. Imaging demonstrated a 4.7 × 1.2-cm enhancing intramedullary lesion in the cervical spine from level C2 to C5 with associated cystic components and syringomyelia. The patient underwent a C2–C5 laminoplasty, with gross total resection of the intramedullary lesion. Histological analysis showed small to medium-sized epithelioid cells, with predominantly a solid architecture focally infiltrating into the adjacent spinal cord tissue. Focal papillary differentiation was present along with peri-vascular pseudorosettes, mucin microcysts, and globules of dense collagen. Focal anaplasia was noted with mitosis (5/10 HPF), focal necrosis, and elevated Ki67 10–15%. These findings were consistent with a myxopapillary ependymoma with anaplastic features. CSF cytology was negative for tumor cells. MYCN amplification was not present. She was treated with targeted proton-beam radiation therapy. This is the fourth case of an intramedullary anaplastic myxopapillary ependymoma to date, and the first case in the cervical spine reported in the literature. [ABSTRACT FROM AUTHOR]

Myxopapillary ependymomas (MPE) of the spinal cord are slow-growing benign tumors most frequently found in adults between 30 and 50 years of age. They arise from the ependyma of the filum terminale and are located in the area of the medullary conus and cauda. The recommended treatment option is gross total resection, while patients undergoing subtotal resection usually require radiotherapy. Complete resection without capsular violation can be curative and is often accomplished by simple resection of the filum above and below the tumor mass. Nevertheless, dissemination and distant treatment failure may occur in approximately 30% of the cases. In this paper, we propose an original MPE classification, which is based upon our personal series report concerned with tumor location and its correlation with the extent of resection. We also provide literature review, discussing surgical technique, tumor recurrence rate and dissemination, and adjuvant treatment. In conclusion, our findings suggest that MPE management based on the proposed 5-type tumor classification is favorable when total surgical resection is performed in carefully selected patients. Yet, further studies on a much broader model is obligatory to confirm this.

Myxopapillary ependymomas (MPEs) are rare tumors of the central nervous system, and outcomes are generally worse with recurrent disease. These tumors can rarely metastasize outside the neuraxis. We present a case of a 35-year-old female with a history of MPEs who developed extraneural metastases 11 years after her initial gross total resection. Sites of metastases included multiple bilateral intrapulmonary and pleural-based masses with pleural effusion and a pelvic mass. The patient was treated with dose-dense TMZ and lapatinib and had a mixed radiographic response after 12 cycles of treatment. This is the first known case of extraneural metastases of MPEs to demonstrate a radiographic response to dose-dense TMZ and lapatinib. This case presentation discusses the need to establish optimal treatment of extraneural ependymal metastases, duration of treatment, and strategy for the management of recurrent diseases., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Riegel et al.)

Ependymomas are rare nervous system tumors that can arise anywhere in the neuraxis. While having a high propensity for leptomeningeal dissemination, retrograde dissemination (from the spine to the CNS) remains infrequent. We describe the case of a 31-year-old female who presented with hydrocephalus secondary to an intracranial leptomeningeal metastasis of a giant spinal ependymoma with mixed (classic and myxopapillary) histopathologic features, successfully treated with surgical resection and radiotherapy of the entire neuraxis. This case highlights the importance of including ependymomas in the differential diagnosis for lesions in atypical extra-axial locations, of systematically obtaining imaging of the entire neuraxis when suspecting it, and of considering retrograde dissemination when both intracranial and spinal lesions are present., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Ependymomas are commonly classified as low-grade tumors, although they may harbor a malignant behavior characterized by distant neural dissemination and spinal drop metastasis. Extra-CNS ependymoma metastases are extremely rare and only few cases have been reported in the lung, lymph nodes, pleura, mediastinum, liver, bone, and diaphragmatic, abdominal, and pelvic muscles. A review of the literature yielded 14 other case reports metastasizing outside the central nervous system, but to our knowledge, no studies describe metastasis in the paravertebral muscles. Herein, we report the case of a 39-year-old patient with a paraspinal muscles metastasis from a myxopapillary ependymoma. The neoplasm was surgically excised and histologically and molecularly analyzed. Both the analyses were consistent with the diagnosis of muscle metastases of myxopapillary ependymoma. The here-presented case report is first case in the literature of a paraspinal muscles metastasis of myxopapillary ependymoma.

Background: Myxopapillary ependymoma (MPE) with anaplastic features is extremely rare. There are very few such case reports in the medical literature. Case Description: A 23-year-old female presented with lower back pain, and both urinary and fecal dysfunction. The patient underwent gross total surgical excision of the MR documented expansile intrasacral tumor. The histology was compatible with a MPE containing anaplastic features. Conclusion: The medical literature contains a few comparable cases of subcutaneous sacrococcygeal MPE with anaplastic components. Here, however, we have a young female with an anaplastic intrasacral MPE treated with gross total surgical excision. [ABSTRACT FROM AUTHOR]

Ependymoma is a malignant central nervous system tumor arising from the lining of the ventricles or central canal of the spinal cord. Extradural spinal ependymomas arise from heterotopic ependymal cells or the coccygeal medullary vestige and are extremely infrequent. We present a rare case of presacral extradural ependymoma. Extradural ependymomas typically demonstrate an extraneural spread and, thus, surveillance of the entire central nervous system is not typically recommended. A radiograph of the chest, liver profile, and attention to palpable lymphadenopathy (especially inguinal) on physical examination are vital for surveillance. Obtaining an R0 resection is the most important prognostic factor in survival and local recurrence. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

To retrospective review a series of patients with ependymomas of filum terminale (FTEs) who underwent microsurgical resection and had long-term follow-up. Five adults with FTEs were surgically treated and enrolled in the study. Clinical manifestations, imaging data, intraoperative findings, and postoperative outcome were recorded. Six FTEs were discovered on preoperative lumbar magnetic resonance imaging, with 1 rare case of a single FTE accompanied by a neurofibroma. Gross total resection was achieved in all patients; en bloc excision was carried out in 2 patients for 2 FTEs and 1 neurofibroma, while piecemeal resection was done in 3 patients for 4 FTEs. Most preoperative symptoms were relieved soon after surgery; 1 patient with urination difficulty achieved complete recovery 1 year postoperatively. Tumor recurrence was found in 1 patient 54 months after her first surgery. FTEs are rare subdural extramedullary tumors. Patients usually present initially with back and leg pain. Elaborate microsurgical manipulations and intraoperative electrophysiologic monitoring are helpful in ensuring satisfactory excision and neurological prognosis. Regular long-term follow-up is recommended for all postoperative patients. [ABSTRACT FROM AUTHOR]

Background: Normal pressure hydrocephalus (NPH) associated with tumors of the cauda equina is rare. Here, we report two cases of NPH attributed to cauda equina ependymomas. Case Description: A 63-year-old male presented with progressive gait disturbance, dementia, and urinary incontinence. When the lumbar MR documented an intradural tumor involving the cauda equina at the L2- L3 level; the tumor was excised; pathologically, it proved to be a myxopapillary ependymoma. Postoperatively, however, the patient's continued gait disturbance led to a brain CT that documented ventricular dilation consistent with NPH; following ventriculoperitoneal (VP) shunt placement his symptoms improved. A 65-year-old female also presented with gait disturbance, dementia, and urinary retention. Here, procedures were performed in reverse. When a brain CT showed hydrocephalus, a VP shunt was placed. When symptoms persisted, a lumbar MR demonstrated a T12-L2 intradural tumor; following a lumbar laminectomy for tumor excision, symptoms stabilized. The pathological diagnosis was also consistent with a conus/cauda equina ependymoma. Over the next 10 years, the patient had residual bladder dysfunction (e.g., requiring straight catheterization), but had no shunt dysfunction. Conclusion: We observed two cases of ependymomas of the cauda equina and brain CTs documenting NPH that was successfully surgically managed with stabilization of neurological deficit. In the first case, L2-L3 laminectomy for tumor removal was succeeded by shunting for NPH, while in the second case, initial VP shunting for NPH was followed by a T12-L2 laminectomy for tumor excision. [ABSTRACT FROM AUTHOR]

• This report presents the first example of cauda equina/filum terminale paraganglioma in the middle east. • This case presentation stress on the value of immunohistochemical staining in correct diagnosis of paragangliomas. • With respect to similarities of paraganglioma and ependymoma in H&E staining, and additional IHC staining is necessary. • Magnetic resonance with Gadolinium administration gives useful information for surgical planning but is not diagnostic. • Total removal of the tumor is usually possible in the vast majority of the cases. There is also an extremely low chance of recurrence. Paraganglioma of the filum terminal/cauda equina is a rare slow growing tumor which originates from the ectopic sympathetic neurons. Surgically, total excision may be difficult for this well demarcated tumor surrounded by couple of rootlets but is usually possible in nearly all cases. Actually, final diagnosis cannot be determined intraoperatively, but is possible only after an immunohistochemical staining. Herein, the authors present a middle age woman whose initial symptoms were lower back pain and radiculopathy. Her MRI was found to be compatible with a cauda equina tumor. During her excisional surgery a hard and relatively vascular tumor was fully removed. The Immunohistochemical results were compatible with paraganglioma of the filum terminale. In addition to case presentation, thorough review of the literature is also done. [ABSTRACT FROM AUTHOR]