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1
Romero, Flávio Ramalho, Marco Antônio Zanini, Luis Gustavo Ducati, Roberto Bezerra Vital, Newton Moreira de Lima Neto, and Roberto Colichio Gabarra. "Purely Cortical Anaplastic Ependymoma." Case Reports in Oncological Medicine 2012 (2012): 1–4. http://dx.doi.org/10.1155/2012/541431.
Анотація:
Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. It may occur outside the ventricular structures, representing the extraventicular form, or without any relationship of ventricular system, called ectopic ependymona. Less than fifteen cases of ectopic ependymomas were reported and less than five were anaplastic. We report a rare case of pure cortical ectopic anaplastic ependymoma.
2
Neumann, Julia E., Michael Spohn, Denise Obrecht, Martin Mynarek, Christian Thomas, Martin Hasselblatt, Mario M. Dorostkar, et al. "PATH-16. HISTOPATHOLOGICAL EPENDYMOMA VARIANTS ARE ASSOCIATED WITH DISTINCT CLINICAL PARAMETERS AND DNA METHYLATION PATTERNS." Neuro-Oncology 21, Supplement_6 (November 2019): vi146. http://dx.doi.org/10.1093/neuonc/noz175.612.
Анотація:
Abstract According to the current WHO classification, ependymal tumors are classified as subependymomas, myxopapillary ependymomas, classic ependymomas, anaplastic ependymomas and RELA-fusion positive ependymoma (RELA-EPN). Among classic ependymomas, the WHO defines rare histological variants, i.e. the clear-cell, papillary, and tanycytic ependymoma. In parallel to this WHO classification scheme, DNA methylation patterns can distinguish nine distinct molecular ependymoma subgroups, some of which tightly overlap with certain histopathological subgroups, e.g. subependyomas or myxopapillary ependymomas. Since very little is known about the molecular background of histological classic ependymoma variants, we analyzed histomorphology, clinical parameters and global DNA methylation patterns of diagnosed tanycytic ependymomas (n=12), clear-cell ependymomas (n=14) and papillary ependymomas (n=19). Surprisingly, up to 42% of these variants did not match to ependymomas using a previously published DNA methylation-based classifier for brain tumors. Among the tumors with a match to one of the nine known ependymoma methylation classes, tanycytic ependymomas were predominantly located in the spine, but showed diverse molecular methylation patterns. Most clear-cell ependymomas showed a common histomorphology, were found supratentorially and fell into the methylation class of RELA-EPN. Papillary ependymomas showed a “papillary”, “trabecular” or “pseudo-papillary” growth pattern. Interestingly, a true papillary growth pattern was strongly associated with the molecular class B of posterior fossa ependymoma (PFB), but tumors displayed DNA methylation sites that were significantly different when compared to PFB ependymomas without papillary growth. Our results show that the diagnosis of classic histological ependymoma variants can be challenging. While clear-cell and papillary ependymomas harbor common molecular features, tanycytic ependymoma may not represent a molecularly distinct subgroup.
3
Dimopoulos, Vassilios G., Kostas N. Fountas, and Joe Sam Robinson. "Familial intracranial ependymomas." Neurosurgical Focus 20, no. 1 (January 2006): 1–5. http://dx.doi.org/10.3171/foc.2006.20.1.9.
Анотація:
Familial cases of intracranial ependymomas have been well documented in the literature. The authors present two cases from a family in which three members harbored intracranial ependymomas. A 54-year-old man with fourth ventricular ependymoma underwent resection of the tumor followed by radiation therapy. His son presented at age 36 years with a fourth ventricular tanycytic ependymoma and underwent total resection of the ependymoma with postoperative radiation therapy. The father's sister had been treated at another institution for a posterior fossa ependymoma. The association of ependymomas with molecular genetic alterations in chromosome 22 has been previously described. Further investigation of the genetic influences may lead to better therapeutic approaches for this relatively rare clinicopathological entity.
4
Ogino, Shuji, Shigeki Kubo, Fadi W. Abdul-Karim, and Mark L. Cohen. "Comparative Immunohistochemical Study of Insulin-like Growth Factor II and Insulin-like Growth Factor Receptor Type 1 in Pediatric Brain Tumors." Pediatric and Developmental Pathology 4, no. 1 (January 2001): 23–31. http://dx.doi.org/10.1007/s100240010112.
Анотація:
Insulin-like growth factor (IGF)-II is an important growth factor in development of the central nervous system. The purpose of this study was to evaluate expression of IGF-II and IGF receptor type 1 (IGFR1) in various pediatric brain tumors. Immunohistochemistry for IGF-II and IGFR1 was performed on 15 choroid plexus papillomas (CPPs) including 1 atypical CPP, 2 choroid plexus carcinomas (CPCs), 5 anaplastic ependymomas, 7 nonanaplastic ependymomas (simply referred to as “ependymoma”), 5 medulloblastomas, 1 cerebral neuroblastoma, and 1 atypical teratoid/rhabdoid tumor (ATRT) along with 10 non-neoplastic choroid plexus and 3 non-neoplastic ependymal linings. All non-neoplastic choroid plexus, CPPs, CPCs, anaplastic ependymomas, ATRT, 71% of ependymomas, and 67% of non-neoplastic ependymal linings showed cytoplasmic positivity for IGF-II, whereas all medulloblastomas and the cerebral neuroblastoma were negative for IGF-II. In addition to cytoplasmic positivity for IGFR1, membranous positivity was observed in 73% of CPPs, both CPCs, the ATRT, 22% of non-neoplastic choroid plexus, 80% of anaplastic ependymomas, and 29% of ependymomas, but not in any medulloblastoma, cerebral neuroblastoma, or non-neoplastic ependymal lining. IGF-II and IGFR1 may play roles in the pathogeneses of CPP, CPC, anaplastic ependymoma, ependymoma, and ATRT. Immunohistochemical testing for IGF-II and IGFR1 may be useful in differentiating ATRT, CPC, and anaplastic ependymoma from medulloblastoma and cerebral neuroblastoma.
5
Morris, Katrina A., Shazia K. Afridi, D. Gareth Evans, Anke E. Hensiek, Martin G. McCabe, Mark Kellett, Dorothy Halliday, Pieter M. Pretorius, and Allyson Parry. "The response of spinal cord ependymomas to bevacizumab in patients with neurofibromatosis Type 2." Journal of Neurosurgery: Spine 26, no. 4 (April 2017): 474–82. http://dx.doi.org/10.3171/2016.8.spine16589.
Анотація:
OBJECTIVE People with neurofibromatosis Type 2 (NF2) have a genetic predisposition to nervous system tumors. NF2-associated schwannomas stabilize or decrease in size in over half of the patients while they are receiving bevacizumab. NF2 patients treated with bevacizumab for rapidly growing schwannoma were retrospectively reviewed with regard to ependymoma prevalence and response to treatment. METHODS The records of 95 NF2 patients receiving bevacizumab were retrospectively reviewed with regard to spinal ependymoma prevalence and behavior. The maximum longitudinal extent (MLE) of the ependymoma and associated intratumoral or juxtatumoral cysts were measured on serial images. Neurological changes and patient function were reviewed and correlated with radiological changes. RESULTS Forty-one of 95 patients were found to have ependymomas (median age 26 years; range 11–53 years). Thirty-two patients with a total of 71 ependymomas had scans appropriate for serial assessment with a mean follow-up of 24 months (range 3–57 months). Ependymomas without cystic components showed minimal change in MLE. Twelve patients had ependymomas with cystic components or syringes. In these patients, reductions in MLE were observed, particularly due to decreases in the cystic components of the ependymoma. Clinical improvement was seen in 7 patients, who all had cystic ependymomas. CONCLUSIONS Bevacizumab treatment in NF2 patients with spinal cord ependymomas results in a decrease in the size of intratumoral and juxtatumoral cysts as well as adjacent-cord syringes and a decrease in cord edema. This may provide clinical benefit in some patients, although the changes do not meet the current criteria for radiological tumor response.
6
DiLuna, Michael L., Gillian H. Levy, Shreya Sood, and Charles C. Duncan. "Primary Myxopapillary Ependymoma of the Medulla." Neurosurgery 66, no. 6 (June 1, 2010): E1208—E1209. http://dx.doi.org/10.1227/01.neu.0000369513.84063.a6.
Анотація:
Abstract OBJECTIVE Myxopapillary ependymoma is a subclassification of ependymoma that is thought to be nearly exclusive to the conus medullaris or filum terminale. Primary intracerebral or brainstem myxopapillary ependymomas are rare. CLINICAL PRESENTATION An 8-year-old child presented with a 5-month history of nausea and vomiting and a 1-week history of headache. Magnetic resonance imaging revealed a nodular mass in the medulla with an associated cyst extending into the fourth ventricle. INTERVENTION A suboccipital craniotomy was performed, and a gross total resection of the lesion and cyst was achieved. Histological examination confirmed the diagnosis of myxopapillary ependymoma. A discussion of other reported cases of extraspinal myxopapillary ependymomas is presented. CONCLUSION This is the first report of a case of myxopapillary ependymoma, confirmed by histology, in the medulla. Although rare, myxopapillary ependymomas outside of the filum terminale do exist.
7
Maksoud, Yaser A., Yoon S. Hahn, and Herbert H. Engelhard. "Intracranial ependymoma." Neurosurgical Focus 13, no. 3 (September 2002): 1–5. http://dx.doi.org/10.3171/foc.2002.13.3.5.
Анотація:
Object An intracranial ependymoma is a relatively rare but very interesting variety of glioma. In this paper, the authors compiled a review of the pathological features, imaging characteristics, and treatment strategies related to this brain tumor. Methods A Medline search was conducted using the term “ependymoma.” The bibliographies of papers obtained were also checked for articles and chapters that could provide additional understanding of this tumor. Malignant ependy-momas and ependymomas of the spinal cord (including myxopapillary ependymomas) were excluded from this review. Conclusions The posterior fossa is the most frequent site for an intracranial ependymoma. Children are frequently affected. Most authors recommend resecting as much of the tumor as is safely possible. Microscopically, ependymal tumors show both epithelial and glial features. Glial fibrillary acidic protein immunohistochemistry, therefore, helps in identifying ependymomas. Because ependymomas often recur despite surgical intervention, radiotherapy and/or radio-surgery may also play an important role in their treatment. The use of chemotherapy in the treatment of these tumors, especially in the very young, is still being studied.
8
Hallacq, Paul, François Labrousse, Nathalie Streichenberger, Dan Lisii, and Georges Fischer. "Bifocal myxopapillary ependymoma of the terminal filum: the end of a spectrum?" Journal of Neurosurgery: Spine 98, no. 3 (April 2003): 288–89. http://dx.doi.org/10.3171/spi.2003.98.3.0288.
Анотація:
✓ Myxopapillary ependymomas represent the most frequent type of ependymomas found at the conus medullaris—cauda equina-terminal filum level. They are neuroectodermal tumors mainly observed during the fourth decade of life. Pediatric cases have been rarely described at an age range of 10 to 13 years. Myxopapillary ependymomas are typically solitary tumors involving the terminal filum. Simultaneous discovery of two tumors located both on the terminal filum has been reported once. The pathogenesis of this focal ependymoma located at the same embryological level, on the terminal filum, is uncertain; it may represent one end of a spectrum, the other end being the giant ependymoma of the terminal filum.
9
Weinstein, Gene M., Knarik Arkun, James Kryzanski, Michael Lanfranchi, Gaurav K. Gupta, and Harprit Bedi. "Spinal Intradural, Extramedullary Ependymoma with Astrocytoma Component: A Case Report and Review of the Literature." Case Reports in Pathology 2016 (2016): 1–5. http://dx.doi.org/10.1155/2016/3534791.
Анотація:
Ependymomas are common spinal lesions, with the vast majority arising in an intramedullary location. Several cases have been described in the literature of ependymomas in an intradural, extramedullary location. The authors present a case of a 56-year-old female who presented with several weeks of lower back pain and weakness. MRI revealed an intradural, extramedullary enhancing mass at L1-L2. The mass was successfully resected surgically. Pathologic evaluation revealed a low grade glioma with components of both ependymoma and pilocytic astrocytoma with MUTYH G382D mutation. Extramedullary ependymomas are very rare tumors. To the authors’ knowledge, this is the first case of ependymoma/astrocytoma collision tumors described in an extramedullary location.
10
Goto, Kazuya, Hiroko Fujii, Gen Honjo, and Satoshi Kore-eda. "GFAP-Negative Subcutaneous Sacrococcygeal Extraspinal Ependymoma." Case Reports in Dermatology 13, no. 2 (June 14, 2021): 293–97. http://dx.doi.org/10.1159/000516618.
Анотація:
Ependymomas are slowly growing glial tumors derived from the ependymal cells and usually occur in the central nervous system (CNS). Ependymomas rarely occur outside of the CNS and they are called extraspinal ependymomas. In spite of their metastatic potential, extraspinal ependymomas can be misdiagnosed for other benign mass like pilonidal cysts. The diagnosis is confirmed by histopathology and most of the cases are known to show glial fibrillary acidic protein (GFAP), S-100 protein, and keratin (AE1AE3) immunoreactivity. Herein, we present a case of GFAP-negative ependymoma, which presented as asymptomatic subcutaneous tumor of the left buttock and was clinically misdiagnosed as epidermal cyst. Our case indicates that ependymomas cannot be ruled out by lack of GFAP immunoreactivity and an asymptomatic subcutaneous mass could be a malignant tumor like ependymomas, which requires careful examinations.
11
Gavrilov, A. G., D. M. Chelushkin, Ya A. Latyshev, M. V. Ryzhova, and T. N. Panina. "A rare case of the supratentorial extraventricular anaplastic ependymoma. Case report and literature review." Russian journal of neurosurgery 24, no. 2 (June 12, 2022): 54–61. http://dx.doi.org/10.17650/1683-3295-2022-24-2-54-61.
Анотація:
Ependymoma is a brain tumor accounting for 1.9 % of all benign brain tumors and 3.1 % of glial tumors and 2–9 % of all neuroepithelial tumors. Approximately one third of intracranial ependymal tumors are supratentorial. They may be attached to the ependymal walls of the III ventricle and lateral ventricles or may be sited in the white matter without direct connection to the ventricular system pressing the adjacent cortex. In very rare cases ependymomas may lie cortically with blood supply from the dura – so-called «cortical» ependymomas.Posterior fossa ependymomas are more common in pediatric population with mean age of 6 years, whereas supratentorial ependymomas manifest in adults. In adults Grade III anaplastic ependymomas are most commonly seen.These tumors have no specific features and clinical manifestations of extraventricular anaplastic ependymomas may vary greatly depending on localization and size of the tumor.Supratentorial anaplastic ependymomas have no specific neuroimaging features either. Tumors are often hypo- and isointense in T1 and iso- or hyperintense in T2 sequence, Gd -inhancement is variable.Ependymomas are considered to be non-invasive and to have a strict border with brain tissue thus leading to clinical manifestations because of mass-effect.Surgical resection is the main treatment option for ependymoma. Patients with local Grade II tumor recurrence and patients with Grade III ependymomas should under go radiotherapy on the tumor r esection cavity.We present a rare case of a supratentorial extraventricular anaplastic ependymoma. A 21‑year-old female presented to the clinic with paresthesia and numbness in right hand, right half of lower lip and astereognosis. The magnetic resonance imaging of the brain showed a mass lesion in left fronto-parietal region. At 10.10.2017 surgery was performed and tumor was resected. Histological findings matched with features of anaplastic ependimoma, WHO Grade III. There was no tumor progression during 3 months after surgery.
12
Wagemakers, Michiel, Mariska Sie, Eelco W. Hoving, Grietje Molema, Eveline S. J. M. de Bont, and Wilfred F. A. den Dunnen. "Tumor vessel biology in pediatric intracranial ependymoma." Journal of Neurosurgery: Pediatrics 5, no. 4 (April 2010): 335–41. http://dx.doi.org/10.3171/2009.11.peds09260.
Анотація:
Object This study aimed to characterize the pediatric intracranial ependymoma vasculature in terms of angiogenic activity and maturation status so as to provide indications for the applicability of vessel-targeted therapy in cases of pediatric intracranial ependymoma. Methods Tumor samples obtained in patients with ependymomas were immunohistochemically (double) stained for Ki 67/CD34, caspase 3a/CD34, vascular endothelial growth factor (VEGF)–A, –B, –C, –D, collagen Type IV, and smooth muscle actin to determine microvessel density, tumor and endothelial cell proliferation and apoptotic fraction, the relative expression of VEGF family members, and the coverage of the tumor endothelial cells by basal membrane and pericytes. Messenger RNA expression of angiopoietin-1 and -2 was analyzed by real-time reverse transcriptase polymerase chain reaction. These data were compared with those obtained in a glioblastoma series. Results Despite a low endothelial cell turnover, the microvessel density of ependymomas was similar to that of glioblastomas. In ependymomas the expression of VEGF-A was within the range of the variable expression in glioblastomas. The staining intensities of VEGF-B, -C, and -D in ependymomas were significantly lower (p < 0.001). The expression of angiopoietin-1 was higher in ependymomas than in glioblastomas (p = 0.03), whereas angiopoietin-2 expression was similar. The coverage of tumor endothelial cells with basal membrane and pericytes was more complete in ependymomas (p = 0.009 and p = 0.022, respectively). Conclusions The ependymoma vasculature is relatively mature and has little angiogenic activity compared with malignant gliomas. Therefore, the window for vessel normalization as a therapeutic aim might be considered small. However, the status of the tumor vasculature may not be a reliable predictor of treatment effect. Therefore, possible benefits of antiangiogenic treatment cannot be excluded beforehand in patients with ependymomas.
13
Thomas, Christian, Felix Thierfelder, Malte Träger, Patrick Soschinski, Michael Müther, Dominic Edelmann, Alexandra Förster, et al. "TERT promoter mutation and chromosome 6 loss define a high-risk subtype of ependymoma evolving from posterior fossa subependymoma." Acta Neuropathologica 141, no. 6 (March 23, 2021): 959–70. http://dx.doi.org/10.1007/s00401-021-02300-8.
Анотація:
AbstractSubependymomas are benign tumors characteristically encountered in the posterior fossa of adults that show distinct epigenetic profiles assigned to the molecular group “subependymoma, posterior fossa” (PFSE) of the recently established DNA methylation-based classification of central nervous system tumors. In contrast, most posterior fossa ependymomas exhibit a more aggressive biological behavior and are allocated to the molecular subgroups PFA or PFB. A subset of ependymomas shows epigenetic similarities with subependymomas, but the precise biology of these tumors and their potential relationships remain unknown. We therefore set out to characterize epigenetic traits, mutational profiles, and clinical outcomes of 50 posterior fossa ependymal tumors of the PFSE group. On histo-morphology, these tumors comprised 12 ependymomas, 14 subependymomas and 24 tumors with mixed ependymoma–subependymoma morphology. Mixed ependymoma–subependymoma tumors varied in their extent of ependymoma differentiation (2–95%) but consistently exhibited global epigenetic profiles of the PFSE group. Selective methylome analysis of microdissected tumor components revealed CpG signatures in mixed tumors that coalesce with their pure counterparts. Loss of chr6 (20/50 cases), as well as TERT mutations (21/50 cases), were frequent events enriched in tumors with pure ependymoma morphology (p < 0.001) and confined to areas with ependymoma differentiation in mixed tumors. Clinically, pure ependymoma phenotype, chr6 loss, and TERT mutations were associated with shorter progression-free survival (each p < 0.001). In conclusion, our results suggest that subependymomas may acquire genetic and epigenetic changes throughout tumor evolution giving rise to subclones with ependymoma morphology (resulting in mixed tumors) that eventually overpopulate the subependymoma component (pure PFSE ependymomas).
14
H, Bekralas, Bouallag M, and Habchi N. "Extradural Ependymoma Mimicking A Schwannoma: Case Report and Review of the Literature." Neuroscience and Neurological Surgery 4, no. 1 (June 12, 2019): 01–02. http://dx.doi.org/10.31579/2578-8868/048.
Анотація:
Intracranial ependymomas are tumors derived from ependymal cells. In addition to the problems of excision posed by their location, intracranial ependymomas have a prognosis that is difficult to define because of the risk of recurrence or neurological dessimination at a distance from the initial focus. We report a clinical case of a patient operated in 2009 of an ependymoma grade II in right temporal, reoperated in 2014 for a grade III anaplastic ependymoma in the sacral region. This case has been studied in order to draw attention to this particular secondary localization of intracranial ependymoma.
15
Gembruch, Oliver, Mehdi Chihi, Merle Haarmann, Ahmet Parlak, Marvin Darkwah Oppong, Laurèl Rauschenbach, Anna Michel, et al. "Surgical outcome and prognostic factors in spinal cord ependymoma: a single-center, long-term follow-up study." Therapeutic Advances in Neurological Disorders 14 (January 2021): 175628642110556. http://dx.doi.org/10.1177/17562864211055694.
Анотація:
Objective: Spinal cord ependymomas account for 3–6% of all central nervous system tumors and around 60% of all intramedullary tumors. The aim of this study was to analyze the neurological outcome after surgery and to determine prognostic factors for functional outcome. Patients and Methods: Patients treated surgically due to a spinal cord ependymoma between 1990 and 2018 were retrospectively included. Demographics, neurological symptoms, radiological parameters, histopathology, and neurological outcome (using McCormick Score [MCS]) were analyzed. Possible prognostic factors for neurological outcome were evaluated. Results: In total, 148 patients were included (76 males, 51.4%). The mean age was 46.7 ± 15.3 years. The median follow-up period was 6.8 ± 5.4 years. The prevalence was mostly in the lumbar spine (45.9%), followed by the thoracic spine (28.4%) and cervical spine (25.7%). Gross-total resection was achieved in 129 patients (87.2%). The recurrence rate was 8.1% and depended on the extent of tumor resection ( p = 0.001). Postoperative temporary neurological deterioration was observed in 63.2% of patients with ependymomas of the cervical spine, 50.0% of patients with ependymomas of the thoracic spine, and 7.4% of patients with ependymomas of the lumbosacral region. MCS 1–2 was detected in nearly two-thirds of patients with cervical and thoracic spinal cord ependymoma 36 months after surgery. Neurological recovery was superior in thoracic spine ependymomas compared with cervical spine ependymomas. Poor preoperative functional condition (MCS >2), cervical and thoracic spine location, and tumor extension >2 vertebrae were independent predictors of poor neurological outcome. Conclusion: Neurological deterioration was seen in the majority of cervical and thoracic spine ependymomas. Postoperative improvement was less in thoracic cervical spine ependymomas compared with thoracic spine ependymomas. Poor preoperative status and especially tumor extension >2 vertebrae are predictors of poor neurological outcome (MCS >2).
16
Little, Nicholas S., Michael K. Morgan, and Robert P. Eckstein. "Primary ependymoma of a cranial nerve." Journal of Neurosurgery 81, no. 5 (November 1994): 792–94. http://dx.doi.org/10.3171/jns.1994.81.5.0792.
Анотація:
✓ Ependymomas arising from peripheral or spinal nerves are extremely rare. Other ectopic ependymomas are found mostly in the sacrococcygeal region, possibly in association with developmental anomalies. The case of an ependymoma arising from a cranial nerve in a 40-year-old man is described.
17
Alkhaibary, Ali, Fahd AlSufiani, Ali H. Alassiri, Makki Almuntashri, and Salma Tarik Al Qutub. "Chondro-Osseous Metaplasia in Ependymoma: A Rare Histopathological Finding." Case Reports in Pathology 2020 (May 5, 2020): 1–6. http://dx.doi.org/10.1155/2020/1528698.
Анотація:
Ependymoma is a circumscribed glioma composed of uniform glial cells with bland nuclei in a fibrillary matrix. It is characterized by the presence of perivascular pseudorosettes. Unusual histopathological findings have rarely been reported in ependymomas, 0.5% of all diagnosed cases. Such unusual and exceedingly rare histological findings include osseous or chondroid metaplasia. To the best of our knowledge, only 15 cases of osseocartilaginous ependymomas have been reported in English literature. We report a 3-year-old boy who presented with ataxia, vomiting, and headache for three months. Radiological imaging revealed a posterior fossa lesion. Histopathological examination of the lesion confirmed a posterior fossa ependymoma with chondro-osseous metaplasia. The present case outlines the clinical presentation, histopathological findings, and outcome of chondro-osseous metaplasia in ependymomas. To date, the etiology of chondro-osseous metaplasia in ependymomas remains uncertain. Further research exploring such phenomenon is of paramount importance to explain how these tumors develop.
18
Bertrand, Kelsey. "EPEN-32. Leveraging cell surface targets as therapeutic vulnerabilities for pediatric ependymoma." Neuro-Oncology 24, Supplement_1 (June 1, 2022): i46. http://dx.doi.org/10.1093/neuonc/noac079.168.
Анотація:
Abstract Brain tumors are the leading cause of cancer-associated death in children. Ependymoma, an aggressive type of childhood brain tumor, is currently treated with surgery and radiotherapy. Ependymomas are a molecularly heterogeneous group of tumors driven by distinct genetic and epigenetic alterations. In children, 90% of ependymomas arise intracranially, with two thirds occurring in the posterior fossa (PF) and one third in the supratentorial brain (ST). PF ependymomas are divided into at least two groups termed, PFA and PFB, with PFA tumors associated with poor clinical outcomes. Over 70% of ST ependymoma are characterized by an oncogenic fusion between ZFTA and RELA and shown in some cohorts to have poor clinical outcome, particularly in the context of CDKN2A tumor suppressor gene loss. A major challenge in identifying therapies against ependymoma, has been the paucity of genetic abnormalities available for targeting. PFA ependymomas harbor largely balanced genomes with no recurrent CNVs, fusions, or somatic mutations that are amenable to pharmacologic inhibition. ZFTA-RELA ependymoma while representing a clear disease driver, functions as a transcription factor and lacks clear binding surfaces available for direct inhibition using small molecules. Therefore, alternative approaches are needed to identify new targets and effective therapies in ependymoma to be evaluated in pre-clinical models. In both human ependymoma cell culture lines and PDX models, we demonstrate that a multi-omic approach is promising for cell surface target discovery, and further, focused cell surface profiling can identify lead targets that can be rapidly translated for CAR T-cell therapy.
19
Whittemore, Darren E., Robert E. Grondahl, and Kondi Wong. "Primary Extraneural Myxopapillary Ependymoma of the Broad Ligament." Archives of Pathology & Laboratory Medicine 129, no. 10 (October 1, 2005): 1338–42. http://dx.doi.org/10.5858/2005-129-1338-pemeot.
Анотація:
Abstract Primary extraneural ependymomas are rare tumors that arise in ectopic sites, including pulmonary, sacrococcygeal region, ovarian, and paraovarian tissues. Four such ependymomas reported in the literature involve the paraovarian tissues, including 2 broad ligament ependymomas. Here we describe a myxopapillary ependymoma of the broad ligament in a 22-year-old woman, which may be the first tumor of this type to be reported in this location. Cytology, histology, cytochemistry, immunohistochemistry, and flow cytometry ploidy analysis are studied and described. Identification of perivascular ependymal rosettes, ependymal canals, vimentin and glial fibrillary acidic protein immunoreactivity, cytochemical staining of blepharoplasts or terminal bars by phosphotungstic acid hematoxylin, and presence of multiple foci of myxoid degeneration among the ependymal rosettes characterized a myxopapillary ependymoma.
20
Naruse, Takahiro, Yukihiro Matsuyama, and Naoki Ishiguro. "Cyclooxygenase-2 expression in ependymoma of the spinal cord." Journal of Neurosurgery: Spine 6, no. 3 (March 2007): 240–46. http://dx.doi.org/10.3171/spi.2007.6.3.240.
Анотація:
Object Cyclooxygenase-2 (COX-2), also known as prostaglandin endoperoxide synthase, has been reported to play an important role in the tumorigenicity of many types of tumors. The expression of COX-2 in spinal ependymomas, however, has not been studied. The authors evaluated COX-2 expression in ependymoma of the spinal cord. Methods Sixteen ependymoma samples obtained in patients undergoing surgery between 1995 and 2004 were utilized for immunohistochemical studies to evaluate COX-2 and vascular endothelial growth factor (VEGF) expression. Intratumoral microvessels were also stained immunohistochemically using anti–human von Willebrand factor antibody and were quantified to determine the microvessel density (MVD). The clinical features were reviewed and recorded and the association with COX-2 expression was assessed. Seven (43.8%) of the 16 ependymoma specimens expressed COX-2. All three of the myxopapillary-type ependymomas exhibited COX-2–positive staining. Excluding the three myxopapillary-type cases, COX-2 expression was identified in four (30.8%) of 13 cellular-type ependymomas. The COX-2–positive samples exhibited a significant increase in VEGF-positive staining cells and MVD compared with COX-2-negative samples. The clinical features were not associated with COX-2 expression. Conclusions The results of the present study indicate that COX-2 expression may promote angiogenesis through VEGF expression in ependymomas of the spinal cord. It is suggested that the use of selective COX-2 inhibitors may provide a new therapeutic strategy for spinal cord ependymomas due to their inhibition of the COX-2-mediated angiogenesis.
21
Thammaroj, Jureerat, Amnat Kitkhandee, Parinyaporn Tumkot, Pichayen Duangtongpol, and Sakda Waraosawapati. "Magnetic Resonance Imaging Features of Intramedullary Spinal Cord Tumors with Pathological Correlations." ASEAN Journal of Radiology 19, no. 1 (April 29, 2013): 51–66. http://dx.doi.org/10.46475/aseanjr.v19i1.22.
Анотація:
Objective: The purpose of this study was to determine characteristic imaging findings of intramedullary
spinal cord tumor in magnetic resonance imaging (MRI).
Material and Methods: We retrospectively analyzed MRI in 15 patients with histologicaly proven intramedullary spinal cord tumors. The demographic data, MRI findings with histological findings were
recorded in terms of age, location, length, morphology, signal intensity, the presence or absence of cyst
and hemorrhage, enhancement pattern, other associated findings, necrosis, vascular proliferation and
WHO grading.
Results: Among the 15 patients, spinal cord ependymomas were eccentric 75%, well-define border 62.5% and cervicothoracic spine located 37.5%. Spinal cord astrocytomas were eccentrically located and ill-define border 85.7%, cervicothoracic and thoracic spine located 28.5%. A cystic component was seen in 87.5% of spinal cord ependymoma and 71.5% of astrocytomas. Intratumoral hemorrhage occurred in 75% of spinal cord ependymomas, and 57.1% of astrocytomas. In 12.5% of spinal cord ependymomas, a curvilinear low T2 signal, suggesting marginal hemorrhage, was seen at the upper and/or lower margins of the tumors. Twenty-five percent of spinal cord ependymoma and 57.2% of astrocytomas showed heterogeneous enhancement, while in 12.5% of spinal cord ependymomas, enhancement was homogeneous.
Conclusion: Although no statistically significant characteristic MRI feature to distinguish between ependymoma and astrocytoma is detected. By percentage we found that border, length and signal intensity of tumors may help diagnosis. With pathological correlation, all of spinal cord ependymomas are mark hypervascular tumor, but astrocytomas never showed.
22
Cooper, Patrick B., Matthew Katus, Leon Moores, Dennis Geyer, James G. Smirniotopoulos, Glenn D. Sandberg, and Elisabeth J. Rushing. "Rare giant cell ependymoma in an octogenarian." Journal of Neurosurgery 105, no. 6 (December 2006): 908–11. http://dx.doi.org/10.3171/jns.2006.105.6.908.
Анотація:
✓Ependymomas are glial tumors that occur most often in children. In adults, ependymomas most often appear in the spinal cord. The World Health Organization recognizes several rare ependymoma subtypes, including the giant cell ependymoma of the terminal filum. The authors describe an unusual case of a posterior fossa giant cell ependymoma in an 89-year-old man presenting with vertigo and disequilibrium. Only seven cases of this tumor have been reported in the literature to date. The authors discuss the clinical presentation, radiological findings, pathological considerations, and surgical intervention in this patient and review the relevant literature.
23
Fernández-de Thomas, Ricardo J., Natalie Amaral-Nieves, Orlando De Jesus, and Emil A. Pastrana. "Rare sacral extradural grade II ependymoma: a comprehensive review of literature." BMJ Case Reports 14, no. 11 (November 2021): e246540. http://dx.doi.org/10.1136/bcr-2021-246540.
Анотація:
Sacral spinal cord ependymoma is an uncommon pathology. Most of the reported cases are consistent with a myxopapillary ependymoma histopathologic subtype. Non-myxopapillary ependymomas rarely occur in the sacral region. Most lesions are intradural; however, rare extradural cases can occur. We present the case of a 46-year-old female patient diagnosed with a grade II sacral extradural ependymoma, emphasising the importance of an interdepartmental case approach for diagnosis and management. Even though grade II ependymomas are considered low grade, the potential for recurrence and metastatic disease has been reported. There are no treatment guidelines for these rare tumours besides gross total resection.
24
Engertsberger, Lara, Martin Benesch, Martin Mynarek, Svenja Tonn, Martina Stickan-Verfürth, Angela Funk, Kristian W. Pajtler, et al. "EPEN-19. Impact of molecular classification on prognosis in children and adolescents with spinal ependymoma: Results from the HIT-MED database." Neuro-Oncology 24, Supplement_1 (June 1, 2022): i42—i43. http://dx.doi.org/10.1093/neuonc/noac079.156.
Анотація:
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 <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.
25
Rossetti, Diana Valeria, Luca Massimi, Claudia Martelli, Federica Vincenzoni, Susanna Di Silvestre, Gianluca Scorpio, Gianpiero Tamburrini, Massimo Caldarelli, Andrea Urbani, and Claudia Desiderio. "Ependymoma Pediatric Brain Tumor Protein Fingerprinting by Integrated Mass Spectrometry Platforms: A Pilot Investigation." Cancers 12, no. 3 (March 13, 2020): 674. http://dx.doi.org/10.3390/cancers12030674.
Анотація:
Ependymoma pediatric brain tumor occurs at approximate frequencies of 10–15% in supratentorial and 20–30% in posterior fossa regions. These tumors have an almost selective response to surgery and relative and confirmed resistance to radiotherapy and chemotherapic agents, respectively. Alongside histopathological grading, clinical and treatment evaluation of ependymomas currently consider the tumor localization and the genomic outlined associated molecular subgroups, with the supratentorial and the posterior fossa ependymomas nowadays considered diverse diseases. On these grounds and in trying to better understand the molecular features of these tumors, the present investigation aimed to originally investigate the proteomic profile of pediatric ependymoma tissues of different grade and localization by mass spectrometry platforms to disclose potential distinct protein phenotypes. To this purpose, acid-soluble and acid-insoluble fractions of ependymoma tumor tissues homogenates were analyzed by LC-MS following both the top-down and the shotgun proteomic approaches, respectively, to either investigate the intact proteome or its digested form. The two approaches were complementary in profiling the ependymoma tumor tissues and showed distinguished profiles for supratentorial and posterior fossa ependymomas and for WHO II and III tumor grades. Top-down proteomic analysis revealed statistically significant higher levels of thymosin beta 4, 10 kDa heat shock protein, non-histone chromosomal protein HMG-17, and mono-/uncitrullinated forms ratio of the glial fibrillary acidic protein (GFAP) fragment 388–432 in supratentorial ependymomas—the same GFAP fragment as well as the hemoglobin alpha- and the beta-chain marked grade II with respect to grade III posterior fossa ependymomas. Gene ontology classification of shotgun data of the identified cancer and the non-cancer related proteins disclosed protein elements exclusively marking tumor localization and pathways that were selectively overrepresented. These results, although preliminary, seem consistent with different protein profiles of ependymomas of diverse grade of aggressiveness and brain region development and contributed to enlarging the molecular knowledge of this still enigmatic tumor.
26
Tuman, Jaylin, Quinton Anderson, Beau Hsia, Xinxin Wu, and Peter T. Silberstein. "Difference in presentation of pediatric and adult ependymoma: An NCDB analysis." Journal of Clinical Oncology 41, no. 16_suppl (June 1, 2023): e14055-e14055. http://dx.doi.org/10.1200/jco.2023.41.16_suppl.e14055.
Анотація:
e14055 Background: Ependymomas originate from ependymal cells and account for 6.2% of primary brain tumors in the pediatric population. Adult and pediatric ependymomas are identical in histology, but present in different sites, with intracranial ependymomas seen in children and spinal ependymomas more frequent in adults. The unique presentation of ependymoma in children warrants exploration separate from adults; however, there is currently limited data providing a baseline understanding of pediatric ependymoma and those that it impacts. The National Cancer Database (NCDB) was analyzed to identify common presentations and demographic characteristics of ependymoma in children. Methods: Patients aged 18 and younger with a diagnosis of Ependymoma (ICD 9391-9394) were identified utilizing the National Cancer Database (NCDB) from 2004 – 2020 for a retrospective cohort analysis (N = 2,098). Demographic factors such as age, sex, race, ethnicity, primary site, insurance status, and surgical procedure were analyzed by descriptive statistics. Additionally, identical NCDB data was identified for patients over 18 years old for use as a comparison factor (N = 8,227). Results: A total of 2,098 pediatric patients were identified with an average age of diagnosis of 7.0 years (SD = 5.609, range = ( < 1) - 18 years) with a higher percentage being male (55.9%) than female (44.1%). The vast majority of cases of pediatric ependymoma originate in the brain (91.2%), followed by occurrences on the spinal cord and cranial nerves (8.8%). This differs from ependymoma in adults where only 44.8% of cases occur in the brain and 55.2% occur in the spinal cord. Of the 2,098 pediatric patients identified, they were histologically identified as either Ependymoma, NOS (51.5%), or Anaplastic Ependymoma (47.5%). Comparatively, Anaplastic Ependymoma makes up only 9.7% of ependymomas in adult populations. Additionally, the majority of patients were Non-Hispanic (76.3%) and white (76.9%). Most patients were privately insured (58.0%) followed by those insured by Medicaid (33.1%). Pediatric ependymoma is primarily treated with surgical resection (95.7%). Conclusions: Compared to adult occurrences, pediatric cases of ependymoma are not only unique in the site that they affect but also in histology, with a higher proportion of anaplastic ependymoma. This NCDB analysis is the first to specifically analyze pediatric ependymoma and fills a gap in our understanding of how this cancer presents in children. The majority of ependymoma patients are non-Hispanic and White with a primary site occurring in the brain rather than in the spinal cord, consistent with literature. These findings emphasize the importance of a deeper understanding of the nature of this disorder. Future research should further explore the impact of demographic and socioeconomic factors on the treatments utilized, and overall survival of pediatric patients with ependymoma.
27
Zheng, Haiyin, Katherina Baranova, Jun Song, Lei Yan, Saumik Biswas, Subrata Chakrabarti, and Qi Zhang. "Overexpression of Long Noncoding RNA HOTAIR Is a Unique Epigenetic Characteristic of Myxopapillary Ependymoma." Journal of Neuropathology & Experimental Neurology 79, no. 11 (October 5, 2020): 1193–202. http://dx.doi.org/10.1093/jnen/nlaa103.
Анотація:
Abstract 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.
28
Swanson, Amy A., Aditya Raghunathan, Robert B. Jenkins, Martina Messing-Jünger, Torsten Pietsch, Michelle J. Clarke, Timothy J. Kaufmann, and Caterina Giannini. "Spinal Cord Ependymomas With MYCN Amplification Show Aggressive Clinical Behavior." Journal of Neuropathology & Experimental Neurology 78, no. 9 (July 4, 2019): 791–97. http://dx.doi.org/10.1093/jnen/nlz064.
Анотація:
Abstract Adult spinal cord ependymomas are typically low grade and have a relatively favorable clinical course following gross total resection. We report 4 cases of anaplastic spinal cord ependymoma with MYCN amplification, an exceptionally rare finding. All cases occurred in the spinal cord of adolescent and young adult women and had morphological and immunohistochemical features of anaplastic ependymomas (World Health Organization grade III). Chromosomal microarray analysis demonstrated amplification of 2p24 (including MYCN) in all cases. One patient died 6 months after surgery. Another patient recently had removal of metastatic nodules in the thoracic region, following gross total resection and adjuvant radiation therapy of a lumbar ependymoma 1 year previously. One patient responded well after chemotherapy but died after multiple relapses 82 months after diagnosis. We found MYCN amplification reported in 2 other ependymomas, both anaplastic and arising in the spinal cord of adult females (Brain Pathol 2001;11:133–43). One patient had multiple recurrences in the spinal cord and an intracranial metastasis. Although MYCN amplification is rare in ependymomas, the current and previously reported cases suggest that this is associated with higher-grade histology, spinal location, and often unfavorable prognosis. The clinical significance and therapeutic implications of MYCN amplification in ependymomas require further evaluation.
29
Lombardi, Giuseppe, Alessandro Della Puppa, Marco Pizzi, Giulia Cerretti, Camilla Bonaudo, Marina Paola Gardiman, Angelo Dipasquale, et al. "An Overview of Intracranial Ependymomas in Adults." Cancers 13, no. 23 (December 5, 2021): 6128. http://dx.doi.org/10.3390/cancers13236128.
Анотація:
Ependymomas are rare primary central nervous system tumors. They can form anywhere along the neuraxis, but in adults, these tumors predominantly occur in the spine and less frequently intracranially. Ependymal tumors represent a heterogenous group of gliomas, and the WHO 2016 classification is based essentially on a grading system, with ependymomas classified as grade I, II (classic), or III (anaplastic). In adults, surgery is the primary initial treatment, while radiotherapy is employed as an adjuvant treatment in some cases of grade II and in all cases of anaplastic ependymoma; chemotherapy is reserved for recurrent cases. In recent years, important and interesting advances in the molecular characterization of ependymomas have been made, allowing for the identification of nine molecular subgroups of ependymal tumors and moving toward subgroup-specific patients with improved risk stratification for treatment-decisions and future prospective trials. New targeted agents or immunotherapies for ependymoma patients are being explored for recurrent disease. This review summarizes recent molecular advances in the diagnosis and treatment of intracranial ependymomas including surgery, radiation therapy and systemic therapies.
30
Michealraj, Antony, Sachin Kumar, Leo Kim, Jeremy Rich, and Michael Taylor. "TAMI-59. METABOLIC REGULATION OF THE EPIGENOME DRIVES LETHAL INFANTILE EPENDYMOMA." Neuro-Oncology 22, Supplement_2 (November 2020): ii226. http://dx.doi.org/10.1093/neuonc/noaa215.946.
Анотація:
Abstract Ependymomas are malignant glial tumors that occur throughout the central nervous system. Of the nine distinct molecular types of ependymoma, Posterior Fossa A(PFA) ependymomas, found in the hindbrain of infants and young children, are the most prevalent type. They rarely harbor recurrent somatic single nucleotide or copy number aberrations, but rather display epigenetic dysregulations. Transcriptional analyses show that PFAs, but not other molecular ependymoma variants, have elevated hypoxic signaling. We hypothesized that the metabolic environment of the developing human fetal hindbrain contributes to PFA ependymomas through the intermediary metabolic mechanism. Hypoxia microenvironment is essential for PFA survival and upholding their epigenetic dysregulation. Hypoxia blocks methyltransferase activity by upregulating EZHIPs expression and restricting SAM abundance. Fine-tuning the abundance of a-KG and acetyl-CoA, hypoxia fuels demethylase, and acetyltransferase activity which collectively resulting in H3K27 hypomethylation and hyperacetylation. Genome-wide essentially screen further underscore that minimal basal level of H3K27me3 is essential for PFA survival and further attenuation of H3K27me3 decreases the fitness of PFAs. PFA Ependymomas have a unique epigenome, suggesting a model in which they thrive in a narrow Goldilocks zone, with deviation to either increased or decreased H3K27me3 levels leading to diminished cellular fitness. PFAs are maintained under hypoxia, associated with restricted availability of specific metabolites to diminish histone methylation and increase histone demethylation and acetylation at H3K27. PFAs initiate from a cell lineage in the first trimester of human development that resides in restricted oxygen. Unlike other ependymomas, transient exposure of PFA cells to ambient oxygen induces irreversible cellular toxicity. PFA tumors exhibit a low basal level of H3K27me3 and, paradoxically, inhibition of H3K27 methylation specifically disrupts PFA growth. Microenvironmental regulation of PFA ependymoma epigenome appears to play a major role in tumorigenesis. Targeting metabolism and/or the epigenome presents a unique opportunity for rational therapy for infants with PFA ependymoma.
31
Kano, Hideyuki, Or Cohen-Inber, Jason P. Sheehan, David Mathieu, Yan-Hua Su, Hslu-Mei Wu, Rachel Jacobs, and L. Dade Lunsford. "224 Stereotactic Radiosurgery for Intracranial Ependymomas: An International Multicenter Study." Neurosurgery 64, CN_suppl_1 (August 24, 2017): 261. http://dx.doi.org/10.1093/neuros/nyx417.224.
Анотація:
Abstract INTRODUCTION Stereotactic radiosurgery (SRS) is a potentially important option for patients with intracranial ependymoma. We analyzed the outcomes of intracranial ependymoma patients who underwent SRS as a part of multimodality management. METHODS Four participating centers of the International Gamma Knife Research Foundation (IGKRF) identified 74 patients who underwent SRS for 95 intracranial ependymomas. The median patient age was 21 years (range, 1.8-80). All patients had previous surgical resection of their ependymomas, 64 had previous fractionated radiation therapy, and 26 had previous chemotherapy. Forty-three patients had low-grade ependymomas (53 tumors) and 31 patients had high-grade ependymomas (42 tumors). The median radiosurgery target volume was 3.7 cc (range, 0.03-36.8) and the median margin dose was 15 Gy (range, 10–22). RESULTS >At a median follow-up of 23 months after SRS (range, 3.3-220), 37 patients died. The overall survival after SRS was 77% at 1 year, 60% at 2 years, 52% at 3 years, and 45% at 5 years. The progression-free survival after SRS was 75% at 1 year, 65% at 2 years, 53% at 3 years, and 47% at 5 years. Factors associated with better PFS included low-grade ependymoma (P = 0.021) and higher margin dose (P = 0.013). The distant tumor relapse rate after SRS was 17% at 1 year, 26% at 2 years, 37% at 3 years, and 40% at 50 years. Symptomatic adverse radiation effects developed in 9 patients (12%). CONCLUSION SRS provides another management option for patients with intracranial ependymomas that have failed surgery and radiation therapy.
32
Elsharkawy, Alaa Eldin, Raid Abuamona, Markus Bergmann, Shadi Salem, Evariste Gafumbegete, and Ernst Röttger. "Cortical Anaplastic Ependymoma with Significant Desmoplasia: A Case Report and Literature Review." Case Reports in Oncological Medicine 2013 (2013): 1–6. http://dx.doi.org/10.1155/2013/354873.
Анотація:
Ectopic brain anaplastic ependymomas with no connection to the ventricles are rare. We present a rare case of a 25-year-old male who presented with generalized convulsions. Computed tomography (CT), Magnetic Resonance Imaging (MRI), and magnetic resonance spectroscopy (MRS) showed characters of an intra- and extra-axial lesion. Intraoperatively, the lesion was a cortical solid mass that had no connections to the dura or to the ventricle. The histological diagnosis showed an anaplastic ependymoma with WHO grade III with distinctive desmoplasia. A literature review of ectopic anaplastic ependymomas regarding their clinical presentations, management, and prognostic factors was performed. There is a need to establish a clinically based histopathological grading system for anaplastic ependymomas. Ectopic anaplastic ependymomas should be included in the preoperative differential diagnosis.
33
Lopez, Giselle, Roger E. McLendon, and Katherine B. Peters. "Supratentorial Tanycytic Ependymoma in an Adult Male: Case Report and Review of Literature." Case Reports in Oncology 8, no. 1 (March 12, 2015): 159–63. http://dx.doi.org/10.1159/000380906.
Анотація:
Ependymomas, tumors of the ependymal cells, are very rare and usually present in the pediatric population. Furthermore, there are even rarer variants of ependymomas that can include cellular, papillary, clear cell, and tanycytic subtypes. We present a case of a supratentorial tanycytic ependymoma in an adult male and review the literature in regard to this rare primary central nervous system neoplasm.
34
Kupp, Robert, and Richard J. Gilbertson. "PDTM-45. MECHANISM OF NEURAL STEM CELL TRANSFORMATION BY C11ORF95-RELA TRANSLOCATIONS." Neuro-Oncology 21, Supplement_6 (November 2019): vi197. http://dx.doi.org/10.1093/neuonc/noz175.820.
Анотація:
Abstract Highly recurrent chromosome translocations that produce oncogenic fusions are a prime example of practical therapeutic targets translating into clinical successes. We have previously described the genetic driver of supratentorial ependymomas as translocation fusions of the C11orf95 gene of unknown function and RELA, the canonical nuclear transducer of NF-κB signaling5. Subsequent studies have reinforced our original findings on the frequency (>70%) of these C11orf95-RELA (CR) fusions in human disease, as well as their transformative potential in murine models. However, the mechanisms by which CR fusions transform neural stem cells into ependymomas remain elusive. Using a multi-OMICS approach, we show that oncogenic C11orf95 fusions co-opt spatio-temporally regulated transcriptional activators in ependymoma. We henceforth demonstrate that supratentorial ependymomas, which have been previously described as a RELA fusion disease6, are truly a C11orf95 fusion disease, with C2H2-ZFs serving as a scaffold for transcriptional dysregulation. Our studies lay the architectural framework for a molecular roadmap into the heart of C11orf95 fusion ependymomas, elucidating the biochemical mechanisms which furnish the capacity of these fusions to operate as single gene oncogenes, and henceforth providing potential avenues for therapeutic intervention in ependymoma.
35
Zschernack, Valentina, Stephanie T. Jünger, Martin Mynarek, Stefan Rutkowski, Maria Luisa Garre, Martin Ebinger, Marie Neu, et al. "Supratentorial ependymoma in childhood: more than just RELA or YAP." Acta Neuropathologica 141, no. 3 (January 22, 2021): 455–66. http://dx.doi.org/10.1007/s00401-020-02260-5.
Анотація:
AbstractTwo distinct genetically defined entities of ependymoma arising in the supratentorial compartment are characterized by the presence of either a C11orf95-RELA or a YAP-MAMLD1 fusion, respectively. There is growing evidence that supratentorial ependymomas without these genetic features exist. In this study, we report on 18 pediatric non-RELA/non-YAP supratentorial ependymomas that were systematically characterized by means of their histology, immunophenotype, genetics, and epigenomics. Comprehensive molecular analyses included high-resolution copy number analysis, methylation profiling, analysis of fusion transcripts by Nanostring technology, and RNA sequencing. Based upon histological and immunohistochemical features two main patterns were identified—RELA-like (n = 9) and tanycytic ependymomas (n = 6). In the RELA-like group histologically assigned to WHO grade III and resembling RELA-fused ependymomas, tumors lacked nuclear expression of p65-RelA as a surrogate marker for a pathological activation of the NF-κB pathway. Three tumors showed alternative C11orf95 fusions to MAML2 or NCOA1. A methylation-based brain tumor classifier assigned two RELA-like tumors to the methylation class “EP, RELA-fusion”; the others demonstrated no significant similarity score. Of the tanycytic group, 5/6 tumors were assigned a WHO grade II. No gene fusions were detected. Methylation profiling did not show any association with an established methylation class. We additionally identified two astroblastoma-like tumors that both presented with chromothripsis of chromosome 22 but lacked MN1 breaks according to FISH analysis. They revealed novel fusion events involving genes in chromosome 22. One further tumor with polyploid cytogenetics was interpreted as PFB ependymoma by the brain tumor methylation classifier but had no relation to the posterior fossa. Clinical follow-up was available for 16/18 patients. Patients with tanycytic and astroblastoma-like tumors had no relapse, while 2 patients with RELA-like ependymomas died. Our data indicate that in addition to ependymomas discovered so far, at least two more supratentorial ependymoma types (RELA-like and tanycytic) exist.
36
Dulai, Mohanpal S., Dario V. Caccamo, Anita L. Briley, Michael S. B. Edwards, Paul G. Fisher, and Norman L. Lehman. "Intramedullary papillary ependymoma with choroid plexus differentiation and cerebrospinal fluid dissemination to the brain." Journal of Neurosurgery: Pediatrics 5, no. 5 (May 2010): 511–17. http://dx.doi.org/10.3171/2009.12.peds09130.
Анотація:
This 8-year-old girl presented with a papillary ependymoma in the thoracic spinal cord. Resection was followed by recurrence at the primary site and later in the lumbosacral thecal sac, followed by cerebrospinal fluid dissemination to the brain approximately 5 years after her initial presentation. The tumor showed cytological and immunohistochemical features overlapping those of classic ependymomas and choroid plexus tumors similar to those seen in uncommon supratentorial papillary ependymomas, also known as papillary tumors of the pineal region. The histopathological and clinical courses of this rare spinal papillary ependymoma exhibiting mixed ependymal and choroid plexus–like differentiation are discussed.
37
Tarapore, Phiroz E., Peter Modera, Agne Naujokas, Michael C. Oh, Beejal Amin, Tarik Tihan, Andrew T. Parsa, et al. "Pathology of Spinal Ependymomas." Neurosurgery 73, no. 2 (May 10, 2013): 247–55. http://dx.doi.org/10.1227/01.neu.0000430764.02973.78.
Анотація:
AbstractBACKGROUND:Ependymomas constitute approximately 40% of primary intraspinal tumors. Current World Health Organization (WHO) grading may not correlate with observed progression-free survival (PFS).OBJECTIVE:This retrospective study of prospectively collected data examines whether PFS is influenced by the histological grade or by the extent of resection. It also analyzes the usage and effectiveness of postoperative adjuvant radiotherapy.METHODS:We reviewed 134 consecutive patients with ependymomas of all grades. Pathology slides were re-reviewed and the histological grades were confirmed by a single neuropathologist. Postoperative residual or recurrence was evaluated with follow-up magnetic resonance imaging.RESULTS:There were 85 male and 49 female patients, ranging from 10 to 79 (median 41) years of age. Thirty patients had WHO grade I tumors, 101 had grade II tumors, and 3 had grade III tumors. Kaplan-Meier analysis of PFS demonstrated a mean duration of 6 years for grade I, 14.9 years for grade II, and 3.7 years for grade III (P < .001). In grade II ependymomas, mean PFS was 11.2 years with subtotal resection and 17.8 years with gross total resection (P < .01). PFS of patients who underwent subtotal resection was not significantly changed by adjuvant radiotherapy (P < .36).CONCLUSION:Patients with grade II ependymoma have significantly longer PFS than patients with grade I ependymoma. The extent of resection did not affect PFS in grade I ependymoma but it did in grade II. Contrary to its higher grade, WHO grade II ependymoma carries a better prognosis than WHO grade I ependymoma.
38
Helseth, Are, and Sverre J. Mørk. "Primary intraspinal neoplasms in Norway, 1955 to 1986." Journal of Neurosurgery 71, no. 6 (December 1989): 842–45. http://dx.doi.org/10.3171/jns.1989.71.6.0842.
Анотація:
✓ A survey of all patients (173 males and 294 females) registered with primary intraspinal neoplasms in the Norwegian Cancer Registry from 1955 through 1986 is presented. Annual age-adjusted incidence rates of new tumors per one million population were three for males and five for females. Altogether, 89% of the tumors were verified histologically. Meningioma was the most common tumor type, followed by ependymoma and neurilemoma. Intraspinal ependymomas accounted for 34.5% of all 223 ependymomas of the central nervous system, whereas only 0.2% of the 3046 glioblastomas were found intraspinally. Patients with intraspinal meningioma had a better life expectancy than those with intracranial meningioma. The 5-year relative survival rate for patients with intraspinal ependymoma was 88.9% in contrast to 24.4% for patients with intracranial ependymoma.
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Oh, Michael C., Eli T. Sayegh, Michael Safaee, Matthew Z. Sun, Gurvinder Kaur, Joseph M. Kim, Derick Aranda, Annette M. Molinaro, Nalin Gupta, and Andrew T. Parsa. "Prognosis by tumor location for pediatric spinal cord ependymomas." Journal of Neurosurgery: Pediatrics 11, no. 3 (March 2013): 282–88. http://dx.doi.org/10.3171/2012.11.peds12292.
Анотація:
Object Ependymoma is a common CNS tumor in children, with spinal cord ependymomas making up 13.1% of all ependymomas in this age group. The clinical features that affect prognosis in pediatric spinal cord ependymomas are not well understood. A comprehensive literature review was performed to determine whether a tumor location along the spinal cord is prognostically significant in children undergoing surgery for spinal cord ependymomas. Methods A PubMed search was performed to identify all papers that contained data on patients with spinal cord ependymomas. Only pediatric patients (age < 18 years) who underwent resection with a clearly reported tumor location were included in the analysis. Myxopapillary tumors were excluded from study. Tumor location was subdivided into 6 regions: cervicomedullary, cervical, cervicothoracic, thoracic, thoracolumbar, and conus medullaris. Kaplan-Meier survival and Cox regression analyses were performed to determine the effects of tumor location on progression-free survival (PFS) and overall survival (OS). Results Fifty-eight patients who underwent resection of spinal cord ependymomas were identified. Ependymomas were located all along the spinal cord but occurred with the highest frequency in the cervical region (29.3%). Progression-free survival was significantly better in patients with tumors arising in the upper portion of the spinal cord (p = 0.031), which remained significant in the multivariate Cox regression analysis (p < 0.05). Moreover, OS was significantly better in patients with upper spinal cord ependymomas than in those harboring ependymomas in the lower spinal cord (p = 0.048). Conclusions Although more common in adults, spinal ependymomas can occur anywhere along the spinal cord in the pediatric population; however, tumors occurring in the lower half of the spinal cord carry a worse prognosis with shorter PFS and OS. By comparison, ependymomas in the upper spinal cord recur later and less frequently, with little or no mortality in this patient group.
40
Mozaffari, Khashayar, Michael A. Stellon, Eric J. Chalif, and Michael K. Rosner. "Multifocal Intradural Extramedullary Anaplastic Ependymoma With Intracranial Involvement at Presentation: A Case Report." Iranian Journal of Neurosurgery 7, no. 3 (July 1, 2021): 159–64. http://dx.doi.org/10.32598/irjns.7.3.7.
Анотація:
Background and Importance: Ependymomas are a rare malignant neoplasm. Multifocal intradural extramedullary anaplastic ependymomas are even more of a rare entity with much of the current knowledge derived from case reports. We presented a case of a multifocal intradural extramedullary anaplastic ependymoma with intracranial involvement at presentation. Case Presentation: A 53-year-old male presented with urinary symptoms. Magnetic resonance imaging revealed two lesions along the spinal cord and two lesions, intracranially. Histopathological examination was consistent with the World Health Organization grade III anaplastic ependymoma. The patient was treated with the gross total resections of spinal cord lesions, followed by radiation therapy to the resection cavities and intracranial lesions. At the 10-month follow-up visit, he reported almost complete resolution of symptoms, and magnetic resonance imaging revealed no recurrence. Conclusion: Despite their rarity, ependymomas should be considered as the differential diagnosis when evaluating spinal tumors. Gross total resection followed by targeted radiotherapy appears to be an effective treatment modality for high-grade lesions.
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Dewan, Aditi, Ravindra Kumar Saran, Smriti Nagpal Gupta, Deepanjali Arya, and Ruchi Goel. "Intraocular Ependymoma With Blood-Filled Spaces: Neoplasm or a Reactive Process With Ependymal Differentiation—A Dilemma." International Journal of Surgical Pathology 25, no. 4 (February 14, 2017): 368–73. http://dx.doi.org/10.1177/1066896917692098.
Анотація:
Intraocular glial lesions are rare and include retinal gliosis, hamartomas, and astrocytomas and rarely ependymomas. Ependymomas are slow-growing glial tumors preferentially arising in the central nervous system (CNS), occasionally presenting at sites outside the CNS, with only 2 cases of primary retinal ependymoma reported till date. We report herein the third such case of a 20-year-old male who presented with a painful blind eye. The enucleated specimen showed presence of a glial tumor with cells arranged in sheets as well as few true rosettes and pseudo-rosettes and an immunohistochemical profile similar to a classical ependymoma at usual sites in the CNS. Additionally, the presence of blood-filled spaces and few proliferating blood vessels made it a diagnostic challenge. All retinal glial lesions are positive for GFAP and S100. Therefore, immunostaining for EMA as well as the MIB-1-labeling index maybe vital in differentiating ependymomas from other intraocular glial lesions.
42
Duc, Nguyen Minh, and Huynh Quang Huy. "Magnetic Resonance Imaging Features of Common Posterior Fossa Brain Tumors in Children: A Preliminary Vietnamese Study." Open Access Macedonian Journal of Medical Sciences 7, no. 15 (August 14, 2019): 2413–18. http://dx.doi.org/10.3889/oamjms.2019.635.
Анотація:
BACKGROUND: Magnetic Resonance Imaging (MRI) nowadays plays an important role in the evaluation of posterior fossa brain tumours in children for appropriate diagnosis, treatment planning, and follow-up.
AIM: To assess the MRI features of common posterior fossa brain tumours including medulloblastomas, ependymomas, and pilocytic astrocytomas along with the postoperative parameters to contribute the local knowledge to the neuroradiology and neurosurgery fields.
METHODS: The study was performed at Children's Hospital 02 from January 2016 to June 2019. In this study, all pediatric patients adopted MRI to evaluate the posterior fossa brain tumours’ characteristics and then underwent surgery to eradicate the posterior fossa tumours. We retrospectively compared the baseline parameters, MRI parameters, and postoperative parameters among medulloblastomas, ependymomas, and pilocytic astrocytomas.
RESULTS: There were 62 patients (27 medulloblastomas, 20 ependymomas, and 15 pilocytic astrocytomas) in this research. The main structure of medulloblastomas and ependymomas was predominantly solid, whereas the main structure of pilocytic astrocytomas was superiorly cystic (p < 0.05). Ependymoma tended to extend tumour through foramina of Luschka and Magendie (p < 0.05). Medulloblastomas chiefly showed iso intensity on T2W and FLAIR images meanwhile ependymomas and pilocytic astrocytomas predominantly appeared hyperintensity on T2W and FLAIR images. Medulloblastomas and ependymomas were mostly high intensity on DWI, and low intensity on ADC whereas pilocytic astrocytomas were usually low intensity on DWI and high intensity on ADC. After injecting CE, pilocytic astrocytomas showed a mixed intensity whereas the signal intensity of medulloblastoma and ependymoma on T1CE was generally strong. There were positive correlations between FH diameter and estimated blood loss (r = 0.289, p < 0.05); and surgical time (r = 0.312, p < 0.05).
CONCLUSION: MRI plays a crucial role in demonstrating the features of posterior fossa brain tumours for appropriate diagnosis of medulloblastomas, ependymomas, and pilocytic astrocytomas. Medulloblastomas are problematic tumours and the clinicians should also take into consideration in cases of larger feet-to-head diameter of tumours to ensure the efficacy and safety surgery for patients.
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Macêdo, Lívio Pereira de, Benjamim Pessoa Vale, Marx Lima de Barros Araújo, João Cícero Lima Vale, Yally Dayanne Oliveira Ferreira, and Suelen Maria Silva de Araújo. "Ependymoma with Intraorbital Extracerebral Recurrence: Case Report." Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 38, no. 04 (September 3, 2019): 342–47. http://dx.doi.org/10.1055/s-0039-1695761.
Анотація:
AbstractEpendymomas are rare neuroepithelial tumors that originate from a type of glial cell called ependymal cell. In general, they correspond to ∼ 1.2 to 7.8% of all intracranial neoplasms, and to ∼ 2 to 6% of all gliomas. Although it corresponds only to ∼2 to 3% of all primary brain tumors, ependymoma is the fourth most common cerebral neoplasm in children, especially in children younger than 3 years of age.1 2 In patients younger than 20 years of age, the majority (90%) of ependymomas are infratentorial, more precisely from the IV ventricle. In spite of this, in adults, medullary ependymomas are more frequent (60%). In this context, supratentorial and extraventricular ependymomas, as in the case reported in the present article, are infrequent in both adults and children.1 2 Both sexes are equally affected.3 Recurrence of intracranial ependymomas occurs in almost 50% of the cases, and the follow-up outcome is not favorable.4 In another perspective, the recurrence of extracerebral ependymomas is extremely rare, and even more unusual in the intraorbital site, as it occurred in the case in question.
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Boström, Azize, Marec von Lehe, Wolfgang Hartmann, Torsten Pietsch, Mareike Feuss, Jan P. Boström, Johannes Schramm, and Matthias Simon. "Surgery for Spinal Cord Ependymomas." Neurosurgery 68, no. 2 (February 1, 2011): 302–9. http://dx.doi.org/10.1227/neu.0b013e3182004c1e.
Анотація:
Abstract BACKGROUND: Spinal cord tumors account for 5% to 10% of all primary central nervous system tumors. The most common intramedullary neoplasms are ependymomas, composing 50% to 60% of spinal neuroepithelial tumors in adults. OBJECTIVE: To evaluate the clinical and oncological outcomes of patients with spinal ependymoma primarily treated with microsurgery. METHODS: Patient charts and operative notes were analyzed to evaluate the clinical and oncological outcomes of 57 patients (33 men, 24 women) undergoing surgery for spinal ependymal tumors between 1987 and 2007. Mean follow-up was 67 months (range, 1-195 months; median, 56 months). Histopathological findings were 1 subependymoma World Health Organization (WHO) grade I, 16 myxopapillary ependymomas WHO grade I, 39 ependymomas WHO grade II, and 1 anaplastic ependymoma WHO grade III. Histopathological diagnoses were reviewed in 52 cases (91%) using the 2007 WHO classification. RESULTS: There were 47 complete resections (83%). Only 4 patients (7%) underwent (postoperative) radiotherapy. Forty-nine of 57 patients (86%) had stable or improved McCormick grades directly after surgery. A permanent decrease in the McCormick grade was seen in 4 (7%) patients. Multivariate logistic regression revealed only the preoperative neurological status of the patient as an independent predictor of functional outcome (P = .007). Recurrent tumors were diagnosed 12 to 72 months after surgery in 5 of 57 patients (9%) including 3 of 16 myxopapillary ependymomas (19%). In 4 of 5 patients, the primary tumor was incompletely resected. The progression-free survival rate was 89% and 84% for all patients at 5 and 10 years, respectively. An incomplete resection proved the only independent predictor of progression-free survival (P = .05). CONCLUSION: These results support early surgery aiming at complete resection as the primary treatment for presumed spinal ependymomas. The prognosis after surgery for some myxopapillary ependymomas seems worse than generally believed.
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Yan, Xuetao, Xiaoli Cheng, Juyin Liu, Dongqin Luo, Xianghu He, Feng Chen, Chengming Qin, and Yanlin Wang. "Clinicopathological evaluation of immunohistochemical Ki-67 and endothelial nitric oxide synthase expression in intracranial ependymoma." Clinical & Investigative Medicine 31, no. 4 (August 1, 2008): 206. http://dx.doi.org/10.25011/cim.v31i4.4781.
Анотація:
Purpose: To analyze the association between Ki-67 and eNOS expression with the pathological grades of patients with intracranial ependymomas, and to determine its value in distinguishing the progression of the disease. Methods: A clinicopathological study was undertaken in 82 patients with intracranial ependymomas. Tissue samples, obtained by tumour resection, were divided into three groups: low-grade, mid-grade and high-grade ependymomas. Tissue samples obtained from 15 patients with brain contusion were used as control. Immuno-histochemical staining was performed to analyze the association between Ki-67 and eNOS expression with various tumour grades. The cell proliferating marker Ki-67 was assessed by positive cell count. The levels of eNOS positive expression were evaluated as slight, moderate and intense. Results: 48 of 82 cases (58.54%) expressed Ki-67 protein. Expression of Ki-67 and eNOS was negative in all control samples. Positive cell rates were 2.65±0.83 % in the low-grade, 9.63±0.08 % in the mid-grade, and 28.41±0.71 % in the high-grade ependymoma groups. In low-grade ependymomas there were 8 and 12 cases that expressed eNOS slightly or moderately. In the mid-grade ependymoma group eNOS was expressed moderately in 10 cases and intensely in 15. In the high-grade group 20 cases showed intense positive expression of eNOS. The Ki-67 positive cell counts for slight, moderate and intense eNOS expression were 2.20, 6.07 and 22.25, respectively. Conclusion: Ki-67 and eNOS expression in intracranial ependymoma tissue was associated with the histopathological grade and malignant degree.
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Dibs, Khaled, Rahul Neal Prasad, Kajal Madan, Kevin Liu, Will Jiang, Jayeeta Ghose, Dukagjin M. Blakaj, et al. "Cerebellopontine angle ependymoma presenting as isolated hearing loss in an elderly patient: A case report and literature review." Surgical Neurology International 12 (November 23, 2021): 572. http://dx.doi.org/10.25259/sni_781_2021.
Анотація:
Background: Ependymoma is an uncommon tumor accounting for approximately 1.9% of all adult central nervous system tumors. Ependymomas at the cerebellopontine angle (CPA) are even more rare and only previously described in isolated case reports. Typically, acoustic neuromas and meningiomas represent the bulk of adult CPA tumors. Diagnosis can be challenging, as ependymomas have clinical findings and imaging characteristics that overlap with more common tumor histologies at the CPA. Case Description: We present the case of a 70-year-old male patient with progressive, isolated left-sided hearing loss found to have a World Health Organization (WHO) Grade II CPA ependymoma, representing one of the oldest recorded patients presenting with this primarily pediatric malignancy in this unique location. The patient presentation with isolated hearing loss was particularly unusual. When associated with neurologic deficits, CPA ependymomas more characteristically result in facial nerve impairment with fully preserved hearing, while vestibular schwannomas tend to present with isolated hearing loss. The standard of care for pediatric ependymomas is maximal safe resection with adjuvant radiotherapy, but treatment paradigms in adult CPA ependymoma are not well defined particularly for WHO Grade II disease. After resection, he received adjuvant radiation to decrease the risk of local recurrence. Twenty-nine months after resection, the patient remains free of treatment-related toxicity or disease recurrence. Conclusion: We review this patient’s clinical course in the context of the literature to highlight the challenges associated with timely diagnosis of this rare tumor and the controversial role of adjuvant therapy in preventing local recurrence in these patients.
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Chan, Priya P., Nicholas S. Whipple, Arie Perry, David A. Solomon, Holly Zhou, Luke L. Linscott, John R. W. Kestle, and Carol Bruggers. "EPEN-07. PATTERNS OF EXTRANEURAL METASTASES IN PEDIATRIC SUPRATENTORIAL EPENDYMOMA: CASE SERIES AND REVIEW OF THE LITERATURE." Neuro-Oncology 22, Supplement_3 (December 1, 2020): iii309. http://dx.doi.org/10.1093/neuonc/noaa222.148.
Анотація:
Abstract BACKGROUND Ependymomas account for 10% of all malignant pediatric intracranial tumors. Standard therapy includes maximal safe surgical resection followed by involved-field radiation. Up to 50% of localized pediatric ependymomas recur. Extraneural metastases at time of recurrence are rarely reported. OBJECTIVE To describe extraneural metastases of pediatric ependymomas. METHODS Retrospective review of patients’ medical records and literature review. RESULTS Three patients with history of locally recurrent, supratentorial ependymoma developed extraneural metastases: one in a cervical lymph node, one with a scalp nodule, and one with a dural lesion. Each extraneural recurrence had similar histologic and molecular features as the initial diagnosis. The cervical lymph node recurrence was treated with multimodal therapy; she is without disease four years later. The isolated scalp nodule occurred at the exit site of a subgaleal drain placed during prior resection. Following nodule resection, he developed additional scalp and lymph node disease and is receiving palliative care. The isolated dural recurrence occurred at the exit site of a ventriculoperitoneal shunt placed following a previous resection. She died of progressive disease 18 months after dural lesion resection. Reports of lymph node, scalp, and dural metastases of ependymomas are exceedingly rare, and outcomes are poor. CONCLUSIONS Extraneural manifestations of ependymoma are rare. Regional seeding from prior surgical procedures may play a role in metastatic spread. Extraneural metastases should be considered in children previously treated for ependymoma who develop local findings even in the absence of CNS relapse. Salvage therapy with curative intent should be considered using a multimodal approach.
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Malbari, Fatema, Guillermo Aldave, Sherri B. Birchansky, Arnold C. Paulino, Dolores H. Lopez-Terrada, Carrie A. Mohila, Sibo Zhao, and Murali Chintagumpala. "Ependymoma Presenting as a Rim-Enhancing Lesion in the Brainstem." Pediatric Neurosurgery 56, no. 5 (2021): 455–59. http://dx.doi.org/10.1159/000516001.
Анотація:
<b><i>Introduction:</i></b> The posterior fossa is the most common intracranial location for pediatric ependymoma. While ependymoma usually arises from the ventricular lining of the fourth ventricle as a solid mass, it rarely originates from the brainstem. Grade II ependymomas also infrequently appear as a cavitary ring-enhancing lesion. <b><i>Case Presentation:</i></b> We describe a case of a 6-year-old boy with an ependymoma arising within the medulla with imaging features of a thick-walled rim-enhancing cavitary lesion. A stereotactic biopsy was obtained which confirmed a grade II ependymoma. The patient received focal proton beam radiation therapy and is doing well with no concerns for disease progression at 28 months after diagnosis. <b><i>Conclusion:</i></b> Posterior fossa ependymomas typically arise from ependymal cells within the fourth ventricle or foramina of Luschka. They rarely invade or arise within the brainstem parenchyma. Our case had atypical imaging findings in addition to the atypical tumor location. The lesion was described as a thick-walled rim-enhancing focal cystic necrotic lesion centered within the medulla with surrounding nonenhancing expansile infiltrative changes. Ring-enhancing lesions can be seen in patients with anaplastic ependymoma, but is not commonly reported in grade II ependymomas. In summary, this report highlights a unique case of a posterior fossa ependymoma in a pediatric patient arising in an atypical brainstem location as well as having unique imaging features.
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Heuer, Gregory G., Michael F. Stiefel, Robert L. Bailey, and James M. Schuster. "Acute paraparesis from hemorrhagic spinal ependymoma: diagnostic dilemma and surgical management." Journal of Neurosurgery: Spine 7, no. 6 (December 2007): 652–55. http://dx.doi.org/10.3171/spi-07/12/652.
Анотація:
✓Spinal ependymomas are a common type of primary spinal cord neoplasm that frequently occurs in the lumbar spine. The authors report on two patients who presented with acute neurological decline after hemorrhage into ependymomas of the filum terminale. Both were transferred to the authors' institution because of diagnostic uncertainty and a concern about possible intradural vascular abnormalities. Both patients underwent lumbar laminectomies for tumor resection. The pathological finding in each case was myxopapillary ependymoma. Both patients made a significant recovery and were ambulatory and continent at follow-up review. These cases illustrate the rare but clinically significant incidence of acute neurological decline caused by hemorrhagic cauda equina ependymomas, including the potential for delayed diagnosis and treatment.
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Da Costa, Marcos Devanir Silva, Carolina Torres Soares, Patricia Alessandra Dastoli, Jardel Mendonça Nicácio, Frederico Adolfo Benevides Silva, Maria Teresa de Seixas Alves, Michael Jenwei Chen, Andrea Maria Cappellano, Nasjla Saba Silva, and Sergio Cavalheiro. "Posterior fossa ependymomas in children and adolescents - the state of the art." Archives of Pediatric Neurosurgery 5, no. 3 (September 13, 2023): e20102023. http://dx.doi.org/10.46900/apn.v5i3.210.
Анотація:
Central nervous system tumors in pediatric patients represent the most frequent solid tumor type in this age group, especially ependymomas, which comprise 5% of childhood intracranial tumors, and occur mainly in the posterior fossa. The MEDLINE and ClinicalTrials databases were searched for studies on posterior fossa ependymomas, 64 studies met the search criteria.
Symptoms include ataxia, vertigo, headache, vomiting, cranial nerve palsies, or papilledema, resulting from intracranial hypertension due to obstruction of CSF circulation. Imaging examination often reveals posterior fossa ependymomas in the fourth ventricle or cerebellopontine angle; they may extend through the foramina of Luschka, Magendie, and/or magnum. These tumors cause a significant mass effect, displacing rather than invading the hidbrain, and may involve vessels or cranial nerves, making surgical resection difficult. Various radiological features have been studied to distinguish between ependymomas and other histological types, as well as between ependymoma subtypes.
The 2021 WHO classification of central nervous system tumors classifies ependymomas by location. Three types occur in the posterior fossa: subependymomas, PF-A, and PF-B. These are differentiated by the degree of DNA methylation and CpG islands, which can be assessed by immunohistochemical analysis with H3K27me3.
The consensus on the treatment of posterior fossa ependymomas in children advocates the maximum possible surgical resection, followed by radiotherapy in patients >1 year. Chemotherapy remains controversial, with no survival gain. Recent studies suggest molecular subgroups as an independent prognostic factor in posterior fossa ependymomas. The therapeutic challenge of ependymomas requires new treatment options, based on new technologies and molecular discoveries.