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![Survival rates of ependymoma [33,46].](profile/Suranjana-Sikdar/publication/352792113/figure/tbl2/AS:1039680254529540@1624890497604/Survival-rates-of-ependymoma-33-46.png)
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![Survival rates of ependymoma [33,46].](profile/Suranjana-Sikdar/publication/352792113/figure/tbl2/AS:1039680254529540@1624890497604/Survival-rates-of-ependymoma-33-46_Q320.jpg)
![Molecular subgroups of ependymoma [41,51-53].](profile/Suranjana-Sikdar/publication/352792113/figure/tbl3/AS:1039680254537732@1624890497629/Molecular-subgroups-of-ependymoma-41-51-53_Q320.jpg)
Glial cells comprise the non-sensory parts of the central nervous system as well as the peripheral nervous system. Glial cells, also known as neuroglia, constitute a significant portion of the mammalian nervous system and can be viewed simply as a matrix of neural cells. Despite being the “Nervenkitt” or “glue of the nerves”, they aptly serve multi...
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Context 1
... percentage of brain or CNS tumors that were ependymomas decreased with increasing age, with a prevalence of 4% for 15-19 years and 1.9% for adults (>19 years) ( Table 2). From a statistical report, it was found that ependymoma patients aged 20-44 years showed high survival rates (91%) [33,46], whereas the survival rates were lowest for those aged 75+ years (57.8%) and for children aged 0-19 years (75.2%, Table 3). Table 2. Prevalence of ependymomas among the incidence of primary brain or central nervous system (CNS) tumors [33,46]. ...Context 2
... percentage of brain or CNS tumors that were ependymomas decreased with increasing age, with a prevalence of 4% for 15-19 years and 1.9% for adults (>19 years) ( Table 2). From a statistical report, it was found that ependymoma patients aged 20-44 years showed high survival rates (91%) [33,46], whereas the survival rates were lowest for those aged 75+ years (57.8%) and for children aged 0-19 years (75.2%, Table 3). Table 2. Prevalence of ependymomas among the incidence of primary brain or central nervous system (CNS) tumors [33,46]. ...Citations
... Recurrence tends to be local but distant central nervous system (CNS) metastases are not uncommon. Rarely, MPEs can metastasize to extraneural locations, including pelvic structures (i.e., uterus, ovaries) and the lungs [4,[6][7][8][9][10][11][12]. The sacro-coccygeal region is the most common origin of ependymomas that develop extraneural metastases, and of those, 46.5% are MPEs [11]. ...
... There is an absence of consensus on the best treatment approach for extraneural MPE metastases [4,7]. The standard of care for initial MPE diagnosis is GTR, or subtotal resection with radiotherapy (RT) [3,4,7,13]. ...
... There is an absence of consensus on the best treatment approach for extraneural MPE metastases [4,7]. The standard of care for initial MPE diagnosis is GTR, or subtotal resection with radiotherapy (RT) [3,4,7,13]. Systemic treatment for CNS dissemination or extraneural metastasis has shown inconclusive results regarding improvement of clinical outcomes [8,[11][12][13][14]. ...
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.
... The 2016 classification established by the WHO, which takes into account cellular morphological findings, is still used. The treatment will take into account the aforementioned characteristics established in recent guidelines 7,11 . ...
... Seo et al. 11 Surgery + RT GTR when possible. IMRT exhibits great local control and less toxicity than 3D conformal RT/CT efficacy on ependymomas is controversial. ...
... Research in mice showed that a 30-40% dietary restriction led to a 79.5% reduction of tumor growth in astrocytomas. Studies have proven that the ketogenic diet has anti-inflammatory, anti-angiogenic and pro-apoptotic effects, however, it should only be used as a complementary therapy to current treatments, considering that the dietary changes do not prevent tumor growth, only retard it 11,15 . ...
... Ependymomas account for 10% of central nervous system (CNS) tumors in children and 3% in adults. 1 They span a wide histopathologic spectrum, ranging from WHO grade II myxopapillary ependymomas and subependymomas to WHO grade III anaplastic ependymomas. 2 Distinguishing between WHO grade II and III ependymomas with traditional histopathology is difficult and often nonprognostic. 3 However, recent research has highlighted the additional prognostic value of molecular characteristics such as chromosomal instability and/or fusion genes, even leading to two fusion-defined WHO diagnostic classifications. ...
... Ependymomas are the third most frequent glial brain tumors in children and have a prevalence proportion of 0.43 patients per 100000, which represents 6-10% of all childhood brain tumors [1,2]. ...
Purpose
The aim of the study was to evaluate the feasibility of texture analysis in differentiating between posterior fossa ependymoma type A (PF-EPN-A) and type B (PF-EPN-B) among children.
Materials and Methods
Our retrospective study included 43 patients (37 PF-EPN-A and 6 PF-EPN-B) who were pathologically diagnosed with ependymomas in the posterior fossa. The texture features were extracted automatically from the volume of interests (VOIs), which were manually delineated on fluid-attenuated inversion recovery (FLAIR), contrast-enhanced T1-weighted (T1C), and diffusion-weighted imaging (DWI) MRI sequences. A receiver operating characteristic curve (ROC) was built to assess the diagnostic value of the texture parameters, and the prognostic value was evaluated by survival analysis.
Results
Texture parameter [Wavelet-LHH (H: High pass filter, L: Low pass
filter)_glcm (gray-level co-occurrence matrix)_Idn (Inverse difference normalized)] provides valuable information in distinguishing subgroups of ependymomas with higher specificity and positive predictive value (PPV). A total of 27 patients were divided into a high-risk group (IDN value＞0.916) and a low-risk group (IDN value＜0.916) with the most optimistic cut-off value (0.916). The Kaplan–Meier analysis of the survival curves showed significantly longer disease-free survival for low-risk groups compared to high-risk groups [hazard ratio (HR): 0.28, 95% confidence interval (CI): 0.11–0.69; p= 0.017].
Conclusion
Our results suggested that the texture parameters based on DWI images can be used to differentiate PF-EPN-A from PF-EPN-B. Texture analysis could be used as a noninvasive tool in distinguishing subgroup pediatric posterior fossa ependymomas and provide reliable prognostic information upon the verification of its reproducibility and feasibility by further studies.
... Puede extenderse a través del acueducto de Silvio, foramen de Luschka y/o Magendie o proyectarse hacia el canal raquídeo a través del foramen magno. Es infrecuente que presente diseminación a nivel del neuroeje, ya que en menos del 10% de los casos se presenta con enfermedad metastásica al momento del diagnóstico [2][3][4]16 De acuerdo a la edad y la localización tumoral, las manifestaciones clínicas pueden variar desde aumento del perímetro cefálico, pérdida de pautas madurativas, compromiso de par es craneales, convulsiones, déficit motor, alteración de la marcha o signos y síntomas relacionados con la presencia de hipertensión endocraneana (HTE) 2,4 . En resonancia magnética (RM) de cerebro se observa tumoración heterogénea sólida o sólido-quística con áreas de hemorragia, quistes y calcificaciones, que capta contraste en forma variable. ...
Introducción: El ependimoma ocupa el tercer lugar entre los tumores del encéfalo en pediatría siendo más frecuente la localización infratentorial. Los mejores resultados se obtuvieron con resecciones quirúrgicas completas seguidas de radioterapia. En niños pequeños se requieren otras estrategias terapéuticas, como radioterapia de intensidad modulada y las que surgirán de la nueva clasificación de la OMS. Esto ha generado una disminución significativa en el daño neurológica. La nueva clasificación de la OMS basada en localización, histopatología y determinaciones moleculares permitirá estratificar y adecuar tratamientos.El objetivo de este estudio es identificar factores clínico-quirúrgicos relacionados con el pronóstico.Materiales y métodos: Análisis transversal, observacional y retrospectivo teniendo en cuenta la edad, el sexo, el porcentaje de exéresis y la evolución de los pacientes con diagnóstico de Ependimoma intracraneano tratados por los Servicios de Neurocirugía y Oncología del HIAEP “Superiora Sor María Ludovica” entre el año 2011 y el 2020. Los datos fueron obtenidos del análisis de las historias clínicas con consentimiento previo.Resultados: Se incluye ron 12 pacientes con media de edad 5,7 años. Localización: 10 infratentoriales, 2 supratentoriales. La media de edad fue de 5,7 años y 10 fueron varones. Los supratentoriales presentaron al diagnóstico: hemiparesia y alteración de la marcha. Se obtuvo exéresis completa en todos los casos (2) Prevaleció el subtipo anaplásico OMS III. Un paciente recibió radioterapia por presencia de fusión RELA. Ambos se encuentran vivos. A nivel infratentorial predominó la HTE. La exéresis fue subtotal en 6 casos. El subtipo predominante fue OMS II. Los menores de 1 año recibieron quimioterapia. Todos recibieron radioterapia local. Fallecieron 4 pacientes, 1 por recidiva metastásica, el resto por complicaciones posquirúrgicas. De los 6 pacientes vivos la media de seguimiento fue de 49.83 meses (r 21- 85), 2 reciben tratamiento por recidiva siendo menores de 1 año al diagnóstico y, presentando subtipo anaplásico OMS III.Conclusiones: La menor edad, la localización infratentorial y el subtipo anaplásico se relacionaron con mayor morbimortalidad. El grado de resección tiene influencia decisiva en el pronóstico. Se requiere de intervención multidisciplinaria para diseñar la mejor estrategia terapéutica.
In 2021, the World Health Organization released the fifth edition of the central nervous system (CNS) tumor classification. This classification uses histopathology and molecular pathogenesis to group tumors into more biologically and molecularly defined entities. The prognosis of brain cancer, particularly malignant tumors, has remained poor worldwide, approximately 308,102 new cases of brain and other CNS tumors were diagnosed in the year 2020, with an estimated 251,329 deaths. The cost and time-consuming nature of studies to find new anticancer agents makes it necessary to have well-designed studies. In the present study, the pathways that can be targeted for drug development are discussed in detail. Some of the important cellular origins, signaling, and pathways involved in the efficacy of bioactive molecules against CNS tumorigenesis or progression, as well as prognosis and common approaches for treatment of different types of brain tumors, are reviewed. Moreover, different study tools, including cell lines, in vitro, in vivo, and clinical trial challenges, are discussed. In addition, in this article, natural products as one of the most important sources for finding new chemotherapeutics were reviewed and over 700 reported molecules with efficacy against CNS cancer cells are gathered and Drug Dev Res. 2024;85:e22180. wileyonlinelibrary.com/journal/ddr Abbreviations: 4EBP1, eukaryotic initiation factor binding protein 1; AKT, protein kinase B; ATM, ataxia telangiectasia mutated; BBB, blood brain barrier; CAT, catalytic subunit; CDK, cyclin-dependent kinase; CDKs, cyclin-dependent kinases; CHOP, C/EBP HOMOLOGOUS PROTein; CK2, casein kinase 2; CNS, central nervous system; COX, cyclooxygenase; CSF, cerebrospinal fluid; cyt c, Cytochrome c; EC 50 , half maximal effective concentration; ED 50 , median effective dose, which is the dose that produces the effect in 50% of the population that take that dose; EGF, epidermal growth factor; EGFR, epidermal growth factor receptor; eIF4E, eukaryotic initiation factor 4E; ER, endoplasmic reticulum; ERK, extracellular-signal-regulated kinase; FGFR, fibroblast growth factor receptor; FPP, farnesyl pyrophosphate; FTase, farnesyltransferase; GB, glioblastoma; GBM, glioblastoma multiforme; GDP, guanosine diphosphate; GEFs, guanine nucleotide exchange factors; GFAP, glial fibrillary acidic protein; GGTase-I, geranylgeranyltransferase type I; GI50, concentration of any compound required for 50% growth inhibition of cells; Grb2, growth factor receptor-bound protein 2; GSK, glycogen synthase kinase; GTP, guanosine triphosphate; HAAE-2, human abdominal aorta endothelial cells; HDM2, human and/or murine double minute-2 protein; HIF-1α, hypoxia-inducible factor 1 alpha; HMGB1, high mobility group box 1 protein; IC 50 , half maximal inhibitory concentration; JNK, c-Jun N-terminal kinase; LOX, lipoxygenase; LPT, long-term potentiation; LRP, lipoprotein related proteins; LXRs, liver X receptors; mAB, monoclonal antibodies; MAPK, mitogen-activated protein kinase; MAPK, mitogen-activated protein kinase; MAST, microtubule-associated serine-threonine kinase; MDM2, p53-Mouse double minute 2; MEKis, MEK inhibitors; MMP, matrix metalloproteinase; mTORC1, mammalian target of rapamycin complex 1; MVA, mevalonate; MW, molecular weight; NAPA, Napabucasin; NF1, Neurofibromin 1; NIH, National Institutes of Health; NQO1, NAD(P)H Quinone Dehydrogenase 1; NSCs, neural stem cells; PA, pilocytic astrocytoma; PDGFR, platelet-derived growth factor receptor; PDGFRs, platelet-derived growth factor receptors; PERK, protein kinase RNA-like endoplasmic reticulum kinase (PERK); PFS, progression-free survival; PI3K, phosphatidylinositol-4,5-bisphosphate 3-kinase; PI3K, phosphatidylinositol-4,5-bisphosphate 3-kinase; PI3P, intracellular phosphatidylinositol-3,4,5-trisphosphate; PI(3,4,5)P3 or PIP3, phosphatidylinositol (3,4,5)-trisphosphate; PI(4,5)P2 or PIP2, phosphatidylinositol (4,5)-bisphosphate; pRB, retinoblastoma protein; PTEN, phosphatase and tensin homolog deleted on chromosome 10; PTM, posttranslational modification; RAGE, receptor for advanced glycation end products; RECK, reversion-inducing-cysteine-rich protein with kazal motifs; ROCK, RhoA-associated kinase; ROS, reactive oxygen species; RTKs, receptor tyrosine kinases; SAPKs, stress-activated protein kinases; SC, subcutaneous; Sp1, specificity protein 1; TAD, transactivation domain; TGI, concentration eliciting total growth inhibition; TKIs, tyrosine kinase inhibitors; TP53, tumor protein p53; TUNEL, terminal uridine deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick-end labeling; VEGFRs, vascular endothelial growth factor receptors; WHO, World Health Organization. Ardalan Pasdaran and Azadeh Hamedi gathered data. All authors contributed in writing and revising the manuscript.
In 2021, the World Health Organization released the fifth edition of the central nervous system (CNS) tumor classification. This classification uses histopathology and molecular pathogenesis to group tumors into more biologically and molecularly defined entities. The prognosis of brain cancer, particularly malignant tumors, has remained poor worldwide, approximately 308,102 new cases of brain and other CNS tumors were diagnosed in the year 2020, with an estimated 251,329 deaths. The cost and time‐consuming nature of studies to find new anticancer agents makes it necessary to have well‐designed studies. In the present study, the pathways that can be targeted for drug development are discussed in detail. Some of the important cellular origins, signaling, and pathways involved in the efficacy of bioactive molecules against CNS tumorigenesis or progression, as well as prognosis and common approaches for treatment of different types of brain tumors, are reviewed. Moreover, different study tools, including cell lines, in vitro, in vivo, and clinical trial challenges, are discussed. In addition, in this article, natural products as one of the most important sources for finding new chemotherapeutics were reviewed and over 700 reported molecules with efficacy against CNS cancer cells are gathered and classified according to their structure. Based on the clinical trials that have been registered, very few of these natural or semi‐synthetic derivatives have been studied in humans. The review can help researchers understand the involved mechanisms and design new goal‐oriented studies for drug development against CNS malignancies.
The incidence of multiple primary malignancies (MPM) is increasing, and therefore, it has become highly important for clinicians to consider the concept of MPM when treating oncology patients. In this case report, we follow the clinical course of a patient diagnosed with a new intracranial lesion, an ependymoma, on a background of MPM. We explore the barriers implicating the delay in her diagnosis, dissect the challenges in managing her disease and emphasise the importance of social determinants in optimising her care.
Ependymomas account for approximately 5% of all CNS tumors in adults and around 10% in the pediatric population. Contrary to traditional theories supporting that ependymomas arise from ependymal cells, recent studies propose radial glial cells as the cells of origin. In adults, half of the ependymomas arise in the spinal cord, whereas in the pediatric population, almost 90% of ependymomas are located intracranially. Most of the ependymomas are usually low-grade tumors except anaplastic variants and some cases of RELA-fusion-positive ependymomas, a molecular variant consisting the most recent addition to the 2016 World Health Organization (WHO) classification. Of note, the recently described molecular classification of ependymomas into nine distinct subgroups appears to be of greater clinical utility and prognostic value compared to the traditional histopathological classification, and parts of it are expected to be adopted by the WHO in the near future. Clinical manifestations depend on the location of the tumor with infratentorial ependymomas presenting with acute hydrocephalus. Gross total resection should be the goal of treatment. The prognostic factors of patients with ependymomas include age, grade, and location of the tumor, with children with intracranial, anaplastic ependymomas having the worst prognosis. In general, the 5-year overall survival of patients with ependymomas is around 60-70%.
