BACKGROUND Ependymoma with lipomatous differentiation is a rare type of ependymoma.The ZFTA fusion-positive supratentorial ependymoma is a novel tumor type in the 2021 World Health Organization classification of centr...BACKGROUND Ependymoma with lipomatous differentiation is a rare type of ependymoma.The ZFTA fusion-positive supratentorial ependymoma is a novel tumor type in the 2021 World Health Organization classification of central nervous system tumors.ZFTA fusion-positive lipomatous ependymoma has not been reported to date.CASE SUMMARY We reported a case of a 15-year-old Chinese male who had a sudden convulsion lasting approximately six minutes.Magnetic resonance imaging showed a round cystic shadow of approximately 1.9 cm×1.5 cm×1.9 cm under the right parieto-occipital cortex.Microscopic examination showed characteristic perivascular pseudorosettes and adipose differentiation in the cytoplasm.Immunohisto-chemical staining showed that the tumor cells were negative for cytokeratin,NeuN,Syn and p53,but positive for GFAP,vimentin and S-100 protein.Signi-ficant punctate intracytoplasmic EMA immunoreactivity was observed.The level of Ki-67 was about 5%.Genetic analysis revealed ZFTA:RELA fusion.A cranio-tomy with total excision of the tumor was performed.The follow-up time was 36 months,no evidence of disease recurrence was found in magnetic resonance imaging.CONCLUSION Based on these findings,the patient was diagnosed as a ependymoma with ZFTA fusion and lipomatous differentiation.This case report provides information on the microscopic morphological features of ependymoma with ZFTA fusion and lipomatous differentiation,which can help pathologists to make a definitive diagnosis of this tumor.展开更多
Posterior fossa ependymoma comprises two distinct molecular entities,ependymoma_posterior fossa A(EPN_PFA)and ependymoma_posterior fossa B(EPN_PFB),with differentiable gene expression profiles.As yet,the response of t...Posterior fossa ependymoma comprises two distinct molecular entities,ependymoma_posterior fossa A(EPN_PFA)and ependymoma_posterior fossa B(EPN_PFB),with differentiable gene expression profiles.As yet,the response of the two entities to treatment is unclear.To determine the relationship between the two molecular subgroups of posterior fossa ependymoma and treatment,we studied a cohort of 820 patients with molecularly profiled,clinically annotated posterior fossa ependymomas.We found that the strongest predictor of poor outcome in patients with posterior fossa ependymoma across the entire age spectrum was molecular subgroup EPN_PFA,which was recently reported in the paper entitled "Therapeutic impact of cytoreductive surgery and irradiation of posterior fossa ependymoma in the molecular era:a retrospective multicohort analysis" in the Journal ofClinical Oncology.Patients with incompletely resected EPN_PFA tumors had a very poor outcome despite receiving adjuvant radiation therapy,whereas a substantial proportion of patients with EPN_PFB tumors can be cured with surgery alone.展开更多
Ependymomas are a somewhat diverse category of glial tumors that often develop from the lining of the brain’s ventricles, or the spinal cord’s central canal. They make up 5% of all neuroepithelial tumors, 10% of pae...Ependymomas are a somewhat diverse category of glial tumors that often develop from the lining of the brain’s ventricles, or the spinal cord’s central canal. They make up 5% of all neuroepithelial tumors, 10% of paediatric brain tumors, and up to 33% of brain tumors in children under the age of three. Hydrocephalus is one of the complications, and it can be identified as progressive macrocephaly or increasing head circumference crossing percentiles, nausea, vomiting, poor appetite, irritability, and regression of developmental milestones.展开更多
BACKGROUND Myxopapillary ependymomas are rare spinal tumours.Although histologically benign,they have a tendency for local recurrence.CASE SUMMARY We describe a patient suffering from extra-and intradural myxopapillar...BACKGROUND Myxopapillary ependymomas are rare spinal tumours.Although histologically benign,they have a tendency for local recurrence.CASE SUMMARY We describe a patient suffering from extra-and intradural myxopapillary ependymoma with perisacral spreading.He was treated with subtotal resection and postoperative radiation therapy.After treatment,he experienced slight sphincter disorders and lumboischialgic pain with no motor or sensory disturbances.Eight months later,a tumour regression was documented.The patient is still followed-up regularly.CONCLUSION Lumbar myxopapillary ependymomas may present with lumbar or radicular pain,similar to more trivial lesions.Magnetic resonance imaging(MRI)is the primary modality for diagnosis.The treatment aim is to minimize both tumour and therapy-related morbidity and to involve different treatment modalities.展开更多
The current study investigated correlations between the expression of leucine-rich repeats and immunoglobulin-like domain 1 （LRIG1） and antioxidant enzymes and related proteins, including manganese superoxide dismut...The current study investigated correlations between the expression of leucine-rich repeats and immunoglobulin-like domain 1 （LRIG1） and antioxidant enzymes and related proteins, including manganese superoxide dismutase, glutamate cysteine ligase catalytic or regulatory subunit, thioredoxin and thioredoxin reductase, in both human ependymoma and oligodendroglioma. Results revealed that the cytoplasmic expression of LRIG1 was associated with expression of glutamate cysteine ligase catalytic subunit in the human ependymoma, while the nuclear expression of LRIG1 was associated with expression of thioredoxin reductase. In human oligodendroglioma, the cytoplasmic expression of LRIG1 was associated with expression of the glutamate cysteine ligase catalytic subunit. Both the nuclear and perinuclear expressions of LRIG1 were associated with expression of glutamate cysteine ligase regulatory subunit. These results indicated that several antioxidant enzymes and related proteins contributed to LRIG1 expression, and that these may participate in the antioxidation of the cells.展开更多
Purpose: to investigate cognitive and psychological problems in children treated for intracranial ependymoma, the evolution of these disorders over time and the role of age at radiotherapy and tumor site, in their ons...Purpose: to investigate cognitive and psychological problems in children treated for intracranial ependymoma, the evolution of these disorders over time and the role of age at radiotherapy and tumor site, in their onset and persistence. Methods and Materials: 23 patients received a complete evaluation;some of them underwent follow-ups. The clinical data collected included sex, age (at diagnosis, assessment and tumor treatment, thus dividing patients into two cohorts: younger or older than 5 years), site (supratentorial vs. subtentorial), the presence of hydrocephalus, neurological examination, tumor treatment. All the patients received an age-appropriate cognitive and psychological evaluation. Results: The mean cognitive level was within the norm, with lower scores on the Performance Intelligence Quotient (PIQ). The psychological assessment revealed Internalizing problems and impaired independence. Children older than 5 years had a lower Intelligence Quotient (IQ) than the younger children, both at the initial evaluation and at follow-ups. Initially, the supratentorial group appeared to be less impaired than the subtentorial group but then exhibited a progressive decline in the IQ. In the subtentorial group, the children with an IQ within the normal range remained stable at the follow-up, while the children with below-norm scores at the initial evaluation showed deterioration over time. Conclusions: Tumor site seems to affect the cognitive outcome to a greater extent than age at radiotherapy.展开更多
BACKGROUND An ependymoma is a glial tumor that usually occurs in or near the ventricle,close to the ependyma.It rarely occurs exclusively in the brain parenchyma without being associated with the ventricle.CASE SUMMAR...BACKGROUND An ependymoma is a glial tumor that usually occurs in or near the ventricle,close to the ependyma.It rarely occurs exclusively in the brain parenchyma without being associated with the ventricle.CASE SUMMARY Here,we report a rare case of a cerebellar ependymoma completely located in the brain parenchyma.A previously healthy 32-year-old female with a 1-month history of dizziness was admitted to our hospital.During hospitalization,magnetic resonance imaging of the brain revealed a space-occupying lesion measuring 57 mm×41 mm×51 mm in the right cerebellar hemisphere and inferior cerebellar vermis.The patient underwent surgical resection for the right cerebellar mass.Postoperative pathological examination revealed an ependymoma.At 1 year follow-up,the patient was doing well and showed no recurrence.CONCLUSION We conducted a literature review and summarized three theories regarding ependymomas located exclusively in the brain parenchyma,which are key to the diagnosis of intraparenchymal cerebellar ependymomas.Surgery and postoperative radiotherapy are the primary treatment options for ependymomas.展开更多
BACKGROUND Myxopapillary ependymoma(MPE)is a pathological grade I tumor that arises in the filum terminale.MPE with anaplastic features is extremely rare,and only 5 cases have shown malignancy at the time of recurrenc...BACKGROUND Myxopapillary ependymoma(MPE)is a pathological grade I tumor that arises in the filum terminale.MPE with anaplastic features is extremely rare,and only 5 cases have shown malignancy at the time of recurrence.CASE SUMMARY The patient(a 46-year-old woman)had undergone a MPE operation 30 years ago.After subtotal resection of the tumor located in L4-S1,it had a solid component that extended to the adjacent subcutaneous region.Histologically,the tumor consisted of a typical MPE with anaplastic features.The anaplastic areas of the tumor showed hypercellularity,a rapid mitotic rate,vascular proliferation,and connective tissue proliferation.Pleomorphic cells and atypical mitotic figures were occasionally observed.The MIB-1 index in this area was 12.3%.The immunohistochemical study showed immunoreactivity for vimentin,glial fibrillary acidic protein and S100.The morphological pattern and immunohistochemical profile were consistent with anaplastic MPE.The patient tolerated surgery well without new neurological deficits.She underwent local irradiation for the residual tumor and rehabilitation.CONCLUSION Although extremely rare,anaplastic MPE occurs in both pediatric and adult patients,similar to other ependymomas.At a minimum,close monitoring is recommended,given concerns about aggressive biological potential.In the future,further study is needed to determine the WHO classification criteria and genetic indicators of tumor progression.The possibility of malignant transformation of MPE should be taken into account,and patients with MPE should be treated with care and follow-up.展开更多
Ependymoma is a rare and chemotherapy-resistant brain tumor, which has resulted in a delay in the development of drugs to treat it. A subclass of supratentorial ependymomas (ST-EPN), designated ST-EPN-zinc finger-tran...Ependymoma is a rare and chemotherapy-resistant brain tumor, which has resulted in a delay in the development of drugs to treat it. A subclass of supratentorial ependymomas (ST-EPN), designated ST-EPN-zinc finger-translocation-associated (ZFTA, ST-EPN-ZFTA), exhibits the expression of a fusion protein comprising ZFTA and v-rel reticuloendotheliosis viral oncogene homolog A (RELA), an effector transcription factor of the nuclear factor-kappa B (NF-κB) pathway (ZFTA-RELA). The expression of ZFTA-RELA results in the hyperactivation of the oncogenic NF-κB signaling pathway, which ultimately leads to the development of ST-EPN-ZFTA. To identify inhibitors of the NF-κB signaling pathway activated by the expression of ZFTA-RELA, we used a doxycycline-inducible ZFTA-RELA-expressing NF-κB reporter cell line and found that extracts of the fungus Neosartorya spinosa IFM 47025 exhibited NF-κB inhibitory activity. We identified eight compounds [aszonapyrone A (2), sartorypyrone A (3), epiheveadride (4), acetylaszonalenin (5), (R)-benzodiazepinedione (6), aszonalenin (7), sartorypyrone E (8) and (Z, Z)-N,N’-(1,2-bis[(4-methoxyphenyl)methylene]-1,2-ethanediyl)bis-formamide (9)] from N. spinosa IFM 47025 culture extract using a variety of chromatographic techniques. The structures of these compounds were identified through the analysis of various instrumental data (1D, 2D-NMR, MS, and optical rotation). The NF-κB responsive reporter assay indicated that compounds 2, 3, 5, 7, and 9 exhibited inhibitory activity. We further evaluated the inhibitory activity of these compounds against the expression of endogenous NF-κB responsive genes (CCND1, L1CAM, ICAM1, and TNF) and found that compound 2 showed significant inhibitory activity. Further studies are required to elucidate the mechanism of action of compound 2, which may serve as a lead compound for the development of a novel therapy for ST-EPN-ZFTA.展开更多
Supratentorial cortical ependymoma is a rare primary brain tumor in adults. Only 50 cases are reported in the literature including pediatric and adult patients. We report a 30-year-old male with sensory-type seizures ...Supratentorial cortical ependymoma is a rare primary brain tumor in adults. Only 50 cases are reported in the literature including pediatric and adult patients. We report a 30-year-old male with sensory-type seizures who was found to have a right posterior frontal cortical lesion with large cystic component. The lesion was resected completely and histopathology and immunohistochemistry examinations confirmed anaplastic ependymoma WHO grade III. The patient received radiotherapy treatment post-surgery, and he was tumor free one year after treatment.展开更多
Primary intracranial ependymomas originate from ependymal cells. They may migrate mainly in the spinal cord but rarely metastasize outside the central nervous system. Metastases outside the central nervous system are ...Primary intracranial ependymomas originate from ependymal cells. They may migrate mainly in the spinal cord but rarely metastasize outside the central nervous system. Metastases outside the central nervous system are rare. Metastatic diffusion from the central nervous system is low due to the unique interaction of the brain and the tumor with the blood-brain barrier. Nevertheless, three main hypotheses have been mentioned in the literature, the tumor growth, the surgical manipulation (which may be considered to be the case in our patient), and the aggressiveness of the tumor according to the Ki67 index. We report the case of a 16-year-old female, who underwent complete surgical removal of a left occipital 2007 WHO grade II ependymoma. 3 years later, the patient presented multiple cervical and occipital indurated masses. MRI showed a left hemispheric meningeal infiltration, with multiple nodules located on the neck, occiput and mastoid. Histopathological study of a left temporal surgical biopsy and resection of an occipital subcutaneous nodule turned to be metastases of an anaplastic ependymoma. The ependymoma considered as a benign tumor could very quickly turn into malignancy by its metastatic potential. Early diagnosis and longer follow-up of patients would be recommended for a rapid management.展开更多
Introduction: Intra-dural and extramedullary ependymomas are rare lesions. We report a clinical case confirmed by the histology supported by the neurosurgery department of Martinique University Hospital. Clinical, par...Introduction: Intra-dural and extramedullary ependymomas are rare lesions. We report a clinical case confirmed by the histology supported by the neurosurgery department of Martinique University Hospital. Clinical, paraclinical and therapeutic data will be compared to a literature review. Observation: A 38-year-old patient was admitted for tetraparesthesia evolving since 3 months, associated with a left hemiparesis. The clinical examination revealed deterioration of the general condition and a tetrapyramidal syndrome. MRI of the cervical spine revealed extensive cervical extra-medullary intradural lesion of C3 to C5. The treatment had been an excisional surgery that had been subtotal in two stages, supplemented by craniospinal radiotherapy because the pathological examination had found a grade III anaplasic ependymoma. Conclusion: Because of their rarity, the treatment of these forms of ependymoma is not yet well codified.展开更多
We present morphological findings observed from the study of a myxopapillary ependymoma located in the III ventricle. This tumor, which is a rare occurrence at this level, was observed in a 73-year-old woman with no r...We present morphological findings observed from the study of a myxopapillary ependymoma located in the III ventricle. This tumor, which is a rare occurrence at this level, was observed in a 73-year-old woman with no relevant medical history who presented with obstructive hydrocephalus due to compression and blockage of the foramen of Monro. The good delineation of the lesion allowed its surgical excision, and histological examination of the material obtained after excisional biopsy revealed a glial neoplasm with a low degree of cytological malignancy that was strongly positive for glial fibrillary acidic protein (GFAP) as well as a myxopapillary architecture analogous to that observed in ependymomas of the filum terminale. Subsequent radiological examination allowed us to rule out a metastatic nature of the tumor, confirming its primary origin. We briefly discuss the case and review the main characteristics of this neoplasia, which is quite rare at the site identified in our patient.展开更多
Objective To study the clinical feature,image,treatment and prognosis of supratentorial parenchymalependymomas.Methods14cases of patients sufferedfrom supratentorial parenchymal ependymomas were treated.There were10ep...Objective To study the clinical feature,image,treatment and prognosis of supratentorial parenchymalependymomas.Methods14cases of patients sufferedfrom supratentorial parenchymal ependymomas were treated.There were10ependymomas and4malignantependymomas.Tumor located in frontal lobe in4cases,展开更多
Objective To analyze factors affecting operative prognosis of intramedullary ependymomas in medulla oblongata and cervical cord. Methods Thirty patients harboring intramedullary ependymomas in medulla oblongata and ce...Objective To analyze factors affecting operative prognosis of intramedullary ependymomas in medulla oblongata and cervical cord. Methods Thirty patients harboring intramedullary ependymomas in medulla oblongata and cervical cord were operated. The preoperative and postoperative status was taken by follow-up and展开更多
Brain tumors are the leading cause of cancer death in children,with ependymoma being the third most common and posing a significant clinical burden.Its mechanism of pathogenesis,reliable prognostic indicators,and effe...Brain tumors are the leading cause of cancer death in children,with ependymoma being the third most common and posing a significant clinical burden.Its mechanism of pathogenesis,reliable prognostic indicators,and effective treatments other than surgical resection have all remained elusive.Until recently,ependymoma research was hindered by the small number of tumors available for study,low resolution of cytogenetic techniques,and lack of cell lines and animal models.Ependymoma heterogeneity,which manifests as variations in tumor location,patient age,histological grade,and clinical behavior,together with the observation of a balanced genomic profile in up to 50% of cases,presents additional challenges in understanding the development and progression of this disease.Despite these difficulties,we have made significant headway in the past decade in identifying the genetic alterations and pathways involved in ependymoma tumorigenesis through collaborative efforts and the application of microarray-based genetic(copy number) and transcriptome profiling platforms.Genetic characterization of ependymoma unraveled distinct mRNA-defined subclasses and led to the identification of radial glial cells as its cell type of origin.This review summarizes our current knowledge in the molecular genetics of ependymoma and proposes future research directions necessary to further advance this field.展开更多
INTRODUCTION Tanycytic ependymoma is a rare subtype of the World Health Organization Grade il ependymoma most commonly found in the cervical and thoracic segments of the spinal cord. Spinal tumors in neurofibromatosis...INTRODUCTION Tanycytic ependymoma is a rare subtype of the World Health Organization Grade il ependymoma most commonly found in the cervical and thoracic segments of the spinal cord. Spinal tumors in neurofibromatosis type 2 （NF-2） can be intramedullary, extramedullary, or associated with the nerve roots, and there may be multiple tumors at multiple locations in the spine.展开更多
Supratentorial cortical ependymomas are uncommon in the pediatric population and extremely rare in very young children.Histologically,tumors of the anaplastic type are also less common in children.The authors report o...Supratentorial cortical ependymomas are uncommon in the pediatric population and extremely rare in very young children.Histologically,tumors of the anaplastic type are also less common in children.The authors report one case of anaplastic cortical ependymoma in a 16-month-old girl who presented with a 7-day history of left side weakness and rapid neurological deterioration.Brain imaging with computed tomography and magnetic resonance imaging scanning showed a huge right fronto-parietal cystic and solid lesion compressing the brain parenchyma.The young child was operated via a transparietal approach with gross total resection of the lesion.The tumor’s histology was anaplastic ependymoma.Intensive chemotherapy was given post operatively and the patient remained well without recurrence after 20 months of follow-up.展开更多
In this paper,a 41-year-old female with previous history of ependymoma is reported.The patient underwent gross-total resection of the tumor and ventriculo-peritoneal shunt placement,followed by radiotherapy to the pos...In this paper,a 41-year-old female with previous history of ependymoma is reported.The patient underwent gross-total resection of the tumor and ventriculo-peritoneal shunt placement,followed by radiotherapy to the posterior fossa and the upper-cervical spinal cord region.Three years later she developed numbness in her right arm,body and leg.Magnetic resonance imaging(MRI)of the entire neuraxis revealed no evidence of tumor recurrence,while a small enhancing area was noted in the left anterolateral spinal cord at the level of the C1-C2 vertebrae and a left posterior-lateral herniated disk in the C5-C6 level which was not present in the earlier MRI at initial diagnosis.Lumbar punctures were negative for malignant cells.The patient’s symptoms were first attributed to radiation-induced effect.Follow-up results of brain and the cervical spine MRI were performed which showed disappearance of the small abnormality in the left C2 spinal cord area but persistence of the herniated C5-C6 disk.Thus,the current diagnosis of right-sided numbness due to pressure of the left anterolateral spinothalamic tracts from the herniated C5-C6 disk was made.This is a unique case,in which herniated disk pressuring effects needed to be differentiated from both radiation-induced treatment effect and tumor recurrence.展开更多
Spinal ependymomas(SEs) are common adult intramedullary tumors; however,determining the absolute boundary between the tumor and the tumor residual may be difficult. We assessed outcomes following the use of fluorescei...Spinal ependymomas(SEs) are common adult intramedullary tumors; however,determining the absolute boundary between the tumor and the tumor residual may be difficult. We assessed outcomes following the use of fluorescein sodium(FS) during surgical removal of SEs. We performed a retrospective analysis of 112 patients with SEs who were treated at Beijing Tsinghua Changgung Hospital between December 2014 and December 2016. Each patient received intravenous FS(3–4 mg/kg) to determine the SE boundaries during surgery. Tumor removal efficiencies and tumor residuals were assessed using magnetic resonance imaging(MRI) at 10 days and 3 months after surgical recovery; McC ormick's spinal function classification was also performed at the 3-month follow-up. The complete tumor removal rate was 92%(103/112). Ninetyfour patients underwent tumor removal under fluorescent light, which provided distinctive tumor fluorescence. Tumor removal under white light was performed in18 patients; fluorescent images were invisible or indistinctive in these 18 patients. At the 3-month follow-up, sensory function(85.8%(91/106)) and movement(84.3%(86/102)) were improved in patients with pre-surgical dysfunction; urination and defecation functions were improved in 66.7%(16/24). The Mc Cormick spinal cord functional classifications, at the 3-month follow-up, showed significant differences in the percentages of patients with disease classified to each grade(I–IV), compared with preoperative classifications(each, P < 0.05). There was no MRI evidence of tumor relapse or residuals at the 3-month follow-up. FS use during the surgical treatment of SE enables complete tumor removal and detection of tumor residuals.展开更多
文摘BACKGROUND Ependymoma with lipomatous differentiation is a rare type of ependymoma.The ZFTA fusion-positive supratentorial ependymoma is a novel tumor type in the 2021 World Health Organization classification of central nervous system tumors.ZFTA fusion-positive lipomatous ependymoma has not been reported to date.CASE SUMMARY We reported a case of a 15-year-old Chinese male who had a sudden convulsion lasting approximately six minutes.Magnetic resonance imaging showed a round cystic shadow of approximately 1.9 cm×1.5 cm×1.9 cm under the right parieto-occipital cortex.Microscopic examination showed characteristic perivascular pseudorosettes and adipose differentiation in the cytoplasm.Immunohisto-chemical staining showed that the tumor cells were negative for cytokeratin,NeuN,Syn and p53,but positive for GFAP,vimentin and S-100 protein.Signi-ficant punctate intracytoplasmic EMA immunoreactivity was observed.The level of Ki-67 was about 5%.Genetic analysis revealed ZFTA:RELA fusion.A cranio-tomy with total excision of the tumor was performed.The follow-up time was 36 months,no evidence of disease recurrence was found in magnetic resonance imaging.CONCLUSION Based on these findings,the patient was diagnosed as a ependymoma with ZFTA fusion and lipomatous differentiation.This case report provides information on the microscopic morphological features of ependymoma with ZFTA fusion and lipomatous differentiation,which can help pathologists to make a definitive diagnosis of this tumor.
文摘Posterior fossa ependymoma comprises two distinct molecular entities,ependymoma_posterior fossa A(EPN_PFA)and ependymoma_posterior fossa B(EPN_PFB),with differentiable gene expression profiles.As yet,the response of the two entities to treatment is unclear.To determine the relationship between the two molecular subgroups of posterior fossa ependymoma and treatment,we studied a cohort of 820 patients with molecularly profiled,clinically annotated posterior fossa ependymomas.We found that the strongest predictor of poor outcome in patients with posterior fossa ependymoma across the entire age spectrum was molecular subgroup EPN_PFA,which was recently reported in the paper entitled "Therapeutic impact of cytoreductive surgery and irradiation of posterior fossa ependymoma in the molecular era:a retrospective multicohort analysis" in the Journal ofClinical Oncology.Patients with incompletely resected EPN_PFA tumors had a very poor outcome despite receiving adjuvant radiation therapy,whereas a substantial proportion of patients with EPN_PFB tumors can be cured with surgery alone.
文摘Ependymomas are a somewhat diverse category of glial tumors that often develop from the lining of the brain’s ventricles, or the spinal cord’s central canal. They make up 5% of all neuroepithelial tumors, 10% of paediatric brain tumors, and up to 33% of brain tumors in children under the age of three. Hydrocephalus is one of the complications, and it can be identified as progressive macrocephaly or increasing head circumference crossing percentiles, nausea, vomiting, poor appetite, irritability, and regression of developmental milestones.
文摘BACKGROUND Myxopapillary ependymomas are rare spinal tumours.Although histologically benign,they have a tendency for local recurrence.CASE SUMMARY We describe a patient suffering from extra-and intradural myxopapillary ependymoma with perisacral spreading.He was treated with subtotal resection and postoperative radiation therapy.After treatment,he experienced slight sphincter disorders and lumboischialgic pain with no motor or sensory disturbances.Eight months later,a tumour regression was documented.The patient is still followed-up regularly.CONCLUSION Lumbar myxopapillary ependymomas may present with lumbar or radicular pain,similar to more trivial lesions.Magnetic resonance imaging(MRI)is the primary modality for diagnosis.The treatment aim is to minimize both tumour and therapy-related morbidity and to involve different treatment modalities.
基金the Swedish Institute, No. 00287/2006210the North Sweden Cancer Foundation, Specialized Research Fund for the Doctoral Program New Teacher of Higher Education by the Chinese Ministry of Education, No. 200804861039the National Natural Science Foundation of China, No. 30973073, 30973072
文摘The current study investigated correlations between the expression of leucine-rich repeats and immunoglobulin-like domain 1 （LRIG1） and antioxidant enzymes and related proteins, including manganese superoxide dismutase, glutamate cysteine ligase catalytic or regulatory subunit, thioredoxin and thioredoxin reductase, in both human ependymoma and oligodendroglioma. Results revealed that the cytoplasmic expression of LRIG1 was associated with expression of glutamate cysteine ligase catalytic subunit in the human ependymoma, while the nuclear expression of LRIG1 was associated with expression of thioredoxin reductase. In human oligodendroglioma, the cytoplasmic expression of LRIG1 was associated with expression of the glutamate cysteine ligase catalytic subunit. Both the nuclear and perinuclear expressions of LRIG1 were associated with expression of glutamate cysteine ligase regulatory subunit. These results indicated that several antioxidant enzymes and related proteins contributed to LRIG1 expression, and that these may participate in the antioxidation of the cells.
文摘Purpose: to investigate cognitive and psychological problems in children treated for intracranial ependymoma, the evolution of these disorders over time and the role of age at radiotherapy and tumor site, in their onset and persistence. Methods and Materials: 23 patients received a complete evaluation;some of them underwent follow-ups. The clinical data collected included sex, age (at diagnosis, assessment and tumor treatment, thus dividing patients into two cohorts: younger or older than 5 years), site (supratentorial vs. subtentorial), the presence of hydrocephalus, neurological examination, tumor treatment. All the patients received an age-appropriate cognitive and psychological evaluation. Results: The mean cognitive level was within the norm, with lower scores on the Performance Intelligence Quotient (PIQ). The psychological assessment revealed Internalizing problems and impaired independence. Children older than 5 years had a lower Intelligence Quotient (IQ) than the younger children, both at the initial evaluation and at follow-ups. Initially, the supratentorial group appeared to be less impaired than the subtentorial group but then exhibited a progressive decline in the IQ. In the subtentorial group, the children with an IQ within the normal range remained stable at the follow-up, while the children with below-norm scores at the initial evaluation showed deterioration over time. Conclusions: Tumor site seems to affect the cognitive outcome to a greater extent than age at radiotherapy.
基金Supported by The Sichuan Medical Youth Innovation Research Project,No.Q22044.
文摘BACKGROUND An ependymoma is a glial tumor that usually occurs in or near the ventricle,close to the ependyma.It rarely occurs exclusively in the brain parenchyma without being associated with the ventricle.CASE SUMMARY Here,we report a rare case of a cerebellar ependymoma completely located in the brain parenchyma.A previously healthy 32-year-old female with a 1-month history of dizziness was admitted to our hospital.During hospitalization,magnetic resonance imaging of the brain revealed a space-occupying lesion measuring 57 mm×41 mm×51 mm in the right cerebellar hemisphere and inferior cerebellar vermis.The patient underwent surgical resection for the right cerebellar mass.Postoperative pathological examination revealed an ependymoma.At 1 year follow-up,the patient was doing well and showed no recurrence.CONCLUSION We conducted a literature review and summarized three theories regarding ependymomas located exclusively in the brain parenchyma,which are key to the diagnosis of intraparenchymal cerebellar ependymomas.Surgery and postoperative radiotherapy are the primary treatment options for ependymomas.
文摘BACKGROUND Myxopapillary ependymoma(MPE)is a pathological grade I tumor that arises in the filum terminale.MPE with anaplastic features is extremely rare,and only 5 cases have shown malignancy at the time of recurrence.CASE SUMMARY The patient(a 46-year-old woman)had undergone a MPE operation 30 years ago.After subtotal resection of the tumor located in L4-S1,it had a solid component that extended to the adjacent subcutaneous region.Histologically,the tumor consisted of a typical MPE with anaplastic features.The anaplastic areas of the tumor showed hypercellularity,a rapid mitotic rate,vascular proliferation,and connective tissue proliferation.Pleomorphic cells and atypical mitotic figures were occasionally observed.The MIB-1 index in this area was 12.3%.The immunohistochemical study showed immunoreactivity for vimentin,glial fibrillary acidic protein and S100.The morphological pattern and immunohistochemical profile were consistent with anaplastic MPE.The patient tolerated surgery well without new neurological deficits.She underwent local irradiation for the residual tumor and rehabilitation.CONCLUSION Although extremely rare,anaplastic MPE occurs in both pediatric and adult patients,similar to other ependymomas.At a minimum,close monitoring is recommended,given concerns about aggressive biological potential.In the future,further study is needed to determine the WHO classification criteria and genetic indicators of tumor progression.The possibility of malignant transformation of MPE should be taken into account,and patients with MPE should be treated with care and follow-up.
文摘Ependymoma is a rare and chemotherapy-resistant brain tumor, which has resulted in a delay in the development of drugs to treat it. A subclass of supratentorial ependymomas (ST-EPN), designated ST-EPN-zinc finger-translocation-associated (ZFTA, ST-EPN-ZFTA), exhibits the expression of a fusion protein comprising ZFTA and v-rel reticuloendotheliosis viral oncogene homolog A (RELA), an effector transcription factor of the nuclear factor-kappa B (NF-κB) pathway (ZFTA-RELA). The expression of ZFTA-RELA results in the hyperactivation of the oncogenic NF-κB signaling pathway, which ultimately leads to the development of ST-EPN-ZFTA. To identify inhibitors of the NF-κB signaling pathway activated by the expression of ZFTA-RELA, we used a doxycycline-inducible ZFTA-RELA-expressing NF-κB reporter cell line and found that extracts of the fungus Neosartorya spinosa IFM 47025 exhibited NF-κB inhibitory activity. We identified eight compounds [aszonapyrone A (2), sartorypyrone A (3), epiheveadride (4), acetylaszonalenin (5), (R)-benzodiazepinedione (6), aszonalenin (7), sartorypyrone E (8) and (Z, Z)-N,N’-(1,2-bis[(4-methoxyphenyl)methylene]-1,2-ethanediyl)bis-formamide (9)] from N. spinosa IFM 47025 culture extract using a variety of chromatographic techniques. The structures of these compounds were identified through the analysis of various instrumental data (1D, 2D-NMR, MS, and optical rotation). The NF-κB responsive reporter assay indicated that compounds 2, 3, 5, 7, and 9 exhibited inhibitory activity. We further evaluated the inhibitory activity of these compounds against the expression of endogenous NF-κB responsive genes (CCND1, L1CAM, ICAM1, and TNF) and found that compound 2 showed significant inhibitory activity. Further studies are required to elucidate the mechanism of action of compound 2, which may serve as a lead compound for the development of a novel therapy for ST-EPN-ZFTA.
文摘Supratentorial cortical ependymoma is a rare primary brain tumor in adults. Only 50 cases are reported in the literature including pediatric and adult patients. We report a 30-year-old male with sensory-type seizures who was found to have a right posterior frontal cortical lesion with large cystic component. The lesion was resected completely and histopathology and immunohistochemistry examinations confirmed anaplastic ependymoma WHO grade III. The patient received radiotherapy treatment post-surgery, and he was tumor free one year after treatment.
文摘Primary intracranial ependymomas originate from ependymal cells. They may migrate mainly in the spinal cord but rarely metastasize outside the central nervous system. Metastases outside the central nervous system are rare. Metastatic diffusion from the central nervous system is low due to the unique interaction of the brain and the tumor with the blood-brain barrier. Nevertheless, three main hypotheses have been mentioned in the literature, the tumor growth, the surgical manipulation (which may be considered to be the case in our patient), and the aggressiveness of the tumor according to the Ki67 index. We report the case of a 16-year-old female, who underwent complete surgical removal of a left occipital 2007 WHO grade II ependymoma. 3 years later, the patient presented multiple cervical and occipital indurated masses. MRI showed a left hemispheric meningeal infiltration, with multiple nodules located on the neck, occiput and mastoid. Histopathological study of a left temporal surgical biopsy and resection of an occipital subcutaneous nodule turned to be metastases of an anaplastic ependymoma. The ependymoma considered as a benign tumor could very quickly turn into malignancy by its metastatic potential. Early diagnosis and longer follow-up of patients would be recommended for a rapid management.
文摘Introduction: Intra-dural and extramedullary ependymomas are rare lesions. We report a clinical case confirmed by the histology supported by the neurosurgery department of Martinique University Hospital. Clinical, paraclinical and therapeutic data will be compared to a literature review. Observation: A 38-year-old patient was admitted for tetraparesthesia evolving since 3 months, associated with a left hemiparesis. The clinical examination revealed deterioration of the general condition and a tetrapyramidal syndrome. MRI of the cervical spine revealed extensive cervical extra-medullary intradural lesion of C3 to C5. The treatment had been an excisional surgery that had been subtotal in two stages, supplemented by craniospinal radiotherapy because the pathological examination had found a grade III anaplasic ependymoma. Conclusion: Because of their rarity, the treatment of these forms of ependymoma is not yet well codified.
文摘We present morphological findings observed from the study of a myxopapillary ependymoma located in the III ventricle. This tumor, which is a rare occurrence at this level, was observed in a 73-year-old woman with no relevant medical history who presented with obstructive hydrocephalus due to compression and blockage of the foramen of Monro. The good delineation of the lesion allowed its surgical excision, and histological examination of the material obtained after excisional biopsy revealed a glial neoplasm with a low degree of cytological malignancy that was strongly positive for glial fibrillary acidic protein (GFAP) as well as a myxopapillary architecture analogous to that observed in ependymomas of the filum terminale. Subsequent radiological examination allowed us to rule out a metastatic nature of the tumor, confirming its primary origin. We briefly discuss the case and review the main characteristics of this neoplasia, which is quite rare at the site identified in our patient.
文摘Objective To study the clinical feature,image,treatment and prognosis of supratentorial parenchymalependymomas.Methods14cases of patients sufferedfrom supratentorial parenchymal ependymomas were treated.There were10ependymomas and4malignantependymomas.Tumor located in frontal lobe in4cases,
文摘Objective To analyze factors affecting operative prognosis of intramedullary ependymomas in medulla oblongata and cervical cord. Methods Thirty patients harboring intramedullary ependymomas in medulla oblongata and cervical cord were operated. The preoperative and postoperative status was taken by follow-up and
文摘Brain tumors are the leading cause of cancer death in children,with ependymoma being the third most common and posing a significant clinical burden.Its mechanism of pathogenesis,reliable prognostic indicators,and effective treatments other than surgical resection have all remained elusive.Until recently,ependymoma research was hindered by the small number of tumors available for study,low resolution of cytogenetic techniques,and lack of cell lines and animal models.Ependymoma heterogeneity,which manifests as variations in tumor location,patient age,histological grade,and clinical behavior,together with the observation of a balanced genomic profile in up to 50% of cases,presents additional challenges in understanding the development and progression of this disease.Despite these difficulties,we have made significant headway in the past decade in identifying the genetic alterations and pathways involved in ependymoma tumorigenesis through collaborative efforts and the application of microarray-based genetic(copy number) and transcriptome profiling platforms.Genetic characterization of ependymoma unraveled distinct mRNA-defined subclasses and led to the identification of radial glial cells as its cell type of origin.This review summarizes our current knowledge in the molecular genetics of ependymoma and proposes future research directions necessary to further advance this field.
文摘INTRODUCTION Tanycytic ependymoma is a rare subtype of the World Health Organization Grade il ependymoma most commonly found in the cervical and thoracic segments of the spinal cord. Spinal tumors in neurofibromatosis type 2 （NF-2） can be intramedullary, extramedullary, or associated with the nerve roots, and there may be multiple tumors at multiple locations in the spine.
文摘Supratentorial cortical ependymomas are uncommon in the pediatric population and extremely rare in very young children.Histologically,tumors of the anaplastic type are also less common in children.The authors report one case of anaplastic cortical ependymoma in a 16-month-old girl who presented with a 7-day history of left side weakness and rapid neurological deterioration.Brain imaging with computed tomography and magnetic resonance imaging scanning showed a huge right fronto-parietal cystic and solid lesion compressing the brain parenchyma.The young child was operated via a transparietal approach with gross total resection of the lesion.The tumor’s histology was anaplastic ependymoma.Intensive chemotherapy was given post operatively and the patient remained well without recurrence after 20 months of follow-up.
文摘In this paper,a 41-year-old female with previous history of ependymoma is reported.The patient underwent gross-total resection of the tumor and ventriculo-peritoneal shunt placement,followed by radiotherapy to the posterior fossa and the upper-cervical spinal cord region.Three years later she developed numbness in her right arm,body and leg.Magnetic resonance imaging(MRI)of the entire neuraxis revealed no evidence of tumor recurrence,while a small enhancing area was noted in the left anterolateral spinal cord at the level of the C1-C2 vertebrae and a left posterior-lateral herniated disk in the C5-C6 level which was not present in the earlier MRI at initial diagnosis.Lumbar punctures were negative for malignant cells.The patient’s symptoms were first attributed to radiation-induced effect.Follow-up results of brain and the cervical spine MRI were performed which showed disappearance of the small abnormality in the left C2 spinal cord area but persistence of the herniated C5-C6 disk.Thus,the current diagnosis of right-sided numbness due to pressure of the left anterolateral spinothalamic tracts from the herniated C5-C6 disk was made.This is a unique case,in which herniated disk pressuring effects needed to be differentiated from both radiation-induced treatment effect and tumor recurrence.
基金Supported by the Beijing Tsinghua Changgung Hospital Fund(No.12015C1045)
文摘Spinal ependymomas(SEs) are common adult intramedullary tumors; however,determining the absolute boundary between the tumor and the tumor residual may be difficult. We assessed outcomes following the use of fluorescein sodium(FS) during surgical removal of SEs. We performed a retrospective analysis of 112 patients with SEs who were treated at Beijing Tsinghua Changgung Hospital between December 2014 and December 2016. Each patient received intravenous FS(3–4 mg/kg) to determine the SE boundaries during surgery. Tumor removal efficiencies and tumor residuals were assessed using magnetic resonance imaging(MRI) at 10 days and 3 months after surgical recovery; McC ormick's spinal function classification was also performed at the 3-month follow-up. The complete tumor removal rate was 92%(103/112). Ninetyfour patients underwent tumor removal under fluorescent light, which provided distinctive tumor fluorescence. Tumor removal under white light was performed in18 patients; fluorescent images were invisible or indistinctive in these 18 patients. At the 3-month follow-up, sensory function(85.8%(91/106)) and movement(84.3%(86/102)) were improved in patients with pre-surgical dysfunction; urination and defecation functions were improved in 66.7%(16/24). The Mc Cormick spinal cord functional classifications, at the 3-month follow-up, showed significant differences in the percentages of patients with disease classified to each grade(I–IV), compared with preoperative classifications(each, P < 0.05). There was no MRI evidence of tumor relapse or residuals at the 3-month follow-up. FS use during the surgical treatment of SE enables complete tumor removal and detection of tumor residuals.
