Clinical analysis of 14 cases of supratentorial parenchymal ependymomas

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,

Prognostic analysis of intramedullary ependymomas in medulla oblongata and cervical cord

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

Ventricular system-unrelated cerebellar ependymoma:A case report

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.

Lipomatous ependymoma with ZFTA:RELA fusion-positive:A case report

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.

Hydrocephalus Secondary to Intraventricular Myxopapillary Ependymoma: Case Report

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.

Aszonapyrone A Isolated from Neosartorya spinosa IFM 47025 Inhibits the NF-κB Signaling Pathway Activated by Expression of the Ependymoma-Causing Fusion Protein ZFTA-RELA

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.

Treatment implications of posterior fossa ependymoma subgroups

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.

Invasive myxopapillary ependymoma of the lumbar spine: A case report

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.

Expression and sub-cellular localization of leucine-rich repeats and immunoglobulin-like domains are related to antioxidant enzymes in human ependymoma and oligodendroglioma

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.

Anaplastic myxopapillary ependymoma:A case report and review of literature

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.

Cognitive and Psychological Outcomes in Younger vs. Older Children with Subtentorial/Supratentorial Ependymoma after Radiation Therapy

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.

A Report of Two Cases of Tuberous Sclerosis Combined with Subependymal Giant-Cell Astrocytoma

Case 1, was a 23 year old female. The chief complaints were headache, vomiting for over 20 days, which was aggravated upon exercising, and confusion for 5 days. The patient was retarded and had a history of epilepsy for 20 years. Her family said her intelligence was that of a 3 year-old child. Many red papules were dispersed on her cheeks. A highly thick 2.2 cm by 2.3 cm by 2.4 cm round-like neoplasm was found by CT scanning in the body of the left ventricle near the interventricle foramen, and there was a spot-like calcification in the tumor. There was also a spot-like calcification （Fig.1） in a shuttle-like slightly high thickness node at the same place on the opposite side. The above-mentioned neoplasms enhanced evenly （Fig.2）. CT diagnosis： tuberous sclerosis combined with subependymal giant-cell astrocytoma. A 2.0 cm by 2.0 cm by 3.0 cm tumor was seen near the interventricle foramen in the body of the left ventricle and frontal horn upon operation. The tumor had a wine-red color, with a fairly clear border, a tough nature an ample blood supply and calcification. Pathologic diagnosis： subependymal giant-cell astrocytoma.

Supra-Tentorial Cortical Ependymoma in an Adult: Case Report and Review of the Literature

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.

Extracranial Metastasis of an Anaplastic Ependymoma, RELA Fusion-Positive: A Rare Occurrence

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.

Intradural Extra Medullary Cervical Anaplastic Ependymoma. Case Report and Review of the Literature

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.

Myxopapillary Ependymoma of the III Ventricle: Example of a Histological Variant of Ependymoma with Exceptional Localization

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.

Recent Techniques in Management of Lumbosacral Region Tumours: A Prospective Comparative Study

Background: The lumbosacral plexus consists of the ventral rami from the L2 to S3 nerve roots, with some additions from the L1 and S4 nerve roots as well. The standard treatment for spinal cord compression is urgent surgically decompressive laminectomy over the affected level with resection as much as possible of the exposed tumor to relieve the cord compression. We aim to correlate the impact of preoperative clinical picture, duration of symptoms, and the pathological nature of the lumbo-sacral spine tumors on the outcome of comparative study different modalities of management. Patients and Methods: During the period between 2015 and 2017, thirty cases were studied and surgically managed in Al-Azhar University Hospital, Damietta. Cases were selected for surgery if they were diagnosed as having a symptomatizing lumbosacral lesion. Results: Thirty patients were included in the current study;the commonest lesion in this study was ependymoma eight patients (27%), astrocytoma six patients (20%), and meningioma five patients (17%). Nerve sheath tumor comprised five cases (17%) with the hemangioblastomas three cases (10%), epidermoid one case (3%), and ependymoma of the filum terminale two cases (6%). The mean age of the included patients was 38.96 ± 15.42. The mean duration of lesions was 13.66 ± 10.93. They were 15 male patients (50%), and 15 female patients (50%), with male: female ratio 1:1. The mean duration of symptoms ranged for 1:60 months. The most common presenting manifestations were sensory followed by motor and sphinctric disturbance. Complications occurred in 8 patients (23%), and the most common was postoperative CSF leak. 19 out of 30 cases were surgically totally resected (63%), while 11 cases (37%) were grossly totally resected. Conclusions: Ependymoma and meningioma have more favorable outcome compared to other spinal tumors. Accurate and rapid diagnosis prior to surgery also associated with good outcome.

Surgical Management of Intramedullary Spinal Cord Tumors: Surgical Resection and Prognosis

BACKGROUND & OBJECTIVE: The majority of intramedullary spinal cord tumors (IMSCT) are low-grade gliomas. Radical resection for IMSCTs remains challenging. Recently, improved neuroimaging and advanced microsurgical technique have made great success in surgical management of the intramedullary spinal cord tumors. METH-ODS & RESULTS: Twenty-nine patients with intramedullary spinal cord tumors were treated by radical resection dur-ing the past 4 years in our institute. The histological results were as follows: 12 ependymomas, 4 astrocytomas, 4 heman-gioblastomas, 4 epidermoids, 1 cavernoma, 2 lipomas, 2 metastatics. A gross -total resection (】 95%) was achieved in 25 surgical procedures. Subtotal resections (80-95% ) were performed in 4 cases. There was no surgical death.When comparing the preoperative and 3-month postoperative functional grades, 12 patients were stable 14 improved,and 3 deteriorated. Patients with either no deficit or only mild deficit before surgery were rarely impaired by the proce-dure, reinforcing the importance of early diagnosis and treatment. The major determinant of long-term survival was histologi-cal composition of the tumor. Patients in whom an IMSCT was only partially resected (【80% ) fared significantly worse. CONCLUSIONS: The long-term survival and quality of life for patients with low-grade gliomas treated by radi-cal resection alone is comparable or superior to minimal resection plus radiotherapy. The optimal therapy for patients with high-grade glioma is yet to be determined. For benign lesion, such as hemangioblastoma and cavernoma could be cured by total resection of the tumor. For lipoma and epidermoid, fibrous adhesions to the cord make total removal difficult,and thus, removal is not the goal of surgery. The carbon dioxide laser is particularly useful during surgery for this lesion.

Seldom-segment versus multi-segment intramedullary spinal cord gliomas: a comparative clinical study

Dear Editor,Glial tumors (80%), astrocytomas, and ependymomas are the most frequent histological types of intramedullary spinal cord tumors (Bansal et al., 2013). Intramedullary tumors spanning three or more vertebral body levels are considered multi-segment intramedullary spinal cord tumors and have a much lower incidence than less involved tumors (Wang et al., 2012). We prospectively studied 81 consecutive patients who underwent resection of intramedullary spinal cord gliomas at a single medical center (The Neurosurgery Department of Peking University Third Hospital) between January 2010 and December 2015 to compare the neurological function and prognosis of patients with seldom-segment and multiple-segment intramedullary spinal cord gliomas.

Two Cases of Spinal Tanycytic Ependymoma Associated with Neurofibromatosis Type 2

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.