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Neurology India 2016An ependymoma is an uncommon glial tumor, which arises from different parts of the neuroaxis. Considerable variation in presentation and survival in tumors in different...
An ependymoma is an uncommon glial tumor, which arises from different parts of the neuroaxis. Considerable variation in presentation and survival in tumors in different locations after an optimum treatment indicates inherent molecular and genetic differences in tumorigenesis between them. A number of genetic aberrations have been identified to distinctly characterize different subgroups of ependymomas that include a posterior fossa tumor, a supratentorial tumor, and a pediatric tumor. These different groups have substantial genetic alterations, and also distinct demography, clinical characteristics, and prognosis. This article is intended to review the diverse molecular and genetic aberrations that may be helpful in prognostication and prediction of survival in patients suffering from an ependymoma.
Topics: Brain Neoplasms; Ependymoma; Genetic Markers; Humans; Infratentorial Neoplasms; Prognosis; Supratentorial Neoplasms
PubMed: 26954807
DOI: 10.4103/0028-3886.177630 -
Neuro-oncology Jul 2022Radiotherapy (RT) of ependymoma in children is an important part of the interdisciplinary treatment concept. However, feasibility and dose concepts are still under...
BACKGROUND
Radiotherapy (RT) of ependymoma in children is an important part of the interdisciplinary treatment concept. However, feasibility and dose concepts are still under investigation, particularly in very young children. The aim of this study was to evaluate the standard dose and volume of proton therapy (PT) in children with ependymoma.
METHODS
In this analysis, 105 patients with localized, intracranial ependymoma under the age of 18 years treated with PT between 2013 and 2018 were included. Patient characteristics, treatment, outcome, and follow-up data were analyzed using descriptive statistics, Kaplan-Meier, and Cox regression analysis.
RESULTS
The median age of patients at PT was 2.8 years (0.9-17.0 years). The molecular subgroup analysis was performed in a subset of 50 patients (37 EP-PFA, 2 EP-PFB, 7 EP-RELA, 2 EP-YAP, 2 NEC [not elsewhere classified]). The median total dose was 59.4 Gy (54.0-62.0 Gy). The median follow-up time was 1.9 years. The estimated 3-year overall survival (OS), local control (LC), and progression-free survival (PFS) rates were 93.7%, 74.1%, and 55.6%, respectively. Within univariable analysis, female gender and lower dose had a positive impact on OS, whereas age ≥4 years had a negative impact on OS and PT given after progression had a negative impact on PFS. In the multivariable analysis, multiple tumor surgeries were associated with lower PFS. New ≥3° late toxicities occurred in 11 patients.
CONCLUSION
For children with localized ependymoma, PT was effective and well tolerable. Multiple surgeries showed a negative impact on PFS.
Topics: Adolescent; Brain Neoplasms; Child; Child, Preschool; Ependymoma; Female; Humans; Prospective Studies; Proton Therapy; Treatment Outcome
PubMed: 34964901
DOI: 10.1093/neuonc/noab301 -
Neurological Sciences : Official... Aug 2022The aim of this study is to compare the use of 5-aminolevulinic acid (5-ALA) and sodium fluorescein (SF) in IV ventricular ependymoma (IVEP) surgical resection.
PURPOSE
The aim of this study is to compare the use of 5-aminolevulinic acid (5-ALA) and sodium fluorescein (SF) in IV ventricular ependymoma (IVEP) surgical resection.
METHODS
In this retrospective study, six patients with IVEP were enrolled. Gender ratio 2:1 male to female, with mean age 38.9 years old. A 5-ALA oral dose of 20 mg/kg and a SF intravenous dose of 2 mg/kg were administered. Telo-velar approach, operative microscope, and intraoperative monitoring were used in all the operations. We retrospectively compared the two fluorescence techniques at four steps during the surgical procedure: step 1: exposure of the tumor; step 2: dissection of the lesion from the cerebellum; step 3: assessment of the tumor borders and differentiation from normal tissue at the base of implants; and step 4: evaluation of possible residual tissue in the surgical cavity.
RESULTS
At the first step, the ependymomas resulted well delineated by both fluorescent agents. In this step, 5-ALA was particularly helpful in the case of recurrent ependymoma. At step 2, 5-ALA provided a better identification of the ependymoma boundaries and distinction from cerebellum hemispheres than SF. In steps 3 and 4, SF was really helpful to detect tumor tissue.
CONCLUSION
According to our experience, fluorescence-guided surgery of IVEP with 5-ALA and SF is useful to maximize surgical resection with less risk of brainstem injury. Both fluorescence techniques are helpful in different steps of IVEP resection. However, further studies are needed to confirm our preliminary data.
Topics: Adult; Aminolevulinic Acid; Brain Neoplasms; Ependymoma; Female; Fluorescein; Humans; Male; Neoplasm Recurrence, Local; Neurosurgical Procedures; Retrospective Studies
PubMed: 35334013
DOI: 10.1007/s10072-022-06012-z -
Child's Nervous System : ChNS :... Jul 2021Evidence exists, in CNS germinomas and medulloblastomas (MB), that patient sex significantly influences incidence and outcome. The role of sex genotype in other...
PURPOSE
Evidence exists, in CNS germinomas and medulloblastomas (MB), that patient sex significantly influences incidence and outcome. The role of sex genotype in other paediatric CNS tumours remains unclear. This study sought to examine the role of sex genotype in CNS tumour incidence and overall survival (OS).
METHODS
Age-adjusted incidence and OS rates were collected from the Surveillance Epidemiology and End Result (SEER) registry between 2000 and 2011 for common paediatric (<=19 years) CNS tumours: pilocytic astrocytoma (PA), anaplastic astrocytoma, glioblastoma (GBM), medulloblastoma, supratentorial CNS embryonal tumour, ependymoma, and germinoma. All patients with histologically confirmed, ICD-03 coded, first tumours, were included. Kaplan-Meier and Cox regression analyses were used to calculate hazard ratios (HR).
RESULTS
The total cases are as follows: males=3018 and females=2276. Highest incidence was seen in PA (n=2103). GBM displayed the worst OS, whilst PA displayed the best. Higher incidence was observed in males for all tumours, except PA. Females with ependymoma had significantly better OS compared to males, whereas males with germinomas had better OS compared to females. Females <1 year with AA had better OS than males. Increasing age significantly improved male and female survival in ependymoma and medulloblastoma.
CONCLUSION
Interrogating population-based registries such as SEER minimises bias and provides credible data. Observed differences in incidence and OS between the sexes for different paediatric CNS tumours provide useful prognostic information for clinicians. Sex genotype was a significant independent prognostic factor in ependymomas and germinomas. Further investigation of possible epigenetic and hormonal differences may provide sex-specific vulnerabilities that may be exploitable for targeted therapy.
Topics: Central Nervous System Neoplasms; Cerebellar Neoplasms; Child; Ependymoma; Female; Genotype; Humans; Incidence; Infant; Male
PubMed: 33950317
DOI: 10.1007/s00381-021-05165-0 -
Annals of Saudi Medicine 2020There is limited data from Saudi Arabia on the demographic characteristics, outcomes and effectiveness of different treatment modalities in children with intracranial...
BACKGROUND
There is limited data from Saudi Arabia on the demographic characteristics, outcomes and effectiveness of different treatment modalities in children with intracranial ependymoma.
OBJECTIVE
Study the characteristics of pediatric ependymoma and outcomes of treatment modalities in Saudi Arabia.
DESIGN
Retrospective.
SETTING
Tertiary care center.
PATIENTS AND METHODS
Children with intracranial ependymoma who were younger than 14 years of age and treated between 2006 and 2015 were included in the study. Patients with prior radiation, chemo-therapy, or surgical resection at other centers were excluded.
MAIN OUTCOME MEASURES
Kaplan-Meier survival curves were used to estimate the event-free (EFS) and overall survival (OS) rates of the patients.
SAMPLE SIZE
22.
RESULTS
Of the 22 children, 4 (18.2%) were less than three years old. All intracranial ependymomas had upfront surgical resection of the primary tumor. Gross total resection was achievable in 9 (42.9%) cases and subtotal resection in another 9 (42.9%). Near-total resection was done in 3 (14.3%) cases. Median time from surgery to start of radiotherapy was 62 days. RT was given to 17 (77.3%) patients. Both mean and median RT dose was 55.8 Gy. Only 5 (22.7%) of the children received chemotherapy. The median duration of follow-up was 5.38 years and the median time for EFS was 2.27 years. The cumulative OS rate of the study was 44.5%. The cumulative EFS survival rate of the study was 18.6%. Among demographic, pathological, radiological features, none had a statistically significant effect on the survival.
CONCLUSIONS
The outcomes are comparable to those reported by international investigators for similar populations. Further improvements can be achieved by avoiding delays in radiation therapy and adding molecular staging.
LIMITATIONS
The limited number of cases, retrospective nature, lack of molecular biology and size of the tumors.
CONFLICT OF INTEREST
None.
Topics: Brain Neoplasms; Child; Child, Preschool; Disease-Free Survival; Ependymoma; Female; Humans; Kaplan-Meier Estimate; Male; Retrospective Studies; Saudi Arabia; Tertiary Care Centers; Treatment Outcome
PubMed: 33307736
DOI: 10.5144/0256-4947.2020.482 -
Cancer Immunology, Immunotherapy : CII Feb 2019Ependymomas are biologically and clinically heterogeneous tumors of the central nervous system that have variable clinical outcomes. The status of the tumor immune...
Tumor-infiltrating immune cell subpopulations and programmed death ligand 1 (PD-L1) expression associated with clinicopathological and prognostic parameters in ependymoma.
Ependymomas are biologically and clinically heterogeneous tumors of the central nervous system that have variable clinical outcomes. The status of the tumor immune microenvironment in ependymoma remains unclear. Immune cell subsets and programmed death ligand 1 (PD-L1) expression were measured in 178 classical ependymoma cases by immunohistochemistry using monoclonal antibodies that recognized tumor-infiltrating lymphocyte subsets (TILs; CD3, CD4, CD8, FOXP3, and CD20), tumor-associated macrophages (TAMs; CD68, CD163, AIF1), indoleamine 2,3-dioxygenase (IDO)+ cells and PD-L1-expressing tumor cells. Increases in CD3+ and CD8+ cell numbers were associated with a prolonged PFS. In contrast, increased numbers of FOXP3+ and CD68+ cells and a ratio of CD163/AIF1+ cells were significantly associated with a shorter PFS. An increase in the IDO+ cell number was associated with a significantly longer PFS. To consider the quantities of TILs, TAMs, and IDO+ cells together, the cases were clustered into 2 immune cell subgroups using a k-means clustering analysis. Immune cell subgroup A, which was defined by high CD3+, low CD68+ and high IDO+ cell counts, predicted a favorable PFS compared to subgroup B by univariate and multivariate analyses. We found six ependymoma cases expressing PD-L1. All these cases were supratentorial ependymoma, RELA fusion-positive (ST-RELA). PD-L1 expression showed no prognostic significance. This study showed that the analysis of tumor-infiltrating immune cells could aid in predicting the prognosis of ependymoma patients and in determining therapeutic strategies to target the tumor microenvironment. PD-L1 expression in the ST-RELA subgroup suggests that this marker has a potential added value for future immunotherapy treatments.
Topics: Adolescent; Adult; Aged; B7-H1 Antigen; Biomarkers, Tumor; Child; Child, Preschool; Ependymoma; Female; Humans; Infant; Infant, Newborn; Kaplan-Meier Estimate; Lymphocytes, Tumor-Infiltrating; Male; Middle Aged; Prognosis; T-Lymphocyte Subsets; Tumor Microenvironment; Young Adult
PubMed: 30483834
DOI: 10.1007/s00262-018-2278-x -
JPMA. the Journal of the Pakistan... Nov 2022Although myxopapillary ependymoma is a fairly common tumour of the lumbosacral spine, primary multi-focal myxopapillary ependymoma is a rare variant. Drop metastasis and...
Although myxopapillary ependymoma is a fairly common tumour of the lumbosacral spine, primary multi-focal myxopapillary ependymoma is a rare variant. Drop metastasis and leptomeningeal spread in the craniospinal axis is seen more frequently in the paediatric population, although it is unusual in adults. Surgical resection of the primary lesion remains the standard treatment. As per the authors' knowledge, to-date there is only one prior case in literature reporting iatrogenic spinal cord herniation with indentation after surgery for thoracolumbar spinal tumour. Here, we are discussing an unusual case of primary multi-focal ependymoma in a 16-year-old Asian boy, with drop metastasis and lepto-meningeal disease, who developed iatrogenic spinal cord herniation after the first surgery for the primary tumour. He presented to the Shaukat Khanum Memorial Cancer Hospital & Research Centre (SKMCH & RC), Lahore, after his first surgery. He underwent the definitive corrective surgery at SKMCH & RC where he was managed further. We discuss the management options for this patient and the lessons learned along the way.
Topics: Male; Adult; Child; Humans; Adolescent; Spinal Cord Neoplasms; Ependymoma; Spinal Neoplasms; Iatrogenic Disease
PubMed: 37013313
DOI: 10.47391/JPMA.4465 -
BMC Pregnancy and Childbirth Nov 2020Ovarian ependymoma is a rare malignancy. Because of the extreme rarity, certain features of the neoplasm, including its clinical behaviour and optimal therapy, are... (Review)
Review
BACKGROUND
Ovarian ependymoma is a rare malignancy. Because of the extreme rarity, certain features of the neoplasm, including its clinical behaviour and optimal therapy, are incompletely characterized.
CASE PRESENTATION
A 32-year-old pregnant woman at term presented with a left ovarian neoplasm that occurred in the early stage of pregnancy. She underwent left adnexectomy during the caesarean section, and the neoplasm was histologically and immunohistochemically identified to be ovarian ependymoma. Immunohistochemical staining with oestrogen receptors and progesterone receptors showed strong positive staining. According to reports in the literature, the pathological type of ovarian ependymoma in our patient was the extra-axial type. Interestingly, the foetus was also found to have bilateral ependymal cysts during pregnancy. The patient received no further surgical treatment or adjuvant therapy. She and her 14-month-old baby both have no evidence of disease at present. The follow-up of both mother and child is still continuing.
CONCLUSIONS
The case presented here illustrates high levels of oestrogen during pregnancy may stimulate viable malignant ependymal cells to proliferate. Hence, young women with extra-axial-type ependymomas may not be suitable for fertility preservation. Moreover, hormone-based therapies can be a potentially effective treatment for women with extra-axial ependymomas.
Topics: Adult; Cesarean Section; Ependymoma; Female; Humans; Magnetic Resonance Imaging; Ovarian Neoplasms; Ovariectomy; Ovary; Pregnancy; Pregnancy Complications, Neoplastic; Rare Diseases; Treatment Outcome
PubMed: 33208128
DOI: 10.1186/s12884-020-03408-7 -
Journal of Neuro-oncology May 2024Primary treatment of spinal ependymomas involves surgical resection, however recurrence ranges between 50 and 70%. While the association of survival outcomes with lesion...
PURPOSE
Primary treatment of spinal ependymomas involves surgical resection, however recurrence ranges between 50 and 70%. While the association of survival outcomes with lesion extent of resection (EOR) has been studied, existing analyses are limited by small samples and archaic data resulting in an inhomogeneous population. We investigated the relationship between EOR and survival outcomes, chiefly overall survival (OS) and progression-free survival (PFS), in a large contemporary cohort of spinal ependymoma patients.
METHODS
Adult patients diagnosed with a spinal ependymoma from 2006 to 2021 were identified from an institutional registry. Patients undergoing primary surgical resection at our institution, ≥ 1 routine follow-up MRI, and pathologic diagnosis of ependymoma were included. Records were reviewed for demographic information, EOR, lesion characteristics, and pre-/post-operative neurologic symptoms. EOR was divided into 2 classifications: gross total resection (GTR) and subtotal resection (STR). Log-rank test was used to compare OS and PFS between patient groups.
RESULTS
Sixty-nine patients satisfied inclusion criteria, with 79.7% benefitting from GTR. The population was 56.2% male with average age of 45.7 years, and median follow-up duration of 58 months. Cox multivariate model demonstrated significant improvement in PFS when a GTR was attained (p <.001). Independently ambulatory patients prior to surgery had superior PFS (p <.001) and OS (p =.05). In univariate analyses, patients with a syrinx had improved PFS (p =.03) and were more likely to benefit from GTR (p =.01). Alternatively, OS was not affected by EOR (p =.78).
CONCLUSIONS
In this large, contemporary series of adult spinal ependymoma patients, we demonstrated improvements in PFS when GTR was achieved.
Topics: Humans; Male; Ependymoma; Female; Middle Aged; Adult; Spinal Cord Neoplasms; Neurosurgical Procedures; Progression-Free Survival; Follow-Up Studies; Retrospective Studies; Survival Rate; Young Adult; Aged; Prognosis; Adolescent
PubMed: 38438766
DOI: 10.1007/s11060-024-04623-4 -
Journal of Clinical Neuroscience :... Mar 2015Ependymoma is a relatively rare malignancy accounting for 2.0% of all primary central nervous system tumors in adults. Extracranial metastasis is a very uncommon...
Ependymoma is a relatively rare malignancy accounting for 2.0% of all primary central nervous system tumors in adults. Extracranial metastasis is a very uncommon complication of gliomas, especially of anaplastic ependymomas. The objective of this paper is to show that ependymomas can metastasize to soft tissue and lymph nodes as well as to share our approach to this challenge. We report a male patient with anaplastic ependymoma that recurred, metastasizing to the neck and lymph nodes. Metastatic disease was diagnosed based on clinical presentation of a palpable nodule on the right neck and diffuse cervical lymphadenopathies. A biopsy was obtained and pathology revealed anaplastic ependymoma. Whole-body fluorodeoxyglucose positron emission tomography scan showed metastatic disease in the right mastoid region with diffuse uptake in the cervical lymph nodes. Clinical and radiologic response was achieved after three chemotherapy cycles of etoposide, cisplatin, vincristine, and cyclophosphamide. This case highlights extracranial metastasis to the soft tissue as an atypical presentation of recurrent anaplastic ependymoma. Other reported instances of extracranial metastatic ependymoma with this presentation are discussed. The possible metastatic pathways of intracranial disease are discussed. It also illustrates how extracranial disease remains stable with systemic chemotherapy.
Topics: Brain Neoplasms; Ependymoma; Humans; Lymphatic Metastasis; Male; Neck; Soft Tissue Neoplasms; Young Adult
PubMed: 25455735
DOI: 10.1016/j.jocn.2014.09.006