Ependymomas are microglial cancers that most typically develop in the ventricle system’s producing cells, but they can also develop outside the CNS or inside the tissue. They are made up of biologically different cancer clusters that afflict youngsters more frequently than elders.
Epidemiology
Ependymomas account for about five percent of older cerebral gliomas and up to ten percent of all Cerebral malignancies in children.
Ependymomas are the third leading brain tumor in adolescents. There is a maximum occurrence at the age of five, followed by a second spike at the age of thirty-four.
Anatomy
Pathophysiology
Ependymoma is a cancer that looks like regular ependymal cells and grows along the ventricular walls. Tumors can also develop in the parenchyma surrounding the ventricle or anyplace along the width of the filum terminale and spinal column.
Over sixty percent of the total ependymoma are infratentorial located, with the major subsidiaries in the posterior fossa originating mostly from the 4th ventricle. Differentiated (moderate or degree 2) or anaplastic (aggressive or degree 3) tumors are the two types of ependymomas.
The majority are cellular cancers with well-defined cytoplasmic boundaries and homogeneous polygonal particles in a collagen fiber. Tumors could also have lumps, hard exudates, and bleeding sometimes. Anaplastic ependymoma mimics Glioblastoma in appearance. High proliferation activity, endothelial growth, and apoptosis will be seen in these conditions.
Ependymoblastoma is a densely granular, embryonal kind of ependymal cancer that develops in newborns and children under the age of five. This cancer differs from other forms of ependymomas in terms of biology and pathology. The ependymoblastoma frequently propagates across the cerebrospinal fluid channels, necessitating craniospinal axis irradiation (CSA)
Etiology
While ependymomas that originate in different parts of the CNS have similar histology, their clinical history generally differs. In retrospective research, histologic grading alone has not produced consistent and accurate survival results, implying that ependymomas with equivalent histologic grades may have variable clinical outcomes.
Recent research suggests that these tumors can be classified based on various populations of progenitor cells, which could explain why tumors of the same histologic grade have variable clinical outcomes. Several genetic disorders have been linked to ependymoma and span broad genomics areas.
Some of these investigators have found that ependymomas are associated with certain oncogenic products and molecular subgroups, which may be more accurate than histologic classification in predicting clinical outcomes.
Genetics
Prognostic Factors
For intracranial ependymomas, the degree of surgical intervention has proven to be the most stable and consistent significant predictor. Intracranial ependymomas have a limited tumor progression and a histological morphology that is mostly locally invasive. Ependymomas classified as histological grade 3 by the WHO have been linked to a worse prognosis than those classified as grade 2.
Patients who get a complete resection with no evidence of advanced disease have better results and survival rates than those who just had a partial resection. As a result, surgical excision is usually used to treat the condition. Even without therapy, infratentorial ependymomas have a good prognosis across the different categories.
On the other hand, Supratentorial ependymomas frequently have a higher histologic grade and, despite surgery and prophylactic radiotherapy, have a worse survival rate. Pediatric patients’ prognoses vary depending on region, medication, and pathologic description.
Gross complete resection had the greatest overall and progression-free longevity in a previous study. Patients with WHO classification grade II showed better overall survival and progression-free survival following median total excision plus external radiation treatment, as well as after median total excision only.
After complete excision and external radiation treatment, patients with WHO classification grade 3 exhibited significantly better survivability. Patients with infratentorial malignancies who received complete excision in combination with external beam radiotherapy had a higher progression-free rate of survival.
Clinical History
Physical Examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
Hormone Therapy
Immunotherapy
Hyperthermia
Photodynamic Therapy
Stem Cell Transplant
Targeted Therapy
Palliative Care
Medication
Future Trends
Media Gallary
References
https://www.ncbi.nlm.nih.gov/books/NBK538244/
https://www.ncbi.nlm.nih.gov/books/NBK13559/
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Ependymomas are microglial cancers that most typically develop in the ventricle system’s producing cells, but they can also develop outside the CNS or inside the tissue. They are made up of biologically different cancer clusters that afflict youngsters more frequently than elders.
Ependymomas account for about five percent of older cerebral gliomas and up to ten percent of all Cerebral malignancies in children.
Ependymomas are the third leading brain tumor in adolescents. There is a maximum occurrence at the age of five, followed by a second spike at the age of thirty-four.
Ependymoma is a cancer that looks like regular ependymal cells and grows along the ventricular walls. Tumors can also develop in the parenchyma surrounding the ventricle or anyplace along the width of the filum terminale and spinal column.
Over sixty percent of the total ependymoma are infratentorial located, with the major subsidiaries in the posterior fossa originating mostly from the 4th ventricle. Differentiated (moderate or degree 2) or anaplastic (aggressive or degree 3) tumors are the two types of ependymomas.
The majority are cellular cancers with well-defined cytoplasmic boundaries and homogeneous polygonal particles in a collagen fiber. Tumors could also have lumps, hard exudates, and bleeding sometimes. Anaplastic ependymoma mimics Glioblastoma in appearance. High proliferation activity, endothelial growth, and apoptosis will be seen in these conditions.
Ependymoblastoma is a densely granular, embryonal kind of ependymal cancer that develops in newborns and children under the age of five. This cancer differs from other forms of ependymomas in terms of biology and pathology. The ependymoblastoma frequently propagates across the cerebrospinal fluid channels, necessitating craniospinal axis irradiation (CSA)
While ependymomas that originate in different parts of the CNS have similar histology, their clinical history generally differs. In retrospective research, histologic grading alone has not produced consistent and accurate survival results, implying that ependymomas with equivalent histologic grades may have variable clinical outcomes.
Recent research suggests that these tumors can be classified based on various populations of progenitor cells, which could explain why tumors of the same histologic grade have variable clinical outcomes. Several genetic disorders have been linked to ependymoma and span broad genomics areas.
Some of these investigators have found that ependymomas are associated with certain oncogenic products and molecular subgroups, which may be more accurate than histologic classification in predicting clinical outcomes.
For intracranial ependymomas, the degree of surgical intervention has proven to be the most stable and consistent significant predictor. Intracranial ependymomas have a limited tumor progression and a histological morphology that is mostly locally invasive. Ependymomas classified as histological grade 3 by the WHO have been linked to a worse prognosis than those classified as grade 2.
Patients who get a complete resection with no evidence of advanced disease have better results and survival rates than those who just had a partial resection. As a result, surgical excision is usually used to treat the condition. Even without therapy, infratentorial ependymomas have a good prognosis across the different categories.
On the other hand, Supratentorial ependymomas frequently have a higher histologic grade and, despite surgery and prophylactic radiotherapy, have a worse survival rate. Pediatric patients’ prognoses vary depending on region, medication, and pathologic description.
Gross complete resection had the greatest overall and progression-free longevity in a previous study. Patients with WHO classification grade II showed better overall survival and progression-free survival following median total excision plus external radiation treatment, as well as after median total excision only.
After complete excision and external radiation treatment, patients with WHO classification grade 3 exhibited significantly better survivability. Patients with infratentorial malignancies who received complete excision in combination with external beam radiotherapy had a higher progression-free rate of survival.
https://www.ncbi.nlm.nih.gov/books/NBK538244/
https://www.ncbi.nlm.nih.gov/books/NBK13559/
medtigo
Ependymoma
Updated :
June 16, 2022
Ependymomas are microglial cancers that most typically develop in the ventricle system’s producing cells, but they can also develop outside the CNS or inside the tissue. They are made up of biologically different cancer clusters that afflict youngsters more frequently than elders.
Ependymomas account for about five percent of older cerebral gliomas and up to ten percent of all Cerebral malignancies in children.
Ependymomas are the third leading brain tumor in adolescents. There is a maximum occurrence at the age of five, followed by a second spike at the age of thirty-four.
Ependymoma is a cancer that looks like regular ependymal cells and grows along the ventricular walls. Tumors can also develop in the parenchyma surrounding the ventricle or anyplace along the width of the filum terminale and spinal column.
Over sixty percent of the total ependymoma are infratentorial located, with the major subsidiaries in the posterior fossa originating mostly from the 4th ventricle. Differentiated (moderate or degree 2) or anaplastic (aggressive or degree 3) tumors are the two types of ependymomas.
The majority are cellular cancers with well-defined cytoplasmic boundaries and homogeneous polygonal particles in a collagen fiber. Tumors could also have lumps, hard exudates, and bleeding sometimes. Anaplastic ependymoma mimics Glioblastoma in appearance. High proliferation activity, endothelial growth, and apoptosis will be seen in these conditions.
Ependymoblastoma is a densely granular, embryonal kind of ependymal cancer that develops in newborns and children under the age of five. This cancer differs from other forms of ependymomas in terms of biology and pathology. The ependymoblastoma frequently propagates across the cerebrospinal fluid channels, necessitating craniospinal axis irradiation (CSA)
While ependymomas that originate in different parts of the CNS have similar histology, their clinical history generally differs. In retrospective research, histologic grading alone has not produced consistent and accurate survival results, implying that ependymomas with equivalent histologic grades may have variable clinical outcomes.
Recent research suggests that these tumors can be classified based on various populations of progenitor cells, which could explain why tumors of the same histologic grade have variable clinical outcomes. Several genetic disorders have been linked to ependymoma and span broad genomics areas.
Some of these investigators have found that ependymomas are associated with certain oncogenic products and molecular subgroups, which may be more accurate than histologic classification in predicting clinical outcomes.
For intracranial ependymomas, the degree of surgical intervention has proven to be the most stable and consistent significant predictor. Intracranial ependymomas have a limited tumor progression and a histological morphology that is mostly locally invasive. Ependymomas classified as histological grade 3 by the WHO have been linked to a worse prognosis than those classified as grade 2.
Patients who get a complete resection with no evidence of advanced disease have better results and survival rates than those who just had a partial resection. As a result, surgical excision is usually used to treat the condition. Even without therapy, infratentorial ependymomas have a good prognosis across the different categories.
On the other hand, Supratentorial ependymomas frequently have a higher histologic grade and, despite surgery and prophylactic radiotherapy, have a worse survival rate. Pediatric patients’ prognoses vary depending on region, medication, and pathologic description.
Gross complete resection had the greatest overall and progression-free longevity in a previous study. Patients with WHO classification grade II showed better overall survival and progression-free survival following median total excision plus external radiation treatment, as well as after median total excision only.
After complete excision and external radiation treatment, patients with WHO classification grade 3 exhibited significantly better survivability. Patients with infratentorial malignancies who received complete excision in combination with external beam radiotherapy had a higher progression-free rate of survival.
https://www.ncbi.nlm.nih.gov/books/NBK538244/
https://www.ncbi.nlm.nih.gov/books/NBK13559/
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