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Ependymoma originates from ependymal cells lining the ventricles or the central canal of the spinal cord, which participate in cerebrospinal fluid circulation. It commonly occurs in the fourth ventricle, lateral ventricles, and spinal cord, and symptoms are often caused by mass effect and obstruction of cerebrospinal fluid pathways.
1. WHO grade 1
Subependymoma (often asymptomatic) and myxopapillary ependymoma (conus medullaris/filum terminale).
2. WHO grade 2
Classic ependymoma, including cellular, papillary, clear cell, and other histologic variants.
3. WHO grade 3
Anaplastic ependymoma, characterized by high proliferative activity, aggressive behavior, and poorer prognosis.
4. Supratentorial molecular subtypes
ZFTA fusion–positive and YAP1 fusion–positive tumors.
5. Posterior fossa molecular subtypes
PFA (poorer prognosis) and PFB (more favorable prognosis).
6. Spinal molecular subtypes
Including MYCN-amplified tumors and other entities.
1. Genetic factors
A minority of cases are associated with hereditary syndromes such as NF2; ZFTA and YAP1 fusions represent key oncogenic events.
2. Potential environmental factors
Rarely associated with extremely high-dose ionizing radiation; no definitive environmental etiologies have been established.
3. Other factors
The etiology is not fully defined and likely reflects accumulation of specific genetic alterations in ependymal precursor cells.
Jinshazhou Hospital of Guangzhou University of Chinese Medicine emphasizes that the harm of ependymoma arises from obstruction and compression, which can lead to intracranial hypertension and neurological dysfunction. Early, accurate MRI-based diagnosis and maximal safe gross total resection are central. Individualized adjuvant therapy and standardized MDT follow-up — especially for patients with low-grade tumors achieving complete resection — can substantially improve prognosis and enable long-term survival.
Ependymoma originates from ependymal cells lining the ventricles or the central canal of the spinal cord, which participate in cerebrospinal fluid circulation. It commonly occurs in the fourth ventricle, lateral ventricles, and spinal cord, and symptoms are often caused by mass effect and obstruction of cerebrospinal fluid pathways.
1. WHO grade 1
Subependymoma (often asymptomatic) and myxopapillary ependymoma (conus medullaris/filum terminale).
2. WHO grade 2
Classic ependymoma, including cellular, papillary, clear cell, and other histologic variants.
3. WHO grade 3
Anaplastic ependymoma, characterized by high proliferative activity, aggressive behavior, and poorer prognosis.
4. Supratentorial molecular subtypes
ZFTA fusion–positive and YAP1 fusion–positive tumors.
5. Posterior fossa molecular subtypes
PFA (poorer prognosis) and PFB (more favorable prognosis).
6. Spinal molecular subtypes
Including MYCN-amplified tumors and other entities.
1. Genetic factors
A minority of cases are associated with hereditary syndromes such as NF2; ZFTA and YAP1 fusions represent key oncogenic events.
2. Potential environmental factors
Rarely associated with extremely high-dose ionizing radiation; no definitive environmental etiologies have been established.
3. Other factors
The etiology is not fully defined and likely reflects accumulation of specific genetic alterations in ependymal precursor cells.
Jinshazhou Hospital of Guangzhou University of Chinese Medicine emphasizes that the harm of ependymoma arises from obstruction and compression, which can lead to intracranial hypertension and neurological dysfunction. Early, accurate MRI-based diagnosis and maximal safe gross total resection are central. Individualized adjuvant therapy and standardized MDT follow-up — especially for patients with low-grade tumors achieving complete resection — can substantially improve prognosis and enable long-term survival.