Ependymoma

Ependymomas represent a relatively broad group of glial tumors most often arising from the lining the ventricles of the brain or the central canal of the spinal cord. They account for ~5% of all neuroepithelial neoplasms, ~10% of all pediatric brain tumors and up to 33% of brain tumors occurring in those less than 3 years of age. 

Ependymomas can occur anywhere within the neuraxis, but distribution is not even, with the posterior fossa being most common, followed by supratentorium and lastly spinal cord. This distribution correlates with molecularly distinct tumors which in turn have different epidemiology and prognosis 11,13:

The remainder of this article concerns itself with intracranial ependymomas. 

Although there is no overall recognized gender predilection when all ependymomas are treated as a group 3, each subgroup does have different gender and age predilection 13

Again, if treated as a group, ependymomas can occur at any age, the posterior fossa tumors tend to present more commonly in the pediatric age group (mean age at diagnosis is 6 years of age), with a smaller second peak for supratentorial tumors around the 3rd decade 7

Each molecular subgroup has different epidemiological profile 13:

  • infratentorial
    • Posterior Fossa A
      • M:F = 2:1
      • age: young children
    • Posterior Fossa B
      • M:F = 1:1
      • age: older children and teenagers
  • supratentorial

Clinical presentation can vary according to location. Initial presentation with signs and symptoms of raised intracranial pressure is common, particularly with tumors in the fourth ventricle. Other posterior fossa symptoms including ataxia are also encountered 7. Supratentorial ependymomas may also present with seizures or focal neurological deficits 2,5.

In the infrequent scenario of hemorrhage, the presentation will be hyper-acute. 

Ependymomas are glial tumors with ependymal differentiation which tend to arise within or abutting the ventricular system of the brain or central canal of the spinal cord 7,11. Although for many years ependymomas were believed to be tumors arising from dedifferentiated ependymocytes, it now appears fairly certain that in fact, they arise from radial glial stem cells 9-11

Macroscopically, ependymomas tend to be well defined lobulated grey or tan-colored soft and frond-like tumors which are moderately cellular. They may have focal areas of calcification. 

Microscopically, these tumors are characterized by well-differentiated cells. Characteristic features include ependymal rosettes, which are uncommon but pathognomonic and perivascular pseudorosettes which are far more common and seen in most of ependymomas 8,11.

Dystrophic calcification, hemorrhage, myxoid degeneration and even rarely metaplasia (bone or cartilage) are sometimes encountered 11

A number of immunohistochemical markers are useful, including 11,12:

Ependymomas are WHO grade II tumors, with more histologically aggressive tumors denoted WHO III (anaplastic ependymoma), although the reliability of histological features in grading these tumors and the prognostic significance is somewhat controversial, and recently there has been a shift towards molecular markers to subdivide ependymomas 11

In the current (2016 update) WHO classification of CNS tumors the following entities are accepted as belonging to the ependymoma family of tumors 11

Note: the 2016 update to WHO classification of CNS tumors has deleted the variant cellular ependymoma as it was felt that it overlapped that of a standard ependymoma too closely 11

Increasingly, as is the case with many other tumors, molecular classification is proving to be more important than histological classification in predicting treatment response and prognosis. Intracranial ependymomas can be divided into four main groups that correlate largely with location 13:

  • infratentorial
    • Posterior Fossa A: H3 K27 trimethylation
    • Posterior Fossa B
  • supratentorial

The majority of intracranial ependymomas (60%) are located in the posterior fossa (infratentorial), usually arising from the lateral recess of the fourth ventricle (molecular subgroup: Posterior Fossa A) and midline inferior floor of the fourth ventricle near the obex (molecular subgroup: Posterior Fossa B) 5-7,13.

The remainder (40%) are located supratentorially and up to half of these are intraparenchymal 7

Posterior fossa ependymomas are apt to extend through the foramina of Luschka and Magendie, hence the term plastic ependymoma. This is a characteristic feature and can be seen on both CT and MRI. 

Ependymomas are typically heterogeneous masses with areas of necrosis, calcification, cystic change and hemorrhage frequently seen. This results in a heterogeneous appearance on all modalities. 

Intraparenchymal lesions (usually supratentorial) are generally large and variable in appearance, ranging from completely solid, enhancing masses to cysts with a mural nodule, or more heterogeneous masses 7. They are believed to arise from trapping of embryonic rests of ependymal tissue in the developing cerebral parenchyma 5.

  • coarse calcification is common (50%) 
  • cystic areas (50%) 
  • solid component iso- to hypodense
  • heterogeneous enhancement 
  • variable hemorrhage
  • T1
    • solid portions of ependymoma typically are isointense to hypointense relative to white matter 7
  • T2
    • hyperintense to white matter
    • more reliable in differentiating tumor margins than non-contrast T1-weighted images (but less reliable than contrast enhanced T1)
  • T2* (e.g. SWI)
    • foci of blooming from hemorrhage or calcification
  • T1 C+ (Gd)
    • enhancement present but heterogeneous
    • enhancement with gadolinium is useful in differentiating tumor from adjacent vasogenic edema and normal brain parenchyma
  • DWI/ADC
    • restricted diffusion may be seen in solid components, especially in anaplastic tumor
    • diffusion should be interpreted with caution in masses with significant hemorrhage or calcification
  • MRS

Although it is uncommon when compared to tumors like medulloblastomas, careful examination of the entire neuraxis is required to assess for the presence of CSF seeding. 

A total or partial resection could be attempted +/- irradiation.

Prognosis is, however, relatively poor, which is mainly due to tumors occurring in surgically challenging locations, making complete resection difficult. 

Poor prognostic factors include a 4th ventricular location, anaplastic variant and incomplete resection. As such, children have a worse prognosis (both 4th ventricular location and anaplastic variant are more common in children). Overall, the 5-year survival rate in children ranges from 50 to 75% 7

Once recurrence has occurred, the prognosis is very poor, with a mortality rate of 90% 7

The molecular subgroup has a have a significant impact on prognosis 13:

  • infratentorial
    • Posterior Fossa A: poor
    • Posterior Fossa B: good
  • supratentorial

General imaging differential considerations include:

  • medulloblastoma 
    • similar demographic, especially if around the 4th ventricle
    • arises from vermis
    • less 'plastic', does not tend to extend through foramina
    • enhancement more homogenous
    • calcification less common
  • subependymoma
    • tends to occur in older individuals
    • usually not enhancing
  • choroid plexus papilloma
    • in children usually in the trigone of the lateral ventricles
    • in adults usually in the fourth ventricle (i.e. opposite to ependymoma)
    • more vividly and homogeneously enhancing
    • lacks adjacent parenchymal edema
    • NB choroid plexus carcinoma can be heterogeneous and invade the brain
  • choroid plexus metastasis
    • can appear similar
    • older individuals, usually with a history of malignancy
  • glioblastoma
    • difficult to distinguish from intraparenchymal supratentorial ependymoma
    • usually older patients
    • epicenter usually in the white matter
  • central neurocytoma
    • usually arises from/in contact with septum pellucidum
    • less vivid enhancement
  • atypical teratoid/rhabdoid tumor
Share article

Article information

rID: 1290
Synonyms or Alternate Spellings:
  • Ependymomas

Support Radiopaedia and see fewer ads

Cases and figures

  • Figure 1: ependymal rosettes
    Drag here to reorder.
  • Case 1: classical features, posterior fossa
    Drag here to reorder.
  • Figure 2: EMA positive perinuclear dot
    Drag here to reorder.
  • Case 2: on T2W MRI
    Drag here to reorder.
  • Case 3: MRS
    Drag here to reorder.
  • Case 4: in a child, posterior fossa
    Drag here to reorder.
  • Case 5: intraparenchymal
    Drag here to reorder.
  • Case 6
    Drag here to reorder.
  • Case 7
    Drag here to reorder.
  • Case 8
    Drag here to reorder.
  • Case 9: hemorrhagic
    Drag here to reorder.
  • Case 10
    Drag here to reorder.
  • Case 11
    Drag here to reorder.
  • Case 12
    Drag here to reorder.
  • Case 13: supratentorial extraventricular
    Drag here to reorder.
  • Case 14: supratentorial
    Drag here to reorder.
  • Case 15
    Drag here to reorder.
  • Case 16
    Drag here to reorder.
  • Case 17
    Drag here to reorder.
  • Case 18
    Drag here to reorder.
  • Case 19
    Drag here to reorder.
  • Case 20
    Drag here to reorder.
  • Updating… Please wait.

     Unable to process the form. Check for errors and try again.

     Thank you for updating your details.