Ependymoma Clinical Presentation

Updated: Mar 09, 2023
  • Author: Jeffrey N Bruce, MD; Chief Editor: Herbert H Engelhard, III, MD, PhD, FACS, FAANS  more...
  • Print


The clinical history associated with ependymomas varies depending upon the age of the patient and the location of the lesion. The duration of symptoms prior to diagnosis usually varies from 1-36 months; most patients have symptoms from 3-6 months. In one retrospective study of adult ependymoma patients, the most common presenting symptom of both intracranial and spinal ependymoma was pain. Changes in personality, mood, and concentration can be early indicators or may be the only abnormalities observed. Seizures are a presenting symptom in 20% of patients, and focal neurologic deficits may also be prominent.

Because they produce few specific symptoms, ependymomas cannot be diagnosed based on history alone. Clinical suspicion for a brain or spinal neoplasm should be confirmed with neuroimaging.  

Supratentorial Ependymoma

Supratentorial ependymomas may be associated with increased intracranial pressure that manifests as headache, nausea, vomiting, and cognitive impairment. Headaches can vary in intensity and quality and are frequently more severe in the early morning or upon first awakening. In children who present prior to closure of cranial sutures, enlarging head circumference secondary to obstructive hydrocephalus also may be part of the clinical history.

Posterior Fossa Ependymoma

Children or adults with masses in the fourth ventricle may have a history of progressive lethargy, headache, nausea, and vomiting secondary to increased intracranial pressure from obstructive hydrocephalus. As the tumor extends along the floor of the fourth ventricle, it may cause multiple cranial-nerve palsies (primarily VI-X), as well as cerebellar dysfunction.

Spinal Ependymoma

Spinal ependymomas are usually associated with a history of progressive neurologic deficit related to involvement of ascending or descending nerve tracts, exiting peripheral nerves, and pain that correlates with the level of the lesion.

Myxopapillary ependymoma, which primarily affects terminal areas of the spine, primarily presents with low back pain, but can present with bowel or bladder symptoms in around 30% of patients64. They can also be associated with cauda equina syndrome, which can present with lower limb dysfunction, saddle anesthesia, and/or bladder/bowel dysfunction.


Subependymomas are typically asymptomatic and are often identified incidentally on neuroimaging, but symptoms may resemble those above depending on location. Given their prevalence in the fourth ventricle, they may present with symptoms of increased intracranial pressure.  


Physical Examination

In patients with supratentorial ependymomas, neurologic symptoms and signs can be either general or focal, and they reflect the location of the tumor. Patients can present with cranial nerve symptoms. Hemiparesis, sensory loss, visual loss, aphasia, and cognitive impairment are common

Findings in patients with posterior fossa ependymomas including the following:

  • Hydrocephalus secondary to obstruction is a common complication.
  • General symptoms of increased intracranial pressure (headache, nausea, papilledema) are also common.
  • At diagnosis, common symptoms include papilledema, ataxia, and nystagmus.

Patients with spinal ependymomas are more likely to present with sensory changes than with weakness. Signs and symptoms will vary by level of the tumor, as follows:

  • Cervical levels: Weakness or motor dysfunction in upper limbs
  • Thoracic levels: Sensory symptoms, such as loss of cutaneous sensation below level of the lesion; motor symptoms may not be evident 
  • Lumbar levels: Radicular pain, weakness/motor dysfunction in the lower limbs

Myxopapillary ependymomas typically arise in the conus medullaris and cauda equina and therefore can lead to saddle anesthesia, bladder dysfunction, or impotence

Patients with subependymomas are most often asymptomatic. However, patients with intraventricular tumors can present with some symptoms of hydrocephalus.



Complications resulting from ependymoma vary by location and typically arise secondary to tumor mass effect. Nonspecific complications that can occur in any location of tumor include hemorrhage, infection, and worsening of neurologic deficit.

Children who undergo resection of a posterior fossa lesion are at risk for postoperative cerebellar mutism. Intracranial complications can include seizures.