Subependymoma

Subependymomas are uncommon, benign (WHO grade I) tumours which are slow growing and non-invasive. They tend to occur in middle-aged and older individuals and usually identified as an incidental finding. 

These tumours were previously also known as subependymal astrocytomas, not to be confused with subependymal giant cell astrocytomas seen with tuberous sclerosis. They are also considered by some to be variants of ependymomas, with which they may co-exist (see below).

Subependymomas tend to present in middle-aged to older individuals (typically 5th to 6th decades 3). There is a slight male predilection (M:F 2.3:1) 6,8. Rarely there is a genetic predisposition for these tumours 8

Typically patients are asymptomatic and small lesions are discovered incidentally. In some cases, especially when the tumours are larger, presentation is with symptoms of raised intracranial pressure due to obstructive hydrocephalus.

Macroscopic appearance

Subependymomas are sharply demarcated nodules, usually no more than 2cm in diameter, arising from the ependyma by a narrow pedicle 6,8

Microscopic appearance

The histopathology of subependymomas is distinct comprising of a tumour arising from the subependymal glial layer with low cellularity and no high-grade features (no mitoses, Ki-67/MIBI index > 1.5%, no necrosis). These lesions are hypovascular. Loose perivascular pseudorosettes are occasionally seen. They are WHO grade I lesions (see WHO classification of CNS tumours) 8.

Occasionally foci of cellular ependymoma are seen, although the effect on clinical behaviour is unclear 4-5. They are graded according to the ependymoma component and not surprisingly behave similarly to the higher grade (ependymoma) component 6,8.

Immunophenotype

Cells express GFAP 4-6,8. Unlike ependymomas, EMA is usually negative 8.  

Subependymomas are most commonly seen in the fourth ventricle, but can arise anywhere where there is ependyma. They are therefore in the differential for other intraventricular masses. Distribution in the ventricular system is as follows 6,8:

  • fourth ventricle: 50-60%
  • lateral ventricles (usually frontal horns): 30-40%
  • third ventricle: rare
  • central canal of the spinal cord: rare

They are usually small, typically less than 2cm in size 6.

CT

Isodense to somewhat hypodense intraventricular mass compared to adjacent brain, which does not usually enhance. If large, it may have cystic or even calcific (up to half of the cases 3) components. Surrounding vasogenic oedema is usually absent.

MRI
  • T1
    • iso-hypointense to white matter
    • generally homogeneous but may be heterogeneous in larger lesions
  • T2
    • hyperintense to adjacent white and grey matter
    • again, heterogeneity may be seen in larger lesions, with susceptibility related signal drop out due to calcifications occasionally seen
    • no adjacent parenchymal oedema (as no brain invasion is present) 6
  • T1 C+ (Gd)
    • usually no enhancement, although at times may demonstrate mild enhancement
DSA-angiography

As expected from the histology, subependymomas show no or little vascularity 6.

If appearances are characteristic and the patient is asymptomatic, then follow up is a viable option.

Resection should be considered if the patient is symptomatic (hydrocephalus or mass effect), the mass has an atypical appearance or demonstrates growth. Local resection is curative and even debulking has an excellent outcome 8

General imaging differential considerations include other intraventricular neoplasms and lesions. A few specific lesions to consider include:

  • ependymoma
    • usually in children, or younger adults
  • choroid plexus papilloma
    • vividly enhancing
    • usually in children, or younger adults
    • in adults more common in the 4th ventricle
  • central neurocytoma
    • particularly if close to the septum pellucidum
    • overall less common
    • typically seen in younger patients (20-40 years of age)
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Article Information

rID: 4743
Synonyms or Alternate Spellings:
  • Subependymomas

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    Subependymoma T1 C+
    Case 4: large and enhancing
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