Guidelines for the

Management of
Adult Ependymoma

NHS England and NHS Improvement

 West Midlands Cancer Alliance
Coversheet for Cancer Alliance Expert Advisory Group Agreed
Documentation

This sheet is to accompany all documentation agreed by the West Midlands

Cancer Alliance Expert Advisory Groups. This will assist the Cancer Alliance to
endorse the documentation and request implementation.

EAG name Brain and CNS Expert Advisory Group

Document Guidelines for the Management of Adult Ependymoma

Title

Published January 2020

date
Document This guidance has been produced to support the management
Purpose of adults with ependymoma

Authors H Benghiat
P Sanghera

Review Date January 2023

(must be
within three
years)
Approval EAG Chair Cancer Alliance Clinical Director
Signatures:

Rob Gornall
Shabin Joshi 17 January
17 January 2020 2020

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 ADULT EPENDYMOMA GUIDELINES

Ependymal tumours are rare CNS tumours and although may develop at any
age, are more common in the paediatric and Teenage Young Adult (TYA)
population. Consequently, data informing adult practice is from small
retrospective series only. Please refer to recently published International
consensus guidelines by EANO. (Ruda R, Reifenberger G, Didier et al. EANO
guidelines for the diagnosis and treatment of ependymal tumours. Neuro-Onc;
2018(4), 445-456).
TYA cases can be discussed at the national MDT (EMAG) via
teleconference co-ordinated from Nottingham.
Pathological Classification
Ependymal tumours are of neuroectodermal origin and are broadly
classified histologically into 3 grades (I, II and III). Distinction between grade
II and III tumours can be difficult and the prognostic implications of this
remain debated. Most recent WHO classification (2016) comprises 5 distinct
entities:
1. Myxopapillary ependymoma WHO grade I (typically located in
conus medullaris, cauda equina and filum terminale).

2. Subependymoma WHO grade I (typically located in 4th ventricle).

3. Ependymoma WHO grade II (classical / conventional):
a. Papillary ependymoma
b. Clear cell ependymoma
c. Tanycytic ependymoma
4. Ependymoma: RELA fusion- positive: supratentorial tumour with
relatively worse prognosis than other ependymomas in same anatomical
compartment.
5. Anaplastic ependymoma (WHO grade III).
It is known that the prognosis of ependymal tumours is also based on patient
age and tumour location, as well as grade and histological subgroup. DNA
methylation array has identified nine molecular subgroups that more
accurately reflect the variation across these tumours, and although not
currently performed routinely, can be requested via pathology and University
College London Hospital (UCLH) if required for individual cases.

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Staging
Because of the risk of CSF dissemination, Craniospinal MRI with contrast
and lumbar puncture for CSF cytology should be performed post-operatively
(2-3 weeks post-surgery) for all newly diagnosed ependymal tumours. MRI
should be performed pre-LP to avoid artefact.

Management options

Gross total resection (GTR) of tumour should be attempted wherever

possible. GTR is defined as no residual disease on contrast enhanced MRI
3 months post-operatively. Extent of surgery has consistently been one of
the strongest predictors of outcome in published series. Resect ability of
posterior fossa tumours may be limited by encasement of vasculature and
cranial nerves.

Extent of resection should be confirmed on post-operative imaging and

repeat surgery considered if residual disease remains.

Spinal Ependymoma

Gross total resection should be attempted where feasible with

acceptable neurological risks.

• Myxopapillary Ependymoma (WHO GI)

These tumours irregular shape and anatomical location can make

GTR challenging. In cases of incomplete resection, local post-
operative radiotherapy is recommended to doses of ≥ 50Gy,
respecting spinal cord tolerance.

• WHO GII and III ependymoma of spinal cord

Post-operative radiotherapy should be used in cases where GTR has not

been achieved. Doses should be 50-54Gy, respecting tolerance of critical
structures. There is no evidence to support the routine use of adjuvant
radiotherapy where a GTR has been achieved, however, due to difficulties
resecting tumours en bloc, their involvement with vascular and neural
structures making resection technically challenging, and difficulties
interpreting post-operative imaging; adjuvant radiotherapy can be
considered after GTR and can be discussed with patients.

Adjuvant radiotherapy is offered routinely for GIII anaplastic

ependymoma regardless of extent of resection.

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Intra-Cranial Ependymoma

Gross total resection should be attempted where feasible with

acceptable neurological risks.

• Subependymoma

These are rare and indolent tumours (WHO Grade 1) and often diagnosed
as an incidental finding.

Options are observation (if diagnosis can be made on imaging) and

resection. Surgical resection is recommended if patient is symptomatic or
there is uncertainty regarding diagnosis. - Ideally gross total resection
should be carried out where possible. MRI surveillance should be preferred
for incidental intraventricular subependymomas. No adjuvant therapy is
required after gross total resection.

Even following STR regrowth may be slow. Role of radiotherapy remains

uncertain. SRS is commissioned for ependymoma and may provide an
alternative technique for inoperable low volume progressive disease.

• Ependymoma G-II

All patients with subtotal resection should be offered post op radiotherapy

(54- 59.4Gy). Whether patients with gross total resection of intracranial GII
ependymoma should receive adjuvant radiotherapy remains uncertain in
adults and pros and cons should be discussed with individual patients.

• WHO G III Anaplastic Ependymomas:

All G III ependymomas should be offered adjuvant radiotherapy

irrespective of the extent of resection (54-59.5Gy).

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Figure One: Management of Intracranial Ependymoma

Radiotherapy guidelines:

• Radiotherapy dose for intracranial lesions should be in the range of 54 to

59.4*Gy in 30-33 fractions.
• Spinal ependymomas are treated to a dose of 50.4 – 54 Gy in 28-30
Fractions (aim at least 50Gy).
• Craniospinal RT is recommended in case of CSF or spinal dissemination.
Dose for CSI should be 36 Gy in 20 fractions with boost to focal lesions of
up to 54 Gy in 30 fractions (refer to UHB).

*Aim for 59.4Gy in 33 fractions based on paediatric data. Dose reduction to

be considered for inoperable disease into critical OARs.

Consider proton referral for TYA population.

Follow Up:

All patients should be aware of access to service via a key worker. Follow
up may be undertaken according to local set up (which may include
telephone follow up). Standard structural MRI (T2W, FLAIR, DWI, T1 pre
+ post gad; volume scan) should be offered as part of regular clinical
review.

Referral to Endocrinology and ophthalmology services for follow up should

be considered on an individual patient basis.

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Management of recurrent ependymoma:

• There are no standard salvage options for recurrent ependymoma.

• Wherever possible, GTR should be attempted.
• Re irradiation should be considered, taking into account interval
from previous course of radiotherapy and previous dose.
• Chemotherapy may be considered in patients with preserved performance
status when there are no further surgical / radiotherapy options, but
response rates are low. Platinum based chemotherapy and etoposide can
be considered and temozolomide has also been used with low response
rates.

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