Latvian and Lithuanian students Neuro-radiological meeting on 18.03.2017. Presentation made by Kavish Khatib from Riga Stradiņš university.

1. 1
Ependymomas
Kavish Khatib,
11th Semester,
18.03.2017, Rīga

2. 2
• A tumour that arises from the ependymal
cells in the nervous system.
• The Ependyma is the epithelial lining of
the brain ventricles and the central canal
of the spinal cord.
• Involved in the production of the
CSF and has shown as a reservoir
for neuro-regeneration.
• The group of tumours referred to as
Gliomas
• Two predominant types: Spinal and
Brain Ependymoma
Definition
Source: https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq (accessed 07/03/2017)

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Biology
• Described in 1924 by Bailey
• Classified into 4 groups by the WHO classification of tumours:
1. Myxopapillary ependyoma (Grade 1)
2. Subependymomas (Grade 1)
3. Ependymoma (Grade 2)
4. Anaplastic ependymoma (Grade 3)
• Ependymomas tend to grow relatively slowly and displace, rather than
invade adjacent brain or spinal cord tissue.
• Ependymomas rarely metastasize to sites outside of the central nervous
system. When ependymomas recur after treatment, they tend to grow back
locally (i.e. at or near the site of the original tumour), rather than spreading to
other sites.
Source: http://www.childhoodbraintumor.org/medical-information/brain-tumor-types-and-imaging/item/84-ependymomas

4. • Grade I – Myxopapillary
Ependymoma and Subependymoma
• Grade II – Ependymoma (Classic)
• Grade III – Anaplastic Ependymoma
(which is the more cancerous)
Source: http://www.cern-foundation.org/education/diagnosis/pathology

5. 5
• Intracranial ependymomas represent 6-9% of primary
CNS neoplasms and account for 30% of primary CNS
neoplasms in children younger than 3 years.
• The incidence of ependymoma is approximately equal in
males and females.
• Ependymomas generally present in young children with a
mean age of diagnosis of 4 years, yet 25-40% of patients
are younger than 2 years.
• Spinal ependymomas are most common in patients aged
15-40 years, most of which are of a myxopapillary subtype.
Epidemiology

6. 6
• Symptoms are dependant on the location of the
tumour, typically found in three major locations:
• The posterior fossa (below the tentorium, containing the
cerebellum and the brainstem),
• The supratentorium (above the tentorium containing the
cerebral hemispheres),
• The spinal cord In adults, >75% of ependymomas arise
within the spinal canal, but in children, about 90% arise
within the brain in the posterior fossa, in or around the
fourth ventricle and only 10% arise within the spinal cord.
Presentation/Symptoms
Source: https://radiopaedia.org/articles/ependymoma

7. 7
• (60%) are located in the posterior fossa (infratentorial), usually arising from the floor
of the fourth ventricle. This is especially true in children.
• The remainder (40%) are located supratentorially and up to half of these are
intraparenchymal.
• Posterior fossa ependymomas has the propensity to grow out of the -
Foramina of Luschka into the
Cerebellopontine angle into the
Foramen of Magendi hence the term plastic ependymoma.
From here it can spread into the spine.
• Ependymomas are typically heterogeneous masses with areas of necrosis, calcification,
cystic change and haemorrhage frequently seen.
General radiographic features

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Imaging modalities
• X-ray
• CT
• MRI
• Nuclear medicine

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X-ray
• Primarily of historical interest since the
onset of CT in 1974
• Was useful for detecting increased intra-
cranial pressure and intracranial
calcification.

10. 10
• Most intracranial neoplasms are visible on CT
• Tumours may be hypodense, isodense or hypotense
on a non-contrast CT depending on tumour histology
and location.
• Small tumours or isodense tumours may be missed
on non-contrast CT but highlight after contrast
administration
• CT is preferred for visualising tumour calcification or
haemorrhage.
CT

11. Ependymoma

12. 12
MRI
• MRI is the diagnostic modality of choice in the workup and
follow-up observation of intracranial neoplasms, including
ependymoma.
• MRI exploits the use of increased water content of many
neoplasms. This water concentration shows up as increased
signal on T2 weighted images and decreased signal on T1
images
• MRI is used to monitor ongoing treatment and to search for
recurrence.
• Final diagnosis is achieved through biopsy with histopathologic
analysis.

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MRI findings
• On T1-weighted images, ependymomas appear to be heterogeneous and
hypointense or isointense to gray and white matter.
• On T2-weighted they may be isointense or hyperintense to gray and white matter.
• Calcifications appear as hyperintensities on T1-weighted images, appear as
hypointense regions on T2-weighted images, and demonstrate “blooming” on T2
gradient-recalled echo images.
• As many as 50% of ependymomas demonstrate signal heterogeneity, which may
indicate calcification, necrosis, methemoglobin, hemosiderin, or tumor vascularity.
• Cystic changes result in high signal intensity on T2-weighted MRIs variably
depending on cyst contents.

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• Signal heterogeneity is a feature useful in distinguishing
ependymoma from the more homogeneous medulloblastoma.
• Calcification and hemorrhagic foci are more typical of
ependymoma than medulloblastoma.
• Additionally, ependymomas are more apt to extend through the
foramina of Luschka and Magendie, hence the term “plastic
ependymoma.”
• Similarly, choroid plexus papilloma is more homogeneous than
ependymoma and lacks the typical irregular margins of
ependymoma.
MRI findings

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• The lobulated mass in this
proton density–weighted
sagittal image arises from the
fourth ventricle and extends
distally through the foramen
of Magendie.
• There is minimal lateral
ventricular hydrocephalus.
Pathologic analysis
demonstrated cellular
ependymoma.

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MRI Gadolinium
• Contrast (often gadolinium I/V) helps visualise small tumours that don't
cause much oedema.
• Enhancement with IV gadolinium is useful in differentiating tumour from
adjacent vasogenic oedema and normal brain parenchyma.
• Without intravenous contrast enhancement, T2-weighted images are more
reliable in differentiating tumour margins than are T1-weighted images.

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• Anaplastic ependymoma of the lateral ventricle in an 8-week-old girl with
lateral ventricular hydrocephalus.
• Gadolinium-enhanced coronal T1-weighted image demonstrates a large
anaplastic necrotic ependymoma of the left lateral ventricular roof with
severe mass effect and subfalcine herniation.

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Supratentorial Ependymoma
• A. noncontrast CT, B. T2-weighted, C.
FLAIR, and D-F postgadolinium T1-
weighted MRI.
• Unlike posterior fossa ependymomas,
most supratentorial ependymomas
(70%) are extraventricular in origin.
• Supratentorial ependymomas usually
demonstrate more heterogeneous T1
and T2 signal than their infratentorial
counterparts, due to a greater tendency
toward cyst formation, calcifications, and
hemorrhage.
Source: http://clinicalgate.com/posterior-fossa-tumors-in-the-pediatric-population-multidisciplinary-management/

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Supratentorial Ependymoma
• (A) MRI demonstrates canonical features of a right
sylvian fissure ependymoma on precontrast axial T1-
weighted.
• (B) T2-weighted,
• (C) Fluid-attenuated inversion recovery
• (D) Images and on postcontrast T1-weighted
• (E) Sagittal T1-weighted.
• (F) Right pterional craniotomy was performed, and a
gross total resection was achieved after a wide
opening of the sylvian fissure.
• G - I, Histologic analysis shows areas of perivascular
pseudo-rosettes (G) as well as representative areas of
clear cell (H) and tanycytic (I) features of the grade II
tumor.
Source: Youmans and Winn Neurological Surgery, 7th edition

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Posterior fossa Ependymoma
• A. Pre-contrast axial CT shows an
isodense midline cerebellar mass
with calcifications.
• B. On T2-weighted sagittal MRI,
there is a fourth ventricular mass
showing compression of the
surrounding cerebellum and brain
stem.
• C. Post-contrast axial MRI shows a
heterogeneously enhancing mass
extending through left foramen of
Luschka into the cerebello-pontine
angle.
• D. Post-contrast sagittal MRI
showing an ependymoma
extending through the foramen
magnum.
Source: http://clinicalgate.com/posterior-fossa-tumors-in-the-pediatric-population-multidisciplinary-management/

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Posterior Ependymoma
• A. and B. Postcontrast T1-weighted axial
• Demonstrates a midline posterior fossa ependymoma and filling the fourth
ventricle and foramen of Luschka and exiting the foramen of Magendie.
• C. Intraoperative photograph depicts the posterior fossa ependymoma (grade II)
with a classical grayish, smooth appearance.
• D. Postoperative postcontrast T1-weighted magnetic resonance image
demonstrating a gross total resection achieved via a midline suboccipital
craniotomy.
Source: Youmans and Winn Neurological Surgery, 7th edition

22. 22
Spinal Ependymoma
• A 21-year-old female
patient with lumbar pain
for over a month;
• A. preoperative T2-
weighted magnetic
resonance imaging (MRI)
and gadolinium-
enhanced T1-weighted
MRI
• B. Revealing a tumor in
the conus medullaris;
• C. Postoperative MRI
revealing complete tumor
excision
Source: http://www.cancerjournal.net/viewimage.asp?img=JCanResTher_2013_9_1_60_110371_f3.jpg

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Nuclear Medicine: SPECT
• SPECT (Single positron emission computed tomography)
• Gamma rays emitted during radionuclide decay that are detected by a gamma camera that
rotates about the patients head.
• The radionuclide must cross the blood-brain barrier.
• The radionuclides used are:
• 201 TI chloride
• 99m Tc MIBI
• 123 I alpha-methyl tyrosine
• 111 In octreotide
• Can be used in distinguishing between benign lesions, low-grade gliomas and high grade.

24. Radionuclide = 99 m TC
SPECT of normal brain 201 T1 SPECT
Diagnosed by SPECt as a
high grade glioma
and confirmed post-resection

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Nuclear Medicine: PET
• Similar to SPECT but the radioisotopes used decay to produce positrons
• These positrons quickly combine with adjacent electrons to produce two
gamma rays that travel in opposite directions. Detection of these gamma rays
allows the calculation of their exact point of origin,
• Can evaluate different brain process depending on the radioisotope selected.
• Radionuclides useful for PET analysis include:
• Flourodeoxyglucose
• C methionine
• F alpha-methyl tyrosine

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• CT lateral ventricle wall suggested
possible ependymoma (red arrow).
• FDG-PET one week later, there is a
markedly increased uptake in the
tumour extending outside the CT-
defined lesion.
• Additional uptake in the fourth
ventricle is indicative of spread into
the cerebrospinal fluid.
• This accounts for the frequent liquoral
spread of neoplastic cells and the multi-
focality of lesions at later phases of the
disease.

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Treatment
• Although total resection is optimal, it is only possible in approximately 30-
40% of cases because vital structures are frequently involved by the
tumour.
• Ependymomas arising above the tentorium, in the floor or roof of the fourth
ventricle, or in the spinal canal are most amenable to complete resection.
• Adjuvant treatment of histologically confirmed intracranial ependymoma
remains an actively debated topic.
• Currently, a reduced role exists for adjuvant therapy of spinal ependymoma
after complete surgical resection. For patients who have postoperative
residual tumor or early recurrence, radiation is considered on the basis of
the individual patient's medical condition and neurological status.
Source: http://emedicine.medscape.com/article/277621-treatment

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Prognosis
• 10-year overall survival rate for ependymoma can vary from 45-55%.
• The current 5-year survival rate for patients with intracranial
ependymomas is approximately 50%,
• When rates from children and adults are combined. Stratification based on
age reveals 5-year survival rates of 76% in adults and 14% in children.
Source: Youmans and Winn Neurological Surgery 7th edition

29. CASE REPORT

30. Patient data
• Female, 39 years old
• Complains about back pain for a half of year
• There was MRI performed

31. 23.03.2012
• Intradural and intramedullar lesion
approxiamtely 6 x 1 x 1 cm in size
• Inhomogenous, localized more
centrally of myelon
• In T2 the structure is mostly
hyperintenss and in T1 iso-
hypointenss.
• Lesion is not enhancing
convinsingly
Intradural and intramedullar tumor
in level of Th9-Th11, most probably
ependymoma
Sag T2 fr FSE

32. Sag T2 fr FSE Sag T2 FLAIR

33. 22.04.2013.
• After spinal tumor evacuation and
following radiation therapy
• There is post-resection residual
structure in distal part of resection
area. The largest size is 2 cm,
also slightly inhomogenous and is
not enhancing convinciengly
Sag T2 fr FSE

34. Sag T1 FLAIR
Ax T1 FSE

35. 35
Thank you!!!