Transcript Cerebellopontine Angle Cavernous Angioma: In the Setting

Cerebellopontine Angle Cavernous
Angioma:
In the Setting of Multiple Familial Cavernous
Malformations
Justin Mauch, M.D.; Carrie Carr, M.D.; Norbert Campeau, M.D.
Mayo Clinic, Rochester MN
Department of Radiology
ASNR 53rd Annual Meeting
April 25 - 30th, 2015
Chicago, Illinois, USA
Control #: 542
Poster #: EE-05
April 27th, 2015
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Disclosures
• None
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Purpose
• To present a case of cerebellopontine angle
(CPA) cavernous malformation occurring in the
setting of Multiple Familial Cavernous
Malformations.
• The distinguishing imaging and clinical features
of this rare lesion, which was initially diagnosed
as a vestibular Schwannoma, will be
highlighted.
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Case Report
• 58 year old male presented with multiple
insidiously progressive neurologic deficits
•
•
•
•
Neurogenic bladder
Poor right leg motor and balance function
Dizziness
Right-sided hearing loss
• Initial work-up:
• Neurologic evaluation
• MRI brain and MRI cervical/thoracic spine
• Audiometry
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Imaging Findings: Initial MRI
A
B
C
D
C
E
F
Selected axial T2WI of the brain (A-D) and T1 pre and post gadolinium (E and
F respectively) demonstrate multiple intracranial and spinal cord (not shown)
lesions with findings characteristic of cavernous malformations leading to the
diagnosis of Multiple Familial Cavernous Malformations.
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Imaging Findings: CT
B
C
A
A head CT was never performed, however a CT myelogram was obtained
for evaluation of cervical myelopathy related to cervical spinal cord
cavernous malformations. The CT (A) demonstrated a noncalcified mass in
the right CPA (arrow). A spinal cord mass at C5-6 is shown on the sagittal
CT Myelogram (B) with MR imaging characteristics of a cavernous
malformations as seen on sagittal T2WI (C).
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Case Report
• A right CPA mass was identified
• It demonstrated homogenous enhancement and
partial extension into right IAC
• Findings were suggestive of an incidental
vestibular schwannoma.
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Imaging Findings: Initial MRI
A
The MRI demonstrates a
right CPA mass (arrows)
with areas of T2 (A)
hyperintensity and
homogenous enhancement
as seen on pre-contrast T1
(B) and post-contrast T1
(C).
B
C
*All sections at same level
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Case Report
• Patient had vestibular and audiometric testing:
• Decreased right sided function
• Patient elected to forego surgical intervention or
gamma knife therapy due to the stability of his
deficit.
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Case Report
• Follow-up imaging was performed over the next
decade
• Most recently due to hemorrhagic
complication of multiple intracranial and
spinal cord cavernous malformations with
eventual resection
• Follow-up MR imaging included high
resolution FIESTA and susceptibility
weighted sequences
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Imaging Findings: 10 year Follow-Up MRI imaging
2004
2014
Axial T1 post gadolinium images from the MRI follow-up over the next decade
demonstrated subtle changes in the CPA lesion size and appearance, with
most apparent change noted on the 2014 MRI, where the lesion enlarged and
become more heterogenous in appearance.
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Imaging Findings: CPA lesion changes appearance
The 2014 follow-up MR imaging
included pre-contrast T1 (A), high
resolution FIESTA (B), post-contrast
T1 (C), and susceptibility weighted
sequences (D).
A
B
C
D
New T1 (A) hyperintense signal
changes within the right CPA lesion
with associated low signal on SWI
(D), compatible with subacute blood
products.
FIESTA imaging (B) also better
demonstrates that the mass does
not extend into the IAC, not the
typical configuration for a
Schwannoma
The CPA lesion now has findings
consistent with a cavernous
malformation rather than
Schwannoma.
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Discussion
• Cavernous malformations of the CPA are rare entities which
make up an estimated 0.0023% of all CPA lesions*
• To date, there are only two other reported cases of a CPA
cavernous malformation occurring in the setting of Multiple
Familial Cavernous Malformations (Aquilina, Cotton)
• Surgical options for CPA Schwannoma or Meningioma
include primary microsurgery, stereotactic radiosurgery, or
observation*
• It is important to differentiate a CPA cavernous malformation
from these more common CPA lesions due to surgical
planning. CPA cavernous malformation are highly vascular
and can present with copious intraoperative hemorrhage*
• Microsurgical gross total resection has been proposed as the
treatment of choice as subtotal resection can result in
*Kohan, Carlson, Park, Barrera, Deshmukh, Safronova
recurrence*
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Discussion
Distinguishing Imaging Features…
• Specific imaging features of CPA cavernous malformation
can help to distinguish it from other more common CPA
lesions, such as Schwannomas and Meningiomas
• Schwannomas and Meningiomas both enhance strongly
after gadolinium injection while cavernous malformations
will have mild heterogeneous enhancement*
• Hyperintense T1 signal and susceptibility artifact suggest
blood product which is typically not seen with Meningiomas
or Schwannomas*
• Schwannomas are often T2 hyperintense while cavernous
malformations will have areas of low T2 signal
• Schwannomas will typically have a component within the
IAC
• CT may show areas of calcification within CPA cavernous
malformations where as this is less common in
Schwannomas
*Bonneville
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Imaging Differential Diagnosis
Below: Right CPA Schwannoma. Axial T1 (A), Axial FIESTA (B), and Axial T1+ Gd (C).
A
B
C
Below: Right CPA Meningioma. Axial T1 (A), Axial FIESTA (B), and Axial T1+ Gd (C).
A
B
C
Below: Right CPA cavernous malformation. Axial T1 (A), Axial FIESTA (B), and Axial T1+ Gd (C).
A
B
C
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Imaging Findings: Use of Susceptibility Weighted Imaging
SWI (A) and Axial T2 FSE
and (B) images obtained
at the same level
illustrates the utility of
detecting hemosiderin with
the SWI technique.
SWI images (C) and Axial
T2 FSE (D) obtained at the
level of the CPA lesion. The
SWI confirms presence of
hemosiderin within the right
CPA cavernous
malformation (arrows).
A
B
C
D
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Summary
• Cavernous malformations of the CPA are rare
entities, but consideration of this diagnosis is
important when specific imaging and clinical
features are present.
• Hyperintense T1 signal within the lesion and
blooming on susceptibility weighted imaging
indicative of old blood products are suggestive of
this lesion, and essentially diagnostic in individuals
with Multiple Familial Cavernous Malformations.
• Accurate preoperative diagnosis is important for
planning appropriate surgical removal of this highly
vascular mass.
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References
•
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•
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Link MJ. Long-term quality of life in patients with vestibular schwannoma: an international multicenter cross-sectional study comparing microsurgery,
stereotactic radiosurgery, observation, and nontumor controls. J Neurosurg. 2015 Jan 2:1-10.
•
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