與大師對話 黃國茂主任 - tmuh.org.twcystic ovarian tumor left ductal carcinoma in situ 4 ....
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與大師對話
黃國茂主任
臺北醫學大學附設醫院
影像醫學部
新光醫院放射診斷科
2017.06.20 臺北醫學大學附設醫院 影像醫學部 R2莊凱壹醫師/VS謝立群醫師
規則
依照臨床時序,請大師模擬一線放射科醫師;於未知診斷,或者有限度臨床線索之情形下,進行閱片及解讀。
鑑別診斷為主要,確定診斷為次要。
目的在於學習大師之影像判讀邏輯思考。
Focus on neuro images
大師評論本院影像品質建議及改進。
Protocols, techniques, etc.
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CASE 1
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Patient Profile
56 years old, female
Chief complaint
Rapidly progressive cognitive decline in recent 5 months
Poor memory and dysnomia, unsteady gait, visual blurring, and personality change
出門走不到目的地,不會搭公車,不會去廁所上廁所,存款密碼忘記,把乳液當牙膏,穿內衣出門
Past history
Cystic ovarian tumor
Left ductal carcinoma in situ
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Image
2017/04/05 MRI
2017/05/22 MRI
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Clinical Course
Underwent lumbar puncture
CSF: 14-3-3 protein positive
Diagnosis: Creutzfeldt-Jakob Disease
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Creutzfeldt-Jakob Disease
A spongiform encephalopathy that results in a rapidly progressive dementia and other non-specific neurological features and death usually within a year or less from onset
Clinical symptoms
Progressive dementia associated with myoclonic jerks and akinetic mutism
Variable constellation of pyramidal, extrapyramidal, and cerebellar signs
CSF protein biomarkers: 14-3-3 protein, total tau (t-tau) and neuron-specific enolase (NSE)
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Creutzfeldt-Jakob Disease
Four types
Sporadic (sCJD): accounts for 85-90% of
cases
Variant (vCJD): mad cow disease, Kuru
Familial (fCJD): 10% of cases (these
individuals carry a PRPc mutation)
Iatrogenic
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Creutzfeldt-Jakob Disease Best imaging clue: Progressive T2 hyperintensity
of basal ganglia (BG), thalamus, and cerebral cortex
Predominantly gray matter (GM): Caudate and putamen > globus pallidus (GP) Thalamus: Common in variant CJD (vCJD)
Cerebral cortex: Frontal, parietal, and temporal
2 signs seen in 90% of vCJD but can also occur in sporadic CJD (sCJD) "Pulvinar" sign: Symmetric T2 hyperintensity of
pulvinar of thalamus
"Hockey stick" sign: Symmetric pulvinar and dorsomedial thalamic nuclear hyperintensity
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Main Differential Diagnosis
Extrapotine osmotic demyelination
syndrome
Central pons T2 hyperintensity with sparing of
periphery
Location
Basal ganglia (BG)
Cerebral white matter (WM)
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CASE 2
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Patient Profile
42 years old, female
Past history
Sudden onset of reversible stroke on
2015/8/15
Chief complaint
Repeated numbness and transient numbness
and dysarthria for 2 weeks (2017.03)
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Image
2015.12.05 Angiography
2015.12.08 CT perfusion
2017.03.21 Angiography
2017.05.14 Brain MRI
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15 2015 2017
CB
F
CB
V
MT
T
Clinical Course
Clinical diagnosis: moyamoya disease ?
Underwent left fronto-temporal craniotomy
EC-IC bypass on 2017.05.15
2017/05/26 Brain MRI
Successful EC-IC bypass ?
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Moyamoya
Progressive narrowing of distal
ICA/proximal circle of Willis (COW)
vessels
With secondary collateralization → cloud-
like "puff of smoke" (moyamoya) at
angiography
Attenuated COW with multiple tiny basal
ganglia "flow voids" on MR
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Main Differential Diagnosis
PACNS
Circumferential, smooth, long-segment vessel
wall enhancement
"Beaded" arteries on DSA
Leptomeningeal arteries and veins are mostly
affected, but involves intracranial vessels of
any size
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Main Differential Diagnosis
RCVS
Reversible, multifocal cerebral artery
vasoconstrictions
Symptoms: severe headaches ± focal
neurological deficits
Involves large, medium-sized arteries
Diffuse, multifocal, segmental narrowing
Sometimes "string of beads" or "sausage
strings"
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20 Published February 22, 2013
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↓CBF ↑CBV
↑MTT ↑TTP
Before Surgery
CBF CBV
MTT TTP
After
Surgery
Zhang J, Wang J, Geng D, Li Y, Song D, Gu Y. Whole-Brain CT Perfusion and CT Angiography Assessment of
Moyamoya Disease before and after Surgical Revascularization: Preliminary Study with 256-Slice CT. Minnerup J,
ed. PLoS ONE. 2013;8(2):e57595. doi:10.1371/journal.pone.0057595.
Conclusion
The 256-slice whole-brain CTP and 3D-CTA have the potential for the non-invasive
assessment of the abnormalities of intracranial arteries, the graft patency and
cerebral perfusion changes in Moyamoya disease before and after surgery.
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Zhang J, Wang J, Geng D, Li Y, Song D, Gu Y. Whole-Brain CT Perfusion and CT Angiography Assessment of
Moyamoya Disease before and after Surgical Revascularization: Preliminary Study with 256-Slice CT. Minnerup J,
ed. PLoS ONE. 2013;8(2):e57595. doi:10.1371/journal.pone.0057595.
CASE 3
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Patient Profile
39 years old, male
Chief complaint
Persistent neck and low back pain, the worst
symptoms in the morning, the symptoms
worsened by sitting
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Image
2015/10/12 L-spine MRI
2017/05/17 T-spine MRI
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Clinical Course
Image diagnosis: myxopapillary
ependymomas
Underwent laminectomy at T5~T6, and
hemilaminectomy at T4 with removal of
tumor
Pathology: schwannoma
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Myxopapillary Ependymoma
Usually spans 2-4 vertebral segments
May fill entire lumbosacral thecal sac
Ovoid, lobular, sausage-shaped
CT/radiographs
± osseous canal expansion, thinned pedicles, vertebral
scalloping
May enlarge, extend through neural foramina
T1WI: Isointense→ hyperintense to cord
T2WI: Almost always hyperintense to cord
Hypointensity at tumor margin = hemosiderin
T1WI C+: Intense enhancement
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Spinal Schwannoma 70-75% intradural extramedullary
Most common intradural extramedullary mass
15% completely extradural
15% transforaminal, "dumbbell" masses
Size varies from small intradural mass to large intraspinal or paravertebral mass ("giant schwannoma") extending ≥ 2 vertebral segments
Bone remodeling due to large intraspinal or intraforaminal tumor common
Cystic change common
Calcifications, hemorrhage are rare
Uniform, heterogeneous, or peripheral enhancement patterns
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It is very important to differentiate SCHs and MPEs before surgery, because there
are reported cases of dissemination of MPEs through cerebrospinal fluid
throughout the neuraxis.
MPEs must be removed en block to prevent dissemination
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Moriwaki T, Iwatsuki K, Ohnishi Y, Ninomiya K, Yoshimine T.
Extramedullary Conus Ependymoma Involving a Lumbar Nerve Root
with Filum Terminale Attachment. Clinical Medicine Insights Case
Reports. 2015;8:101-104. doi:10.4137/CCRep.S24719.
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Schwannoma Myxopapillary Ependymoma
Moriwaki T, Iwatsuki K, Ohnishi Y, Ninomiya K, Yoshimine T.
Extramedullary Conus Ependymoma Involving a Lumbar Nerve Root
with Filum Terminale Attachment. Clinical Medicine Insights Case
Reports. 2015;8:101-104. doi:10.4137/CCRep.S24719.
CASE 4
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Patient Profile
34 years old, female
Chief complaint
Seizure attack twice since April 2016
(2016.11)
Past history
Nil
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Image
2016/09/20 Brain MRI
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Clinical Course
2016/11/04 underwent left temporal
craniotomy
Pathology
Epithelioid malignant peripheral nerve sheath
tumor (MPNST) and malignant melanoma
However, primary or secondary (metastatic)
origin of the tumor cannot be determined
based on histology
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Image
2016/12/20 Brain MRI (Post operation)
2017/03/20 Brain MRI
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2016.09.20
2016.12.20 2017.03.20
Clinical Course
2017/03/21 underwent left temporal
craniotomy
Pathologic consultation in the USA
Epithelioid GBM
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2016 WHO Classification
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Epithelioid Glioblastomas Predilection for children and younger adults
Present as superficial cerebral or diencephalic masses
Sometimes co-exist with pleomorphic xanthoastrocytomas
Pathology Large epithelioid cells with abundant eosinophilic cytoplasm,
vesicular chromatin, and prominent nucleoli, and variably present rhabdoid cells
Gene Often harbor a BRAF V600E mutation
IDH-wildtype epithelioid glioblastomas often lack other molecular features of conventional adult IDH wildtype glioblastomas, such as EGFR amplification and chromosome 10 losses
Frequent hemizygous deletions of ODZ3
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CT T2 Flair GRE
T1 +C DWI AD
C
Liebelt BD, Boghani Z, Takei H, Fung SH, Britz GW. Epithelioid
glioblastoma presenting as massive intracerebral hemorrhage: Case
report and review of the literature. Surgical Neurology International.
2015;6(Suppl 2):S97-S100. doi:10.4103/2152-7806.153643.
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Diffuse sheets of markedly atypical epithelioid cells
with pleomorphic nuclei are noted in a hemorrhagic
background. Prominent microvascular proliferation
is seen (arrows)
Glial fibrillary acidic protein (GFAP)
immunohistochemical stain reveals cytoplasmic
positivity in tumor cells
Liebelt BD, Boghani Z, Takei H, Fung SH, Britz GW. Epithelioid
glioblastoma presenting as massive intracerebral hemorrhage: Case
report and review of the literature. Surgical Neurology International.
2015;6(Suppl 2):S97-S100. doi:10.4103/2152-7806.153643.
Differential Diagnosis
Ganglioglioma
Pleomorphic xanthoastrocytoma
DIA/DIG, Desmoplastic Infantile
Astrocytoma and Ganglioglioma
Oligodendroglioma
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