Transcript 96_exportSess - Stanley Radiology

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RARE CASE OF MYXOPAPILLARY
EPENDYMOMA FROM MEDULLA
Learning objectives
Myxopapillary ependymoma (WHO Grade I ) is a rare variant of
ependymoma; this tumor was originally defined by Kernohan and it
occurs almost exclusively in the conus medullaris or filum
terminale.
Ependymomas have a wide morphologic spectrum and MPE
constitutes a distinct clinico-pathological variant.
MPE that occurs primarily in the cerebral hemisphere or brainstem
is extremely rare with only seven cases having been reported in the
literature, and all of them have occurred in young patients
background
 A 45 years old male patient presented with several episodes of
syncopal attacks with right upper limb numbness
 Plain CT revealed a well defined ovoid heterogeneous mildly
hypodense lesion measuring ~ 3 cm filling the cisterna magna
with mass effect on adjacent cerebellar hemispheres and medulla
 Plain and contrast enhanced MRI revealed
 A well circumscribed heterogeneous lesion in the cisterna magna
and inferior aspect of fourth ventricle causing compression of
medulla and splaying of inferior cerebellar peduncle with minimal
expansion of cervico medullary junction
CT PLAIN
MRI T1 WI
T2WI- AXIAL
MRI - CONTRAST SAGGITAL /CORONAL VIEW
 Patient had an episode of cardiac arrest and was
revived and taken up for emergency decompression
surgery.
 Midline suboccipital craniectomy was performed
 The lesion was seen arising from medulla and upper
part of cervical cord with soft fleshy wall and a cystic
area containing mucinous material
 Partial resection of the lesion and cyst was achieved.
HISTO PATHOLOGY
FINDINGS
 Pathological Findings: Microscopically, the
peculiar histologic features were papillae or
reticular structures formed by cuboidal or flat
cells with regular nuclei, and there was an
abundant stroma showing conspicuous
mucinous change.
 The latter was strongly positive for alcian-blue
and periodic acid-Schiff stain.
 Multifocal hemorrhagic necrosis with fibrosis
were evident.
HPE ..CONT
The tumor cells showed strong diffuse positivity
for GFAP in the majority of the neoplastic cells.
Focal positive reactions were also identified for S100 protein and vimentin. However, the tumor cells
were negative for cytokeratin. The Ki-67 labeling
index was very low (<1%) and the tumor cells were
negative for p53 protein. histopathological and
clinicoradiologic studies confirmed that the
 primary MPE arising in the medulla.
GFAP
S100
DISCUSSION
 Myxopapillary ependymoma is a sub-classification of
ependymoma that is thought to be nearly exclusive to
the conus medullaris or filum terminale. primary
intracerebral or brainstem myxopapillary ependymomas
are very rare only seven cases of primary intracranial
MPE have been reported
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other regions where MPE presented
 six of these tumors were locatedsupratentorial tumor
group, 2 occurred in the intraventricular, 2 were
intraparenchymal, 1 was transependymal, and 1 was
extra-axial. the infratentorial tumor was restricted to the
4thventricle.
 We present here a case of 45 years old male with
Mxyo papillary ependymoma of the medulla, and
we also review the relevant literature. To the best
of our knowledge, this is the second documented
case of the literature
 Conclusion: Myxopapillary ependymoma thought
to be nearly exclusive to the conus medullaris or
filum terminale. but one should think about MPE
intraparenchymal and brainstem and imaging/
histopathology helps us to treat the patient in
advance
 Patient now on radiotherapy and check MRI done
for drop metastasis
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References
 Primary myxopapillary ependymoma of the medulla:Neurosurgery 2010
Jun;66(6):E1208-9; discussion E1209Michael L DiLuna, Gillian H Levy, Shreya
Sood, Charles C Duncan
 Primary intracranial myxopapillary ependymomas: report of two cases and review of
the literature. Acta Radiol 2004 May;45(3):344-7Y C Tseng, H L Hsu, S M Jung, C J
Chen
 Signet-ring cell ependymoma with intratumoral hemorrhage in the medulla
oblongata.J Clin Neurosci 2005 Aug;12(6):711-4Junichi Mizuno, Hiroshi Nakagawa,
Tatsushi Inoue, Shiro Kondo, Kazuo Hara, Yoshio Hashizume
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Maruyama R, Koga K, Nakahara T, Kishida K, Nabeshima K: Cerebral
myxopapillary Hum Pathol 23:960-962, 1992
 Matyja E, Naganska E, Zabek M, Koziara H: Myxopapillary ependymoma of the
lateral ventricle 33:1017-1025, 1983