neurocytoma accompanied by intraventricular hemorrhage: case … · 2014-05-12 · neurocytoma...
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Neurocytoma Accompanied by Intraventricular Hemorrhage: Case Report and Literature Review Wendy R. K. Smoker,1 Jeannette J. Townsend,2 and Mark V. Reichman3
765
Neurocytomas are a rare subset of primary cerebral neuroblastomas. They occur within the ventricles of young adults, may calcify , and demonstrate a better prognosis than do their parenchymal counterparts . Of the 21 patients reported in the
literature who appear to meet most of the criteria for th is diagnosis, 18 had symptoms referable to increased intracranial pressure [1-1 0]. The lesions were discovered incidentally in the other three patients [5, 7]. The patient who is the
Fig. 1.-A, Unenhanced CT scan at time of admission reveals intraventricular hemorrhage and acute hydrocephalus.
B, Left internal carotid artery angiogram, anteroposterior view, shows slight enlargement of a lenticulostriate artery (small arrowheads), early opacification of thalamostriate vein (arrows), and avascular mass effect bowing other lenticulostriate vessels laterally (large arrowheads).
C and D, Sagittal (C) and axial (D) T1-weighted MR images (600/ 20/ 1) obtained 5 days after presentation. High signal intensity within left lateral ventricle is compatible with subacute hemorrhage. Flow void from early draining left thalamostriate vein is quite prominent (arrowheads).
A
c
B
D
Received August 16, 1990; returned for revision September 27, 1990; revision received January 7, 1991 ; accepted January 9, 1991 . Presented at the annual meeting of the American Society of Neuroradiology, Los Angeles, CA. March 1990. 'Department of Radiology, University of Utah, Salt Lake City, UT 84132. Present address: Department of Radiology, Medical College of Virginia , MCV Station ,
Box 615, Richmond, VA 23298. Address reprint requests toW. R. K. Smoker. 2 Department of Pathology, University of Utah, Salt Lake City, UT 84132. 3 Department of Neurosurgery, University of Utah , Salt Lake City, UT 84132 .
AJNR 12:765-770, July/August 1991 0195-6108/91/1204-0765 © American Society of Neuroradiology
766 SMOKER ET AL. AJNR:12, July/August 1991
A B
D
F G
subject of this report presented with intraventricular hemorrhage.
Case Report
A previously healthy 26-year-old man was seen at an outside emergency department following a single grand mal seizure. His
c
Fig. 2.-A, Unenhanced CT scan 4 months after initial ictus reveals a heterogeneous hyperdense lesion in left lateral ventricle (M) and left lateral ventricular obstruction.
8 and C, Sagittal (8) and axial (C) T1-weighted MR images obtained 5 months after admission reveal a heterogeneous mass (M), isointense relative to cortex. Flow void from left thalamostriate vein is less prominent than on previous MR study (arrowhead) .
D and E, Proton density- (D) and T2- (E) weighted axial images (2800/30-60/0.75) reveal lesion to be generally hyperintense relative to cortex, with hypointense areas due to hemosiderin deposition, most appreciable on T2 weighted image.
F and G, Coronal (F) and axial (G) T1-weighted images obtained after administration of gadopentetate dimeglumine show enhancement of lesion and better define its left lateral ventricular location.
Glasgow coma scale score was 7. Unenhanced CT demonstrated a large intraventricular hemorrhage with acute obstructive hydrocephalus and subarachnoid hemorrhage (Fig . 1 A). After transfer to our institution and bilateral ventriculostomies , cerebral angiography was performed, which demonstrated a small smooth aneurysm in the distal right vertebral artery, prominence of a left lenticulostriate artery , and early opacification of the left thalamostriate vein (Fig. 1 B). There
AJNR :12, July/August 1991 NEUROCYTOMA WITH INTRAVENTRICULAR HEMORRHAGE 767
Fig. 3.-Light microscopic section reveals small, round, uniform nuclei within fibrillary background. Well-formed ring of nuclei is seen surrounding acellular fibrillary center. Wright rosette (arrowheads}. (H and E, original magnification x750)
was no angiographic evidence of neovascularity or late venous tumor blush. T1 -weighted MR imaging, 600/20/1 (TRfTEfexcitations), performed 5 days after the seizure , demonstrated intraventricular hemorrhage and provided no additional information (Figs. 1 C and 1 D) . The diagnosis following these studies was a partially thrombosed intraventricular arteriovenous malformation.
Over the next 3 months the patient slowly made a complete neurologic recovery with the exception of a mild deficit in short-term memory. Serial follow-up unenhanced CT scans demonstrated progressive resolution of the intraventricular hemorrhage and suggested a left lateral ventricular mass (Fig. 2A). Angiography no longer demonstrated the prominent left lenticulostriate artery or the early draining thalamostriate vein. There was no change in the size or configuration of the vertebral artery aneurysm.
An MR study 5 months after initial presentation revealed a mass within the body of the left lateral ventricle (Figs. 2B-2G). The lesion was slightly heterogeneous and isointense relative to the cortex on the T1 -weighted study and slightly hyperintense on the intermediateand T2-weighted images. Tumor enhancement was seen after the administration of gadopentetate dimeglumine (Figs. 2F and 2G).
The patient underwent transcallosal biopsy and resection of the tumor. Light microscopic examination demonstrated small , round , symmetric nuclei forming multiple Wright rosettes (Fig. 3). Hemosiderin was present, but there was no calcification and mitoses were absent . Ultrastructural studies revealed numerous cell processes with neurofilaments and neurotubules, occasional dense core granules, and synaptic vesicles. Although well-formed junctional synapses were not present, the diagnosis of neurocytoma was believed to be appropriate because of the benign appearance of the cells.
Discussion
The terms neurocytoma, intracranial neuroblastoma, and differentiated neuroblastoma have been a source of confusion in recent literature. In 1982, Hassoun et al. [1] described two men who had calcified intraventricular tumors composed of small regular cells with clear cytoplasm that were grouped in
clusters in a fibrillary stroma. Striking neuronal differentiation with numerous synapses was present on electron microscopic examination, and the term central neurocytoma was applied to these unusual tumors [1J. We were able to find only three other cases in the literature that satisfied both the light and electron microscopic criteria of neurocytoma (presence of synapses) as defined by Hassoun; those cases were reported by Townsend and Seaman [5], Poon et al. [6J , and Bolen et al. [9J. Nishio et al. [7J reported six cases designated as neurocytoma and Patil et al. [1 OJ reported one, even though well-formed synapses were not present on electron microscopy in any of these patients. Patil et al. described a second case of neurocytoma in which ultrastructural verification of synapses was not possible because electron microscopy was not performed. To add to the nosologic confusion , Jerdan et al. [2J reported two cases pathologically similar to those described by Hassoun et al. , but , because they lacked synapses , they were termed differentiated neuroblastomas. Wilson et al. [4] reported a similar lesion under the same name in 1985. Ferreol et al. [8J discussed both parenchymal neuroblastomas and intraventricular neurocytomas in their review. The tumor in their case, designated as a neuroblastoma/ neurocytoma, lacked synapses but was similar in other respects to the other neurocytomas reported . The three cases reported by Pearl et al . [3J , under the heading primary cerebral neuroblastoma, were tumors with characteristics similar to those reported as neurocytomas by other authors.
It would appear from our review (see Table 1) that neurocytomas are primarily tumors of the lateral and third ventricles in patients over the age of 15 who have symptoms of increased intracranial pressure. These lesions may or may not be calcified . Of the 18 patients listed in Table 1 in whom CT was performed, calcification was demonstrated in 10. Angiegraphically, these lesions may be vascular or avascular in nature. On light microscopic examination, neurocytomas are composed of mature , small , regular, round neuronal cells with scant cytoplasm, not mature ganglion cells. Ultrastructural examination has revealed the absence of definite synapses in most of the reported cases. Follow-up studies in the majority of these cases have confirmed the benign nature of these tumors , whether or not synapses were present ultrastructurally . We agree with Nishio et al. [7J that these tumors with small , regular, benign-appearing cells should be termed neurocytomas, whether or not synapses are found in the ultrastructure.
Only three tumors reported before our present case were studied by MR imaging [9, 1 OJ . Two were isointense relative to cortex on T1-, proton density-, and T2-weighted imaging while the third demonstrated slight hyperintensity on the proton density- and T2-weighted images. Two demonstrated large vascular flow voids. In our patient, who had an intraventricular hemorrhage, hyperintensity, as well as areas of prominent hypointensity due to the presence of hemosiderin , were seen on proton density- and T2-weighted images obtained 5 months after the ictus. Mild enhancement was seen after administration of gadopentetate dimeglumine.
The most impressive characteristic of these rare lesions is their benign biological activity. A review of the follow-up
TA
BL
E 1
: D
emo
gra
ph
ic a
nd
Im
agin
g F
eatu
res
of
Neu
rocy
tom
as R
epo
rted
in
the
Lit
erat
ure
Refere~=~eT~:
Use
d/
~j,.~
Sex
S
ymp
tom
s at
P
rese
ntat
ion
Lo
catio
n
CT
Fin
ding
s A
ngio
grap
hic
Fin
ding
s M
R F
indi
ngs
Has
soun
et
al.
11 L
19
82
: ce
ntra
l n
eu
rocy
tom
a
1 32
2 39
Jerd
an e
t al
. [2
), 1
98
3:
diff
eren
tiate
d ce
re-
bral
ne
uro
bla
sto
ma
3 4
Pea
rl et
al.
13],
19
85
: pr
i-m
ary
cere
bral
neu
ro-
blas
tom
a 5 6 7
Wils
on e
t a
l.[4
), 1
98
5:
diff
eren
tiate
d ce
re
bral
ne
uro
bla
sto
ma
8
To
wn
sen
d a
nd S
eam
an
[5],
198
6: c
entr
al
ne
uro
cyto
ma
9 10
23
48
23
52
19
25
25
42
M
Pro
gres
sive
me
mo
ry lo
ss,
Late
ral
and
third
Hug
e ca
lcifi
ed m
ass
; N
ot p
erfo
rmed
No
t pe
rfor
med
la
ck o
f in
itiat
ive
, dis
-ve
ntri
cles
no
enh
ance
men
t o
rie
nta
tion
M
P
rogr
essi
ve m
em
ory
loss
, La
tera
l an
d th
ird H
uge
calc
ified
mas
s;
No
t pe
rfor
med
Not
per
form
ed
incr
ease
d IC
P
vent
ricl
es
no m
entio
n o
f en
ha
ncem
ent
M
Incr
ease
d IC
P
M
Incr
ease
d IC
P
M
Incr
ease
d IC
P
F
Incr
ease
d IC
P
M
Incr
ease
d IC
P
M
Incr
ease
d IC
P
F
F
His
tory
of
head
ache
(d
ead
on a
rriv
al)
Dru
g o
verd
ose
Late
ral
vent
ricl
e N
o m
entio
n o
f ca
lci-
Not
per
form
ed N
ot
perf
orm
ed
ficat
ion
or
en-
hanc
emen
t La
tera
l ve
ntric
le
No
men
tion
of
calc
i-N
ot
perf
orm
ed N
ot
perf
orm
ed
ficat
ion
or
en-
hanc
emen
t
Late
ral
vent
ricle
N
o m
entio
n of
cal
ci-
Not
per
form
ed N
ot p
erfo
rmed
fic
atio
n o
r en
-ha
nce
men
t La
tera
l ve
ntric
le
Cal
cifie
d m
ass
; co
ntr
ast
enh
ance
m
ent
Late
ral
ven
tric
le
Ca
lcifi
ed m
ass;
con
tr
ast
enh
ance
m
en
t
Lat
eral
and
thi
rd N
o c
alci
ficat
ion
; no
vent
ricl
es
men
tion
of e
nh
an
cem
en
t
Late
ral
and
thir
d N
ot
perf
orm
ed
vent
ricl
es
Late
ral
vent
ricl
e C
alci
ficat
ion
and
con
tra
st e
nhan
ce
me
nt
Litt
le v
ascu
lar-
Not
per
form
ed
ity;
blus
h in
to v
enou
s ph
ase;
en-
larg
ed p
os-
tero
late
ral,
po
ste
rom
e-
dial
, and
an-
teri
or c
ho-
roid
al a
rter
-ie
s N
o ne
ovas
cu
lari
ty, e
nla
rged
ves
-se
ls, o
r tu
-m
or
stai
n
No
t pe
rfor
med
"Lar
ge m
idlin
e N
ot p
erfo
rmed
va
scu
lar
tu-
mo
r fil
ling
bo
th l
ater
al
vent
ric
les
"
Not
per
form
ed N
ot
perf
orm
ed
Pos
teri
or c
ho
-N
ot p
erfo
rmed
ro
idal
art
ery
su
pp
lyin
g
Pat
holo
gic
Fin
ding
s T
rea
tme
nt
Fol
low
-up
and
Out
com
e
Str
ikin
g ne
uron
al d
iffer
-T
otal
res
ectio
n;
radi
atio
n D
ied
from
men
ingi
tis
entia
tion;
num
erou
s th
erap
y (b
rain
and
at
4 m
on
ths;
no
syna
pses
co
rd)
tum
or
pres
ent
Str
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uron
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-T
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res
ectio
n A
live
at 2
4 m
onth
s;
entia
tion
; nu
mer
ous
no r
ecur
renc
e sy
naps
es
Den
se c
ore
gra
nule
s;
Bio
psy
only
; tr
ea
tme
nt
Fol
low
-up
no
t re
-n
o w
ell-
form
ed s
yn-
no
t de
scri
bed
port
ed
apse
s D
ense
cor
e gr
anul
es;
Bio
psy
only
; tre
atm
en
t F
ollo
w-u
p n
ot
re-
no w
ell-
form
ed s
yn-
no
t de
scri
bed
port
ed
apse
s
No
defin
ite s
ynap
ses
Su
bto
tal
rese
ctio
n; r
a-A
live
at 4
1 12 y
ears
; di
atio
n th
erap
y (w
ho
le
no r
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renc
e br
ain)
N
o d
efin
ite s
ynap
ses
Bio
psy
; rad
iatio
n th
erap
y A
live
at 6
mo
nth
s;
No
defin
ite s
ynap
ses
(who
le b
rain
) no
tu
mo
r g
row
th
bu
t tu
mo
r ce
lls in
C
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psy;
rad
iatio
n th
erap
y A
live;
fol
low
-up
in-
(who
le b
rain
) te
rval
no
t re
po
rted
No
junc
tiona
l sy
naps
es "
Deb
ulki
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Die
d po
stop
erat
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y fr
om b
leed
ing
LM: s
ma
ll, r
ound
nu
clei
, W
rig
ht
rose
ttes
, no
mito
ses;
EM
: un
su
cces
sful
W
ell-d
efin
ed s
ynap
ses
with
ju
nct
ion
s
No
t ap
plic
able
Tot
al r
esec
tion
Dea
d on
arr
ival
Aliv
e at
12
mon
ths;
no
rec
urre
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[6],
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8: p
rim
ary
cere
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neu
ro
bla
stom
a
mas
s; n
o ev
iden
ce o
f in
crea
sed
vasc
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ity
11
17
F
Incr
ease
d IC
P
Th
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entr
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C
alc
ifica
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and
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per
form
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No
t pe
rfor
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S
ynap
tic c
om
ple
xes
with
we
ll-fo
rmed
ju
nctio
ns s
een
thro
ug
ho
ut
Nis
hio
et a
l. [7
], 1
988
: ce
ntra
l ne
uroc
yte-
ma
• 12
13
14
15
16
17
Fer
reol
et
al.
[8],
198
9:
cere
bral
neu
ro
blas
tom
a (n
euro
cy
tom
a)
18
Bol
en e
t al
. [9
], 1
989
: ce
ntra
l neu
rocy
tom
a 19
Pat
il et
al.
[ 1 OJ
, 199
0:
neur
ocyt
oma
20
15
22
22
24
30
39
66
32
34
con
tra
st e
nhan
ce-
men
t
M
Hea
d tr
aum
a (in
cide
ntal
) La
tera
l ve
ntric
le
-
F
Incr
ease
d IC
P
Late
ral
and
third
-
vent
ricle
s F
In
crea
sed
ICP
La
tera
l ve
ntric
le
-
M
Incr
ease
d IC
P
Late
ral
and
third
-
vent
ricle
s M
In
crea
sed
ICP
La
tera
l and
thi
rd
-ve
ntric
les
F
Inci
dent
al h
ead
trau
ma
Late
ral v
entr
icle
-
M
Incr
ease
d IC
P (
head
ache
, T
hird
ven
tric
le
dem
entia
, ata
xia)
C
alci
ficat
ion
(hyp
er
dens
e on
une
nha
nced
sca
n);
dens
e en
hanc
em
ent
F
Incr
ease
d IC
P (
seve
re
head
ache
)
M
Incr
ease
d IC
P (
naus
ea.
vom
iting
, diz
zine
ss, i
nte
rmitt
ent
visu
al l
oss)
, se
izur
e
Late
ral
vent
ricle
La
rge
calc
ified
m
ass
; hom
ogen
eou
s en
hanc
emen
t
Late
ral
vent
ricle
C
alci
ficat
ion
; min
im
al e
nhan
cem
ent
No
t pe
rfor
med
Not
per
form
ed
No
t pe
rfor
med
Not
per
form
ed
No
t pe
rfor
med
Not
per
form
ed
Not
per
form
ed N
ot
perf
orm
ed
Not
per
form
ed N
ot
perf
orm
ed
Not
per
form
ed N
ot
perf
orm
ed
Not
per
form
ed N
ot p
erfo
rmed
N
o m
entio
n o
f sy
nap
ses
on E
M
Ava
scul
ar
T1:
isoi
nten
se r
ei-
Occ
asio
nal
syna
ptic
at
ive
to b
rain
te
rmin
als
, num
erou
s w
ith a
reas
of
neur
onal
cel
l pr
oc-
hypo
inte
nsi
ty
esse
s w
ith m
icro
tu-
(cal
cium
); P
O
buie
s an
d de
nse
and
T2
: inh
o-co
re v
esic
les
mog
eneo
us in
-cr
ease
d si
gnal
in
tens
ity
Not
per
form
ed T
1:
isoi
nten
se r
ei-
Num
erou
s ne
uriti
c at
ive
to c
orte
x;
proc
esse
s w
ith m
i-P
D a
nd T
2:
crot
ubul
es a
nd
isoi
nten
se r
ela
tive
to c
ort
ex
, la
rge
vasc
ular
flo
w v
oids
dens
e co
re g
ranu
les;
no
syn
apse
s pr
esen
t
Tot
al r
esec
tion
Aliv
e at
7 m
onth
s;
no r
ecur
renc
e
Tot
al r
esec
tion
; rad
iatio
n A
live
at 2
9 m
onth
s;
ther
apy
no r
ecur
renc
e P
artia
l re
sect
ion
; rad
ia-
Aliv
e at
19
year
s;
lion
the
rapy
n
o r
esid
ual
tum
or
Tot
al r
ese
ctio
n A
live
at 4
1 12 y
ears
; no
rec
urre
nce
Tot
al r
esec
tion
; rad
iatio
n A
live
at 1
5 m
onth
s;
ther
apy
no r
ecur
renc
e P
artia
l res
ectio
n; r
adia
-A
live
at 1
5 m
onth
s;
tion
ther
apy
no r
esid
ual t
umor
P
artia
l res
ectio
n; r
adia
-A
live
at 2
5 m
onth
s;
lion
the
rapy
re
sidu
al t
umor
pe
rsis
ted
Tot
al r
esec
tion
Sub
tota
l re
sec
tion
Tot
al r
esec
tion
Aliv
e at
4 y
ears
; no
recu
rren
ce
Fol
low
-up
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rval
no
t re
port
ed
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low
-up
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SMOKER ET AL. AJNR :12, July/August 1991
studies in the patients summarized in Table 1 revealed that none had died from tumor growth or recurrence, and survival time was as long as 19 years . This is in contrast to the typical parenchymal neuroblastoma occurring in young children, for which the overall 3-year survival rate is only 60% [11] . Neurocytomas usually arise from the septal region , to which they may be adherent [7] . Complete surgical resection , if possible, appears to be curative [5] . The benefits of adjunctive radiation therapy or chemotherapy in the treatment of these lesions is unclear.
Neurocytomas may be difficult to distinguish from other intraventricular tumors, such as ependymomas, oligodendrogliomas, or meningiomas, which may have similar characteristics on imaging studies. Although neurocytomas are uncommon intraventricular tumors and rarely bleed , they should be considered in the differential diagnosis in patients with intraventricular hemorrhage.
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