Monstroeellu
l
ar Sareo
m
a .
A Case of Primary Br.ain Sareoma
ERTU(;RUL SAYIN al, A.SEDA T ÇÖLOOLU bL
aL Department of Neurosurgery, Cerrahpaşa Faculty of Medicine, University of Istanbul, Turkey bL Institute of Forensic Science, University of Istanbul, Istanbul, Turkey
MONSTROSELLÜLER SARKOM Primer Bir Beyin Sarkomu Olgusu
Özet
19 yaşındaki bir erkek hastada saptadığımız Monstrosellüler sarkom olgusunun bildirildi. Hastamız iki kez ameliyat edildi; birinci ameliyatta subtotal, ikinci ameliyatta tümüyle çıkarılan tümörün histopatolojik incelenmesinde, ender görülen bir Monstrosellüler sarkom olgusu olduğu tesbit edildi.
Hastamız ikinci ameliyattan 1 ay sonra eks oldu. Summary
Here is reported a case of Monstrocellular sarcoma observed at a 19 yea.rs old male. it is reported as a "Case Report" because of the discussions about its anatomy and its scarcety is the matter. The patient is 2 times operated. The firs! time we carried out on subtotal extirpation, the further time all the tumoral tissues extirpated. Buı the patient expired a month af ter thal. The incidence of Monstrocellular Cerebral Sareoma encountered in the 1000 cases of brain turnor operated in our clinic
during the last 20 years is no more than 2. This in our opinion the rarity of this type of brain tumor
among st others. The case is also worthy of attention because of its rapidly progressive clinical course. That is why we thought the presen! case would be worth reporting.
Keywords: Primary brain sareoma - Monstrocellu/ar Sareoma - Space-occupying lesion
Case
Report
The patient M. Y., 19 yrs-old male, was admitted to our elinie in 29.9.1975 with complaints of motor weakness of the left upper and lower extremities and strong headache.
History: The first symptoms according to the patient's history consisted of epileptiform attaes
with loss of eonsciousness that started 4 months prior to admission.
Examination: The neurologic examination diselosed bilateral papilledema, positive pathological
reflexes as well as hyperactive tendon reflexes in the left upper and lower extremities, flask paralysis of the left leg as well as hyperesthesia in the left side of the body. Routine laboratory examinations revealed no abnormalities other than hypochrome anemia. A eraniography disclosed mild osteoporosis in sellae turcıca. in the angiographic examination a shift of 2.5 cm of the anterior cerebral artery was found to lefL The middle cerebral artery was depressed at the Sylvian poinL This
findings were in accordance with a space-occupying lesion in the right posterior supra-sylvian
region.
Operation: At the 15th day of admission the patient was operated. A partial extirpation of a subcortical tumoral mass was carried out by right parieto-temporooccipitale craniotomy and. the bone
flap was removed. The postoperative in1provement of the patien!'s general eondition lasted only 3 days followed by a rapid deterioriaıion.
Adlf Tıp Derg.,4 : 97 - 100 (1988)
ADL
İ TIP DERGİSİ
Journal of Forensic Medicine
98
E.SA YIN, A.S.ÇÖlOGlUThe angiographic examination was repeated with similar findings to the previous. The patient was thereafter reoperated and the tumor was totally extirpated.
4 weeks af ter the second operation the paticnt dided.
Figurc 1. Gemistoeytie astroeytes whieh show highly plcomorphism and typical bird-cye nuclcolus eclis that with the dense ehromatine in periphery with visible nuclcus mcmbrane.
( H+E. x 80 )
Figure 2. Highly pleomorphic gi"nt eells. ( H+E, x 250 )
Monstroeellular sareoma
99
DISCUSSION
Giant eeU sareoma are among st rarely eneountered pri
m
ary tumors of the brain and
knowledge on them accumulated in the literature rather searse.
llıisfact
is
refleeted by
the spectrum of names given
to
the same entity by different authors;
MonstroeeııularSareoma, Ganglioglioma, Malignant Ganglioneuroma, Spongioblastoma Multiforme
Ga
n
glio
i
des,
Spongioastroblastoma, Spongioneuroblastoma, Gangliob
lastoma,
Ganglioneuroma or Spongioblastoma Ganglioides, Ganglioneuroma, Limited Sareoma
of
Blood
Vessels, Giant Cell Glioblastoma, G
l
i
oblastoma
GigantoeeııulareGanglioidcs
(1-15).
These are tumors of high malignaney with extremely rapid growth rates. They invade
the dura
mater early in the
ir
course and proliferate into the ex
tr
adural space. Thcir
tendeney for metastasis alsa is considerab
l
e. In one of reported cases, eraniotomy
h
ad
been
followed
by metastasis
to cervical lymph no des (4).
The clinical
hi
story as a mle is of short duration. The first complaints
generaııybegin 5 to 6 months prior to hospital admission and prog
r
ess
rather rapidly.
Cerebral
edema caused by
the tumor
is followed
by
findings of early intraeranial hypertension.
Convu
l
sive phenomena alsa may be presenting symptom in 25% of cases (3,4,12).
Under
the
t
erm of monstroceU
ular sareoma Zülch (12-15) has described
tumors
of
high malignancy characterised with the existenee of bizzare, large giant eells in light
microseopy. The tumor
ceııswith their large, hyperehromatic nuc1ei are suggestive of a
brain groups. A charaeteristie light
mieroscopie
findings is the existenee of
nuCıeolitermed as "bird-eye nucleolus". Tumor vessels are thin and
fomıa retieulum web
(1-3,10-15).
Recent work w
i
th electron mieroseopy has shown that the tumors
eeııs also contaİnnumero
u
s nuclear profiles. One of these profiles is covered with eoncentric such as
m
it
oehondriae, Go
l
giapparatus and ribosomes. The ceU cytoplasm also contains
microtubuli.
The
ceııularorigin of this type of neoplasm
stiııremains controversial.
Replacement of
coııagenousfibres with glial fibres in certain fields,
the
resemblanee of
the
i
nfiltraLiye and invasive pattem to
the one of malignant astrocytoma drew alsa
attenLİon
to a probabIc astroeytie o
ri
gin (5,8).
The
incidence of
monstroccııuIarsareoma is of 1 to 20 amongst
the
glioblastoma as a
whole
and
constitutes only 2.7% of
aııbrain tumors
in
all age groups (4,12-15). It
is
basieally a tumor of adults neverthlcss it
has
alsa been reported in children of the 10-15
age group. It
is more frequent
in girls with a girl to boy of about 5/3 (6,12).
100 E.SA YIN, A.S.ÇÖLOCLU
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15- Zülch, K.I. (1971) Atlas of the Histology of Brain Tumors, pp.220-227, Springer-Verlag, Berlin, Heidelberg, New York.
Reprints request to Doç.Dr. Ertuğrul Sayın Istanbul Üniversitesi Cerrahnpaşa Tıp Fakültesi Nöroşiruriji Anabilim Dalı 34303 Cerrahpaşa, Istanbul
