Intraventricular, Neurologia i Neurochirurgia Polska od 2012
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CASE REPORT/OPIS PRZYPADKU
Intraventricular dysembryoplastic neuroepithelial tumour: case report
Wewn¹trzkomorowy dysembrioplastyczny guz neuroepitelialny – opis przypadku
Hakan Emmez
1
, Aydemir Kale
1
, Emrah Egemen
1
, P
I
nar Eser
2
, Memduh Kaymaz
1
, Ayd
I
n Pa¸aoˇlu
1
1
Department of Neurosurgery, Gazi University Faculty of Medicine, Ankara, Turkey
2
Department of Pathology, Gazi University Faculty of Medicine, Ankara, Turkey
Neurologia i Neurochirurgia Polska 2012; 46, 2: 192-195
DOI: 10.5114/ninp.2012.28266
Abstract
Streszczenie
Dysembryoplastic neuroepithelial tumour (DNT) is located
in the cerebral cortex with very few exceptions. In this arti-
cle, an extremely rare case of intraventricular DNT originat-
ing from the septum pellucidum is reported.
A 25-year-old woman presented with 5-month history of
headache. Cranial magnetic resonance imaging (MRI) scans
revealed a mass in the right lateral and third ventricle which
was hypointense on T1-weighted image, and hyperintense on
T2-weighted images. No contrast enhancement was detect-
ed. The lesion was excised totally using a transcallosal-trans-
ventricular approach. Immunohistochemical examination
revealed DNT. The patient was discharged without any neu-
rological deficits.
Intraventricular DNT presents with symptoms of increased
intracranial pressure rather than seizures. Distinguishing
DNT from other intraventricular tumours is essential as
DNT is characterized by benign clinical course and does not
require adjuvant therapy.
Key words: dysembryoplastic neuroepithelial tumour, cere-
bral ventricles, headache, septum pellucidum.
Dysembrioplastyczne guzy neuroepitelialne (DNT) s¹ umiej-
scowione, z nielicznymi wyj¹tkami, w korze mózgowej.
W bie¿¹cej pracy autorzy przedstawiaj¹ wyj¹tkowo rzadki
przypadek chorej z DNT po³o¿onym wewn¹trzkomorowo,
wychodz¹cym z przegrody przezroczystej.
Chora, 25 lat, zg³osi³a siê z powodu utrzymuj¹cego siê od
5 miesiêcy bólu g³owy. W badaniu za pomoc¹ rezonansu
magnetycznego uwidoczniono guz po³o¿ony w komorze bocz-
nej prawej i w komorze trzeciej, hipointensywny w obrazach
T1-zale¿nych, hiperintensywny w obrazach T2-zale¿nych
i niewzmacniaj¹cy siê po podaniu œrodka kontrastowego. Guz
wyciêto w ca³oœci z dojœcia przez cia³o modzelowate i przez
komorê. W badaniu immunohistochemicznym stwierdzono
DNT. Chora zosta³a wypisana do domu bez ubytkowych
objawów neurologicznych.
Wewn¹trzkomorowe DNT przejawiaj¹ siê raczej wyst¹pie-
niem objawów wzmo¿onego ciœnienia œródczaszkowego ni¿
napadów padaczkowych. Odró¿nianie DNT od innych guzów
wewn¹trzkomorowych jest nieodzowne ze wzglêdu na fakt, ¿e
guz przebiega ³agodnie i nie wymaga leczenia wspomagaj¹cego.
S³owa kluczowe: dysembrioplastyczny guz neuroepitelialny,
komory mózgu, ból g³owy, przegroda przezroczysta.
nagh
et al.
in 1958 [1,2]. Daumas-Duport
et al.
in 1988
[1] improved our understanding of DNT by revealing
that the tumour of that type is mostly seen in children
and young adults and typically occurs in the supraten-
torial cortex. The tumour usually manifests with heada-
Introduction
Dysembryoplastic neuroepithelial tumour (DNT)
was first defined as an oligodendroglioma-like tumour
in temporal lobe specimens of epileptic patients by Cava-
Correspondence address: Aydemir Kale, Gazi Üniversitesi T
I
p Fakültesi, Beyin ve Sinir Cerrahisi AD 1. Kat, 06500, Ankara, Turkey,
phone: 00 90 533 223 31 32; fax: 00 90 312 212 90 04, e-mail: aydemirkale@gmail.com
Received: 17.05.2011; accepted: 19.09.2011
192
Neurologia i Neurochirurgia Polska 2012; 46, 2
Intraventricular dysembryoplastic neuroepithelial tumour
che and intractable seizures. Although DNT behaves
in a benign fashion, radiological features are similar to
low-grade gliomas. To the best of our knowledge, pre-
vious studies have reported 22 intraventricular DNT
cases including intraventricular, nucleus caudatus and
septum pellucidum [2-13]. This report presents clini-
cal, radiological and histological features of an intra-
ventricular DNT in a young adult.
images. No contrast enhancement was observed. The tu-
mour was removed totally using the transcallosal and
transventricular approach (Fig. 2). The greyish and soft
tumour originated from the septum pellucidum. The tu-
mour extended to the third ventricle through the fora-
men of Monro and had very low vascularization. Immu-
nohistochemical studies showed columnar nature,
floating neuronal cells and synaptophysin reactivity that
established the diagnosis of DNT (Fig. 3). The patient
was discharged three days after the operation without
any neurological deficit.
Case report
A 25-year-old woman presented with progressive
headache, nausea and vomiting for 5 months. There was
no seizure in her medical history. No abnormal findings
were present in general or in neurological examination.
Cranial magnetic resonance imaging (MRI) scans reve-
aled a non-enhanced mass lesion (19
Discussion
Dysembryoplastic neuroepithelial tumour is a be-
nign, focal, intracortical tumour including a cystic com-
ponent inside [10,11]. Dysembryoplastic neuroepit-
helial tumours present mostly with headache and
intractable seizures. Nolan
et al.
[11] reported that
DNT is involved in 14-18% of surgically treated epi-
lepsy cases. Headache, which is the primary symptom
12 mm)
originating from the inferior septum pellucidum that
extended to the third ventricle through the right fora-
men of Monro (Fig. 1). The tumour was hypointense on
T1-weighted image and hyperintense on T2-weighted
×
15
×
A
B
C
D
E
F
Fig. 1. A-C) The tumour adjacent to the inferior part of the septum pellucidum is hypointense on T1-weighted image and hyperintense on T2-weighted MR image.
D-F) Non-enhancing tumour extending to third ventricle through foramen of Monro
193
Neurologia i Neurochirurgia Polska 2012; 46, 2
Hakan Emmez, Aydemir Kale, Emrah Egemen, P
I
nar Eser, Memduh Kaymaz, Ayd
I
n Pa¸aoˇlu
in intraventricular DNTs, was also the dominant symp-
tom in the presented case.
Although the most common location of DNT is the
supratentorial cortex, often the temporal lobe, it is also
possible to encounter DNT in various extracortical loca-
tions corresponding to the secondary germinal layers,
including the dentate fascia, subpial germinal layer, sub-
ependymal germinal layer, and external granular layer
in the cerebellum. So far, 22 DNT cases with extracor-
tical locations have been reported. The most common
extracortical locations are the septum pellucidum and
nucleus caudatus. As in the present case, it is also possi-
ble to encounter cases with extension of the tumour from
the foramen of Monro to the third ventricle [3,5,6].
The relation to the septum pellucidum, multilobu-
lated appearance, T2-bright signal reflecting high mucin
content, and a lack of contrast enhancement are the ima-
ging features of intraventricular DNTs [5].
Although DNT shares some features with oligo-
dendroglioma, it generally occurs intracortically and has
distinctive multinodular form. Oligodendrocyte-like
cells and floating neurons in a mucinous matrix which
are the typical histological features of DNT were obse-
rved in the present case.
The pathophysiology is still controversial. Although
malignant transformation of DNT has been reported,
it is still unclear whether DNT is a neoplasm or a ha-
martoma [14]. Some factors, such as accompanying cor-
tical dysplasia, young age and deformity of the overly-
ing skull, connote dysembryogenetic origin [13].
The familial occurrence of intraventricular DNT was
reported by Saito
et al
. [15], which suggests that these
tumours may arise from germline mutation [15].
The largest series was reported by Baisden
et al.
in
2001 and included 3 children and 7 young adults [3].
Cervera-Pierot
et al.
[6] reported four patients with
Fig. 2. Computed tomography performed 5 days after surgery shows the gross-
total excision of the tumour
tumours that occurred in the nucleus caudatus and
extended to the intraventricular area. Cataltepe
et al.
[5]
recently reported a similar case, a 15-year-old boy pre-
senting with epilepsy. Unlike the previous cases, the
lesion extended from the corpus callosum to the septum
pellucidum [3]. Bilginer
et al.
reported a very intere-
sting intraventricular DNT in a 9-year-old boy with dis-
seminated spinal tumour [4].
In the presented case and in the previous cases, the
tumour was soft and sparsely vascular. Concerning the-
se features, endoscopic removal of intraventricular DNT
seems to be a safe and easy procedure. Harter
et al.
[8]
reported a similar case which was successfully removed
by endoscopy.
Intraventricular DNT is extremely rare but distin-
guishing this entity from more common intraventricu-
A
B
C
Fig. 3. A) Columns formed by bundles of axons oriented perpendicularly to the cortical surface. Neurons with normal cytology float between those columns on the pale
eosinophilic background (H&E, ×40). B) Cytologically normal floating neurons with ganglion cells (H&E, ×200). C) Synaptophysin immunoreactivity of neuronal
cell processes and ganglion cells (synaptophysin, ×200)
194
Neurologia i Neurochirurgia Polska 2012; 46, 2
Intraventricular dysembryoplastic neuroepithelial tumour
lar tumours, such as low-grade gliomas, ependymomas,
or neurocytomas, is vital, since total removal provides
a favourable prognosis and adjuvant therapy is not requ-
ired. Rushing
et al.
[16] reported a patient with DNT
that showed malignant transformation after radiation
and chemotherapy [14].
11. Nolan M.A., Sakuta R., Chuang N., et al. Dysembryoplastic
neuroepithelial tumors in childhood.
Neurology
2004; 62:
2270-2276.
12. Ongürü O., Deveci S., Sirin S., et al. Dysembryoplastic
neuroepithelial tumor in the left lateral ventricle.
Minim Invasive
Neurosurg
2003; 46: 306-309.
13. Wang F., Qiao G., Li X., et al. A dysembryoplastic neuro-
epithelial tumor in the area of the caudate nucleus in a 57-year-
old woman: case report.
Neurosurgery
2007; 61: E420.
14. Hammond R.R., Duggal N., Woulfe J.M., et al. Malignant
transformation of a dysembryoplastic neuroepithelial tumor. Case
report.
J Neurosurg
2000; 92: 722-725.
15. Saito T., Sugiyama K., Yamasaki F., et al. Familial occurrence of
dysembryoplastic neuroepithelial tumor-like neoplasm of the
septum pellucidum: case report.
Neurosurgery
2008; 63: 370-372.
16. Rushing E.J., Thompson L.D., Mena H. Malignant trans-
formation of a DNT after radiation and chemotherapy.
Ann
Diagn Pathol
2003; 7: 240-244.
Conclusions
Intraventricular DNTs usually originate from the
septum pellucidum and present with headache. They
should be considered in the differential diagnosis of
intraventricular tumours.
Disclosure
Authors report no conflict of interest.
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Neurologia i Neurochirurgia Polska 2012; 46, 2
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