H. Guesmi et al., Dysembryoplastic neuroepithelial tumors. Features in eight cases, including two with unusual locations., NEUROCHIRE, 45(3), 1999, pp. 190-200
Objectives. - Dysembryoplastic neuroepithelial tumors (DNTs) are usually lo
cated within the supratentorial cortex. We present a series of eight cases
of DNTs including two cases with an extracortical location, one in the caud
ate nucleus, the other one expanded in the lateral ventricule, septum and f
ornix. An origin from secondary germinal layers, as previously suggested, c
an explain these extracortical locations.
Material and methods. - Of the eight patients; seven had partial epileptic
seizures es and one intracranial hypertension. All patients underwent clini
cal examination, a computed tomographic (CT) scan, a magnetic resonance ima
ging (MRI) and a surgical removal of the lesion with histological examinati
on.
Results. - Clinical examination was normal except in the case with intracra
nial hypertension where a bilateral papillary oedema was found In seven cas
es the CT scan showed a hypodense lesion of pseudocystic appearance. All le
sions were hypointense on T2-weighted and hyperintense on T2-weighted MRI.
Contrast enhancement was observed in two cases. The lesion was intracortica
l in six cases and extracortical in the remaining two : one in the head of
the caudate nucleus and one in the trigo-noseptal region. Histological exam
ination identified an appearance of DNT with a specific glioneuronal elemen
t in six cases.
Conclusion. - The diagnosis of DNT can be suspected before histological exa
mination on radiological features, chiefly because the tumor is located in
the supratentorial cortex. However, extracortical locations no exist, even
if unusual. As DNTs are always benign, knowledge and accurate diagnosis of
these atypical cases are mandatory in order to avoid useless and even delet
erious additional treatments, such as radiotherapy.