Please cite this article in press as: Yaakup NA, et al. Pilomyxoid astrocytoma of the fornix imitating a colloid cyst. Eur J Radiol Extra
(2009), doi:10.1016/j.ejrex.2009.05.009
ARTICLE IN PRESS
G Model
EJREX-796; No. of Pages 4
European Journal of Radiology Extra xxx (2009) xxx–xxx
Contents lists available at ScienceDirect
European Journal of Radiology Extra
journal homepage: intl.elsevierhealth.com/journals/ejrex
Pilomyxoid astrocytoma of the fornix imitating a colloid cyst
Nur Adura Yaakup
a
, Kartini Rahmat
a,*
, Norlisah Mohd Ramli
a
, N. Vairavan
b
,
Vicknes Waran
b
, K.T. Wong
c
a
Department of Biomedical Imaging, Faculty of Medicine, University of Malaya, 50603 Kuala Lumpur, Malaysia
b
Department of Neurosurgery, University of Malaya, 50603 Kuala Lumpur, Malaysia
c
Department of Pathology, University of Malaya, 50603 Kuala Lumpur, Malaysia
article info
Article history:
Received 20 April 2009
Accepted 25 May 2009
Available online xxx
Keywords:
Pilomyxoid astrocytoma
Imaging features
Colloid cyst
abstract
Pilomyxoid astrocytoma is a recently described pediatric brain tumour with distinct histological features
and has been shown to behave more aggressively than pilocytic astrocytoma. Pilomyxoid astrocytomas
usually involve the hypothalamic/chiasmatic region with imaging features similar to pilocytic astrocy-
toma. We report a case of pilomyxoid astrocytoma in the fornix with imaging features imitative of colloid
cyst.
© 2009 Elsevier Ireland Ltd. All rights reserved.
1. Introduction
Pilomyxoid astrocytoma (PMA) is a recently described pediatric
brain tumour. PMAs were previously classified within the pilocytic
astrocytoma (PA) category. However, PMA has distinct histologi-
cal features and has been shown to behave more aggressively than
PA. Majority of previously reported PMAs involved the hypotha-
lamic/chiasmatic region with similar imaging features of PA. We
describe a case of PMA in the fornix with an atypically imaging
features imitative of colloid cyst.
2. Case report
A 6-year-old girl presented with progressive headache and vom-
iting of 1 week with 2 episodes of generalized tonic clonic seizures.
She was brought to the Emergency Department in a confused state
with Glasgow Coma Scale of 13 (E4M5V4). Magnetic resonance
imaging (MRI) of the brain revealed a homogenous, well circum-
scribed, oval mass in the anterior aspect of the third ventricle. This
mass was isointense on T1-weighted, hyperintense on T2-weighted
and FLAIR images. There was no enhancement with intravenous Gd-
DTPA. There was acute obstructive hydrocephalus with obstruction
of the foramina of Monro bilaterally. The third and fourth ventri-
cles were not dilated (Figs. 1 and 2). Computed Tomography (CT)
scan showed a slightly hyperdense lesion (Fig. 3). Initial diagno-
sis of colloid cyst was made based on the clinical presentation
*
Corresponding author. Tel.: +60 163114559; fax: +60 379494603.
E-mail address: katt xr2000@yahoo.com (K. Rahmat).
and imaging findings. The patient underwent a stereotactic endo-
scopic procedure with the purpose to decompress and excise the
cyst as well as to relieve the hydrocephalus. Intraoperatively, the
lesion was found to be solid and lobulated, occupying the anterior
part of the third ventricle measuring about 1.5cm × 2.5 cm. Biopsy
was performed along with septum pellucidotomy and placement of
extra-ventricular drain to relieve the hydrocephalus. Patient subse-
quently underwent craniotomy for excision of the tumour, where a
gel-like tumour arising from the fornix was excised. A residual rim
of tumour was left behind.
Histopathological examination of the specimen showed piloid
astrocytes disposed in a fibrillary to myxoid background. The
tumour cells were uniform with round to oval nuclei and hair-like
cytoplasmic processes. There were no Rosenthal fibers, eosinophilic
granular bodies, mitosis or endothelial proliferation (Fig. 4). The
diagnosis of pilomyxoid astrocytoma WHO Grade 1 was made.
Post-operative recovery was complicated by transient diabetes
insipidus with hypocortisolism and bilateral subdural collections
requiring surgical drainage. There were also neurological compli-
cations where the patient developed generalized spasticity and
cognitive impairment. Patient was discharged 2 months later. A
follow-up MRI was scheduled about 4 months after discharge to
assess the residual tumour and plan further management, but
unfortunately the patient defaulted follow-up.
3. Discussion
Pilomyxoid astrocytoma (PMA) was introduced as a clinico-
pathological entity by Tihan et al. in 1999 [1]. Similar to pilocytic
astrocytoma (PA), PMA may occur anywhere along the neuraxis and
1571-4675/$ – see front matter © 2009 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ejrex.2009.05.009