G. Yoshikawa et al.

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Neurol Med Chir (Tokyo) 50, 220¿223, 2010

Massive Intracranial Hemorrhage Associated
With Pleomorphic Xanthoastrocytoma
—Case Report—
Gakushi YOSHIKAWA, Shunsuke KAWAMOTO,
Kyoko YAKOU, and Kazuo TSUTSUMI
Department of Neurosurgery, Showa General Hospital, Kodaira, Tokyo

Abstract
A 60-year-old woman with a history of intermittent headaches and frequent seizures for 30 years
presented with a massive intracranial hematoma in the left medial temporal lobe with thick
subarachnoid hemorrhage. She had been treated with anticonvulsant medication under a diagnosis of
left mesial temporal sclerosis based on magnetic resonance imaging findings. Cerebral angiography on
admission revealed occlusion of the P2 segment of the left posterior cerebral artery (PCA) and extravasation of contrast medium during the procedure. The patient underwent left temporal lobectomy including the lesioned mesial temporal cortex, and the ruptured P2 segment of the PCA was removed as well.
The operative finding of the ruptured aneurysm was pseudoaneurysm. Histological examination of the
resected PCA segment demonstrated a pleomorphic xanthoastrocytoma invading the outer wall of the
PCA. Presumably the bleeding was caused by the rupture of a pseudoaneurysm secondary to leptomeningeal involvement of this typically benign tumor.
Key words: intracranial hemorrhage, leptomeningeal involvement,
subarachnoid hemorrhage, pseudoaneurysm

Received

April 9, 2009;

Accepted

pleomorphic xanthoastrocytoma,

August 3, 2009

Neurol Med Chir (Tokyo) 50, March, 2010

Intracranial Hemorrhage With Pleomorphic Xanthoastrocytoma

221

Introduction
Pleomorphic xanthoastrocytoma is a rare subtype of glial
neoplasm, which was first described as a distinct form of
supratentorial astrocytoma in 1979.8) The tumor is typically located in the superficial temporal cortex, and the most
common clinical presentation is seizures, although
headache is also common in young patients. In contrast to
the pleomorphic cytology, the tumor shows benign biological behavior.3,8) Hemorrhage associated with low-grade
astrocytoma is unusual, with few reports of hemorrhage
associated with pleomorphic xanthoastrocytoma.11,22,23)
Leptomeningeal involvement is a frequent finding in pleomorphic xanthoastrocytoma, but arterial invasion is
unknown.
We report an unusual case of intracranial hemorrhage
supposedly due to rupture of a pseudoaneurysm secondary to leptomeningeal involvement by pre-existing
pleomorphic xanthoastrocytoma in the adjacent area.

Fig. 1 Coronal T2-weighted magnetic resonance image obtained 3 years before onset showing high intensity in the left
parahippocampal gyrus with no enhancement.

Case Report
A 60-year-old-woman presented with sudden onset of
coma and right hemiparesis. She had a history of intermittent headache and frequent seizures for 30 years, and had
been treated with anticonvulsants by a neurologist at
another hospital under a diagnosis of left mesial temporal
sclerosis based on magnetic resonance imaging (Fig. 1),
which was obtained 3 years before the onset. She had no
history of hypertension or trauma, no sign of infection,
and was afebrile on admission. Computed tomography on
admission showed a massive hematoma compressing the
midbrain in the left ambient cistern and partly extending
to the left thalamus accompanied by diffuse thick
subarachnoid hemorrhage (Fig. 2). Cerebral angiography
demonstrated occlusion of the posterior cerebral artery
(PCA) at the P2 segment and subsequent extravasation of
the contrast material (Fig. 3). The angiographical findings
were consistent with rupture of the P2 segment of the PCA.
Since her left pupil was dilated at the time of the extravasation, the patient was directly taken to surgery.
Emergent decompressive craniectomy was performed.
The hematoma was evacuated, and temporal lobectomy
was performed to alleviate uncal herniation. During the
procedure, troublesome re-bleeding from the friable wall
of the PCA was encountered, as likely with a pseudoaneurysm. Therefore, the proximal and distal ends of the
P2 segment of PCA were obliterated with clips, but unfortunately a specimen of the most friable segment of the
PCA, which was supposed to be the rupture site, could not
be obtained because of the technical damage caused by the
hemostatic procedure. Both the medial temporal segment
and the residual P2 segment of the PCA were resected.
Histological examination showed a glial tumor with
marked pleomorphism of the tumor cells, including spindle cells and `bizarre' giant cells with foamy cytoplasm.
Eosinophilic granular bodies were seen in some foci (Fig.
4A). Reticulin staining showed a fine network of reticulin
surrounding the tumor cells. Glial fibrillary acidic protein
(GFAP) staining was positive in most parts of the tumor,

Neurol Med Chir (Tokyo) 50, March, 2010

Fig. 2 (A) Computed tomography scan at the onset demonstrating massive hematoma in the left ambient cistern and
thick subarachnoid hemorrhage. (B) Computed tomography
scan demonstrating intraaxial hematoma in left thalamus.

Fig. 3 (A) Left vertebral angiogram (anteroposterior view)
showing occlusion of the posterior cerebral artery. (B) Subsequent left vertebral angiogram (anteroposterior view) showing
extravasation of contrast medium.

222

G. Yoshikawa et al.
invading the adventitia of the vessel (Fig. 5A). Specimens
obtained from the perivascular areas were positive for
GFAP (Fig. 5B).
The patient underwent cranioplasty for the skull defect,
and ventriculo-peritoneal shunting for hydrocephalus, but
she remained bed-ridden due to the initial brain damage
including the left thalamus and left cerebral infarction
caused by subsequent vasospasm (Fig. 6).

Fig. 4 (A) Photomicrograph showing markedly pleomorphic
cells with giant and bizarre nuclei, and eosinophilic granular
bodies, but no foci of necrosis. Hematoxylin and eosin stain,
original magnification ×100. (B) Photomicrograph showing
MIB-1 index of less than 1%. ×200.

Fig. 5 (A) Photomicrograph of the posterior cerebral artery
showing abnormal giant and bizarre cells around the perivascular space. Foci of invasion into the adventitial layer are seen.
Hematoxylin and eosin stain, ×100. (B) Photomicrograph of
the abnormal giant cells around the perivascular area showing
positive immunoreactivity to glial fibrillary acidic protein
(GFAP) staining. The blue layer is the elastic laminae of the artery. GFAP and elastica stain, ×100.

Fig. 6 Computed tomography scan demonstrating the defect
of the left temporal lobe 3 months after the surgery.

though the MIB-1 index was less than 1% (Fig. 4B). No
necrotic foci were identified. These findings were consistent with pleomorphic xanthoastrocytoma. Areas adjacent
to the ruptured PCA contained numerous bizarre and giant cells aggregating around the perivascular space and

Discussion
Bleeding due to intracranial neoplasms is relatively uncommon and occurs in only 1.5% of cases.12) Intracranial
hematoma is more frequent in glioblastoma/anaplastic
gliomas and meningiomas than in other tumors. The incidence of bleeding from gliomas is approximately 5%,9,13)
and is especially more common in high-grade gliomas
such as glioblastoma/anaplastic astrocytoma, which
shows tumor necrosis or endothelial proliferation,7,16)
whereas low-grade astrocytomas comprise less than 1% of
all tumors associated with hemorrhage.10)
Only a few cases of hemorrhage associated with lowgrade astrocytomas have been reported.2,4,5,14,17,20) Possible
causes of intratumoral hemorrhage include endothelial
proliferation with vascular obliteration, vessel compression and/or distortion due to rapid tumor growth, vascular
necrosis, abnormal structure of tumor capillaries such as
retiform capillaries, and invasion of vessel walls by the
tumor.7,13,16,20) However, which histological features and
what mechanisms actually cause such bleeding remain unclear.4)
In the first reported case of intracerebral and
subarachnoid bleeding from a pleomorphic xanthoastrocytoma, temporal lobe and adjacent meningeal involvement by the neoplasm was proposed to have eventually
produced erosion of an artery on the surface of the brain,
but the cause of bleeding was not identified by imaging
such as angiography.11) Two other case reports described
pleomorphic xanthoastrocytoma presenting with intratumoral bleeding.22,23) An unknown microscopic vascular
anomaly within the tumor parenchyma was proposed to
induce massive bleeding, whereas leptomeningeal invasion was unlikely because of the absence of subarachnoid
hemorrhage.
Our present case is the fourth hemorrhagic presentation
of pleomorphic xanthoastrocytoma. Cerebral angiography
revealed occlusion of the PCA and subsequent extravasation in areas adjacent to the hematoma. Operative findings
included the very friable wall of the PCA that resulted in
troublesome intraoperative re-bleeding, which was consistent with a pseudoaneurysm. She had no history of hypertension, trauma, or infectious event. Histological examination revealed that typical pleomorphic xanthoastrocytoma had surrounded the PCA and invaded its outer wall,
although no specimen of the ruptured site of the PCA was
obtained because of damage caused by the hemostatic
procedure during the operation. A previous case of
anaplastic pleomorphic xanthoastrocytoma with tumor
bleeding had MIB-1 index of 9.8% indicating that malignant transformation might induce tumoral hemorrhage.1)

Neurol Med Chir (Tokyo) 50, March, 2010

Intracranial Hemorrhage With Pleomorphic Xanthoastrocytoma
In contrast, our case of bleeding pleomorphic xanthoastrocytoma had MIB-1 index of less than 1% and no foci of
necrosis, so malignant transformation was unlikely as the
cause of tumor bleeding.
The mechanisms of intracerebral hemorrhage from
brain tumor are complex and involve multiple factors, but
intracerebral hemorrhage from the neoplastic cerebral
aneurysm is rare.6,15) Massive intracerebral hematoma induced by a neoplastic aneurysm caused by metastasis of
lung carcinoma may have originated from a tumor cell
migrating from the original site invading the wall of the
peripheral artery, resulting the formation of an aneurysmal dilatation. On the other hand, glioblastoma with
intratumoral bleeding was caused by the feeding artery
pedicle aneurysm, which was presumably be formed by
hemodynamic stress in the arteriovenous shunting associated with high-grade glioma such as glioblastoma.
Unfortunately we were not able to detect tumoral invasion
penetrating the arterial wall because of tissue damage, but
we could show the pre-existing brain tumor and the
resultant hemorrhage from the ruptured aneurysm which
was directly attached to the tumor. Moreover, we demonstrated GFAP-positive tumor cells surrounding the outer
wall of the posterior cerebral artery. Therefore, we
speculate that the direct mural invasion of the tumor most
likely caused formation of the pseudoaneurysm and subsequent rupture of the PCA, which led to the intraparenchymal and subarachnoid hemorrhage. Pleomorphic xanthoastrocytoma is well known for the presence of prominent basal lamina surrounding the tumor cells, which, in
addition to its tendency to involve the leptomeninges, suggests subpial astrocytes as the possible origin of this rare
tumor.8,18,19,21) Invasion of vessel walls has not been recognized as a clinicopathological feature of pleomorphic xanthoastrocytoma, presumably because this tumor mostly involves the cerebral cortex and is usually not located in the
proximity of major vessels. The present case is an important example of this interesting phenomenon, which
may be helpful in the management of pleomorphic xanthoastrocytoma.

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Address reprint requests to: Gakushi Yoshikawa, M.D., Department of Neurosurgery, Showa General Hospital, 2–450 Tenjin–cho, Kodaira, Tokyo 187–8510, Japan.
e-mail: gyoshika-tky＠umin.ac.jp