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Pleomorphic xanthoastrocytoma (PXA) is uncommon
slow growing tumor which is responsible for <1% of all astrocytic tumors.
PXA incidence is frequently in the second decade of life and has a favorable
outcome. We present the clinical, radiological and histopathological features
of PXA involving the right thalamus and midbrain in a 9 year old girl with
right upper motor neuron facial paresis of three weeks duration. Histopathology
confirmed the diagnosis of PXA. Our case is unique in that our patient is the
first one reported in the literature with midbrain and thalamus involvement.
Radiotherapy may have a role to play in patients with incomplete resection. PXA
usually presents with seizures, dizziness, and headache or rarely patients are
asymptomatic. Patterns of presentation can be purely cystic, mixed cystic-solid
and purely solid
Keywords: Pleomorphic xanthoastrocytoma, Midbrain,
Thalamus
INTRODUCTION
Pleomorphic
xanthoastrocytoma (PXA) accounts for <1% of all astrocytic neoplasms. PXA
was first described by Kepeset [1]. In 1979 as a distinct clinic pathological
entity in 12 young patients and was subsequently classified as Grade 2
astrocytoma by WHO in 1993 [2]. PXAs developed gradually and usually happen in
the second decade of life with no sex preference [3,4]. Headache and
long-lasting seizures are the presenting properties [5,6]. PXAs are superficial
tumors with a preference for the temporal lobe followed by parietal, frontal and
occipital lobes. Other uncommon sites are cerebellum, spinal cord and retina
[3]. In spite of favorable prognosis, PXA malignant transformation has been
depicted as an anaplastic variant [3,7,8].
Although PXA
commonly affects young patients, this entity has been depicted in elderly
patients with variable prognosis [5,6,9]. We present a rare case of PXA
involving the right thalamus and midbrain a 9 year old Iranian girl with right
upper-motor neuron (UMN) facial paresis. Our case is unique in that our patient
is the first one reported in the literature with midbrain and diencephalic PXA.
CASE REPORT
A 9 year old girl
presented to the neurosurgery department of Shohada Tajrish hospital with right
sided facial paresis of three weeks duration left simple partial motor seizure
lasting about one year. She also complained of headache in occipital area.
There was no history of decreased sensations over the face or similar episodes
in the past. She had no history of trauma, vomiting, fever or other associated
symptoms.
On examination,
higher mental functions were intact. There was an UMN type of facial paresis on
the right side. Rest of the cranial nerves and neurological examination was
intact.
Computed tomography scan of the brain with
and without contrast revealed an intra-axial heterogeneously hyper dense mass
in the right thalamus with extension to cerebral peduncle and quadrigeminal
cistern. Brain MRI revealed an intra-axial solid lesion in the right thalamus
extending into cerebral peduncle and quadrigeminal cistern. The lesion was hypo
to isointense on T1-weighted images, heterogeneously hyper intense on
T2-weighted and showed heterogeneous enhancement on gadolinium contrast.
The patient underwent a streotactic biopsy of
thalamic lesion. The tumor was intra-axial in location, grayish white in color
and partially firm consistency (Figure
1).
Histolopathological
examination revealed brain tissue involved by a low moderately neoplasm
composed of neoplastic astroglial cells with fibrillary background. Occasional
large bizarre cells were identified .no evidence of increased mitotic activity.
No evidence of increased mitotic activity, necrosis or vascular endothelial
proliferation was seen. IHC study revealed diffusely positive CD34 and Ki-67
was positive in less than 1% of tumoral cells. Pathological findings were in
favor of low grade glioma compatible with pleomorphic xanthoastrocytoma (Figure 2).
Pleomorphic
xanthoastrocytoma is an uncommon slow growing astrocytic tumor. These tumors
frequently involve the supratentorial compartment with a preference for the
temporal lobe [3,6]. They are thought to originate from the subpial astrocytes
as a result of presence of “basal lamina”, which is a typical property of these
astrocytes [1,2,10,11]. Affected patients are frequently in their first three
decades of life [3,5,9,12]. Reported a left fronto-temporal PXA in a 76 year
old female, who underwent subtotal excision and palliative radiation therapy
[6]. However, the patient died 6 months later as result of the rapid
progression of the tumor. Perry et al. [9] described a composite PXA and
ganglioglioma in an 82 year old male patient involving the left frontal lobe,
who was subsequently lost to follow-up. Similarly, Bucciero et al. [5] reported
an atypical PXA in a 65 year old man affecting the left capsulo-thalamic region
that was treated with subtotal excision and fractionated external beam
radiotherapy. The patient died 22 months after the excision as result of a
massive recurrence of the tumor. Our patient was a 9 year old Iranian girl who
was treated with brain stereotactic biopsy.
The prominent
histopathological features of PXA as described by Kepes et al. [1] includes
marked cellular pleomorphism with spindle cells, multinucleated giant cells
with bizarre nuclei, prominent lipid droplets, eosinophilic granular bodies,
perivascular lymphocytic infiltration and dense reticulin network. Mitotic
figures are rare and necrosis is frequently absent. On immunohistochemical
analysis, GFAP and S-100 is positive in all tumors [12]. Synaptophysin
reactivity ranges from 38% to 100% and neuro filament protein expression is
seen in 8-71% of tumors [3,12,13]. Although PXA has been known as a tumor with
a favorable prognosis, 20% of these tumors can progress to malignancy and
categorized anaplastic PXA [3,6-8,14]. Various factors such as increased
mitotic activity, high Ki-67, MIB-1, proliferating cell nuclear antigen
labeling index, endothelial proliferation, presence of necrosis, diffuse
proliferation of monomorphic cells, decreased reticulin fibers and the extent
of surgical resection, have been described to predict the unfavorable outcome
in patients with PXA [3,6,7,9,12,15].
1. Kepes
JJ, Rubinstein LJ, Eng LF (1979) Pleomorphic xanthoastrocytoma: A distinctive
meningocerebral glioma of young subjects with relatively favorable prognosis. A
study of 12 cases. Cancer 44: 1839-1852.
2. Kepes
JJ (1993) Pleomorphic xanthoastrocytoma: The birth of a diagnosis and a
concept. Brain Pathol 3: 269-274.
3. Giannini
C, Scheithauer BW, Burger PC, Brat DJ, Wollan PC, et al. (1999) Pleomorphic xanthoastrocytoma:
What do we really know about it? Cancer 85: 2033-2045.
4. Herpers
MJ, Freling G, Beuls EA (1994) Pleomorphic xanthoastrocytoma in the spinal
cord: Case report. J Neurosurg 80: 564-569.
5. Bucciero
A, De Caro M, De Stefano V, Tedeschi E, Monticelli A, et al. (1997) Pleomorphic
xanthoastrocytoma: Clinical, imaging and pathological features of four cases.
Clin Neurol Neurosurg 99: 40-45.
6. Ng
WH, Lim T, Yeo TT (2008) Pleomorphic xanthoastrocytoma in elderly patients may
portend a poor prognosis. J Clin Neurosci 15: 476-478.
7. Bayindir
C, Balak N, Karasu A, Kasaroglu D (1997) Anaplastic pleomorphic
xanthoastrocytoma. Childs Nerv Syst 13: 50-56.
8. Lubansu
A, Rorive S, David P, Sariban E, Seligmann R, et al. (2004) Cerebral anaplastic
pleomorphic xanthoastrocytoma with meningeal dissemination at first
presentation. Childs Nerv Syst 20: 119-122.
9. Perry
A, Giannini C, Scheithauer BW, Rojiani AM, Yachnis AT, et al. (1997) Composite
pleomorphic xanthoastrocytoma and ganglioglioma: Report of four cases and
review of the literature. Am J Surg Pathol 21: 763-771.
10. Gill
M, Pathak HC, Madan R, Bhattacharya S, Choudhary GS (2010) Primary spinal
pleomorphic xanthoastrocytoma. Neurol India 58: 771-773.
11. Nakamura
M, Chiba K, Matsumoto M, Ikeda E, Toyama Y (2006) Pleomorphic xanthoastrocytoma
of the spinal cord: Case report. J Neurosurg Spine 5: 72-75.
12. Sharma
A, Nand Sharma D, Kumar Julka P, KishorRath G (2011) Pleomorphic
xanthoastrocytoma - A clinico-pathological review. Neurol Neurochir Pol 45:
379-386.
13. Giannini
C, Scheithauer BW, Lopes MB, Hirose T, Kros JM, et al. (2002) Immunophenotype
of pleomorphic xanthoastrocytoma. Am J Surg Pathol 26: 479-485.
14. Asano
K, Miyamoto S, Kubo O, Kikkukawa T, Yagihashi A (2006) A case of anaplastic
pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal
fluid dissemination. Brain Tumor Pathol 23: 55-63.
15. Macaulay
RJ, Jay V, Hoffman HJ, Becker LE (1993) Increased mitotic activity as a
negative prognostic indicator in pleomorphic xanthoastrocytoma: Case report. J
Neurosurg 79: 761-768.
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