doi:10.1111/j.1750-3639.2009.00277.
x
CASE OF THE MONTH OCTOBER 2008 (CASE 2)
A 33-YEAR-OLD CHINESE WOMAN WITH A LEFT FRONTAL
TUMOR
CASE HISTORY
A 33-year-old Chinese woman presented with intermittent slurring
of speech, dysphasia together with right upper limb and facial
weakness for two months with gradual worsening of the symptoms.
Physical examination found decreased pin-prick sensation over
right C6 to C8 dermatome and impaired proprioception in right
hand. CT scan with contrast showed a well-demarcated contrast-
enhancing left frontal tumor measuring 4.5 ¥ 4.2 ¥ 3 cm with
perilesional edema and slight mass effect. Cystic changes were
observed. The tumor was close to the cortical surface but not con-
nected to the meninges (figure 1). Surgical exploration found a
non-encapsulated, well-circumscribed, vascularized tumor in the
left frontal lobe. Tumor debulking under intraoperative cerebral
function monitoring was performed. Around 95% of tumor was
removed but complete excision could not be achieved due to sig-
nificant decrease in amplitude of the brain motor evoked potentials.
The patient recovered well after the operation with complete resto-
ration of the function in the precentral and postcentral gyri as well
as Broca’s area.
PATHOLOGIC FINDINGS
Multiple pieces of soft greyish fragments altogether measuring
3 ¥ 2 ¥ 2 cm in aggregate were sent for pathological examination
in fresh state. They were used in intraoperative cytologic smear,
frozen section and subsequent histology as well as ultrastructural
examination.
Intraoperative smears showed loose aggregates of polygonal and
rhabdoid cells with eccentric nuclei and abundant cytoplasm. Intra-
cytoplasmic inclusion bodies were occasionally seen. No signifi-
cant cellular atypia or necrotic material was discerned. A glial
fibrillary background was not evident. Neither psammoma bodies,
intranuclear inclusions, nor papillary structures were found.
Figure 1.
Brain Pathology 19 (2009) 337–340
© 2009 The Authors; Journal Compilation © 2009 International Society of Neuropathology
Histologic sections showed a malignant tumor arranged in sheets
and pseudopapillary pattern (figure 2). A distinct border was appre-
ciated between the tumor and non-tumorous glial tissue. Most
of the tumor cells displayed a rhabdoid appearance with uniform,
eccentric nuclei and eosinophilic cytoplasm. Intracytoplasmic
globules were observed in places (figure 3). Some of the tumor
cells showed a primitive appearance. Perivascular pseudorosettes
as characterized by stout cytoplasmic processes radiating towards
central hyalinized blood vessel were easily found (figure 4). No
fibrillary matrix was seen in the stroma. Stromal hyalinization and
calcifications were focally present. No palisaded necrosis was seen.
Four mitotic figures were identified per 10 high power fields.
The rhabdoid tumor cells displayed a multilineage immunohis-
tochemical profile. They were focal but strongly immunoreactive
for glial marker glial fibrillary acidic protein (GFAP) (figure 5),
diffuse and strongly positive for neural marker S100-protein
(figure 6) and focal but strongly reactive for cytokeratin markers
MNF116 and Cam5.2 (figure 7). In addition, diffuse and strong
membranous and cytoplasmic dot-like pattern was appreciated
with epithelial membrane antigen (EMA) (figure 8). The tumor
cells were diffusely positive for vimentin. No neuronal differentia-
tion was demonstrated with synaptophysin and neurofilament.
There was no loss of INI-1 protein (not shown). Dual-color FISH
analysis was performed on paraffin sections with a target probe
generated from BAC clone RP11-71G19 (22q11.23), which covers
the entire SMARCB1 gene (associated with rhabdoid tumors), and
the reference probe from RP11-494O16 (22q13.33). No deletion
was discerned. The proliferation index was approximately 10%.
The tumor cells were negative for HMB-45, actin and desmin.
Ultrastructural studies showed whorls of intermediate filaments
in the cytoplasm of the rhabdoid cells (figure 9). In addition,
microvillous projections were observed on the cell surface
(figure 10). An occasional intercellular junction was identified. No
cilia were seen.
Figure 2.
337
Correspondence

Figure 3.

Figure 4.

338 Brain Pathology 19 (2009) 337–340

© 2009 The Authors; Journal Compilation © 2009 International Society of Neuropathology
 Correspondence

Figure 5. Figure 6.

Figure 7. Figure 8.

Figure 9. Figure 10.

Brain Pathology 19 (2009) 337–340 339

© 2009 The Authors; Journal Compilation © 2009 International Society of Neuropathology
Correspondence
FINAL DIAGNOSIS
Astroblastoma
DISCUSSION
For a more complete discussion and differential diagnosis and
additional references please visit: http://path.upmc.edu/divisions/
neuropath/bpath/cases/case173.html.
According to Bonnin and Rubinstein, astroblastomas are defined
histologically by the presence of astroblastic pseudorosettes and
prominent perivascular hyalinization (2). However, pseudorosettes
may be observed in other tumors (4). We agree that designation of
astroblastoma should be based on a constellation of clinical, radio-
logical and histological findings as illustrated in the current case.
Astroblastomas show an unusual organization of two cell types:
more primitive cells appear nesting within the cytoplasm of the
differentiated (“nurse”) cells (7). Both types of cells are nicely
illustrated in this tumor. To the best of our knowledge, this is the
second published case on astroblastoma with rhabdoid morphology
in the English literature (1).
A long differential diagnosis should be considered once rhab-
doid cells are encountered in a CNS tumor. These tumor cells are
typically observed in atypical teratoid/rhabdoid tumor (AT/RT)
which are rare in adults and show high proliferation (5). The pres-
ervation of INI1 protein expression in the current tumor speaks
strongly against the diagnosis of AT/RT. Another important differ-
ential diagnosis is ependymoma, especially the unusual type giant
cell ependymoma. However, the foot processes in the perivascular
pseudorosette of ependymoma should be long and indistinct rather
than short and broad as seen in this case. Finally, ultrastructural
ependymal differentiation as characterized by cilia, intracytoplas-
mic mircolumina and long zonular adherens are not identified in
our case. Other possible diagnoses include glioma, melanoma,
rhabdomyosarcoma and metastatic carcinoma, and meningioma.
Apart from the unusual rhabdoid appearance in the tumor cells,
all the histopathological features typical for astroblastoma are
present in this case. These features include pseudopapillary
arrangement, astroblastic pseudorosettes, perivascular hyaliniza-
tion and calcifications, absence of fibrillary background and a
pushing tumor border. This diagnosis is also well supported by the
age of presentation, anatomical location and radiological features
of the tumor. Survival for patients with astroblastoma is variable
and depends of multiple factors including completeness of exci-
sion, histological grade and radiosensitivity (2, 3, 6). Astroblas-
toma appears to be radiosensitive but no definite chemotherapy is
available.
ACKNOWLEDGEMENT
We would like to thank Dr. Colin Smith, Senior Lecturer in Pathol-
ogy (Neuropathology) of University of Edinburgh for his expert
opinion in this case.
340
© 2009 The Authors; Journal Compilation © 2009 International Society of Neuropathology
REFERENCES
1. Bannykh SI, Fan X, Black KL (2007) Malignant astroblastoma with
rhabdoid morphology. J Neurooncol 83:277–278.
2. Bonnin JM, Rubinstein LJ (1989) Astroblastomas: a pathological study
of 23 tumors, with a postoperative follow-up in 13 patients.
Neurosurgery 25:6–13.
3. Brat DJ, Hirose Y, Cohen KJ, Feuerstein BG, Burger PC (2000)
Astroblastoma: Clinicopathologic features and chromosomal
abnormalities defined by Comparative Genomic Hybridization. Brain
Pathology 10:342–352.
4. Burger PC, Scheithauer BW (1994) Tumors of the central nervous
system. Atlas of tumor pathology, 3rd series, fascicle 10. Washington,
DC: Armed Forces Institute of Pathology.
5. Ho DM, Hsu CY, Wong TT, Ting LT, Chiang H (2000) Atypical
teratoid/rhabdoid tumor of the central nervous system: a comparative
study with primitive neuroectodermal tumor/medulloblastoma. Acta
Neuropathol 99:482–488.
6. Lau PP, Thomas TM, Lui PC, Khin AT (2006) “Low-grade”
astroblastoma with rapid recurrence: a case report. Pathology
38:78–80.
7. Mierau GW, Tyson RW, McGavran L, Parker NB, Partington MD
(1999) Astroblastoma: Ultrastructural observations on a case of
high-grade type. Ultrastruct Pathol 23:325–332.
Contributed by:
Yuen Shan Fan, FRCPA Philip C.W. Lui*,
FRCPA Fiona K.Y. Tam, MBBS Kwan Ngai Hung†,
FRCS(Ed) Ho Keung Ng*, FRCPath
Suet Yi Leung, FRCPath
Department of Pathology, Queen Mary Hospital, The University
of Hong Kong; * Department of Anatomical and Cellular
Pathology, Prince of Wales Hospital, The Chinese University of
Hong Kong; † Department of Neurosurgery, Queen Mary
Hospital, Hong Kong
ABSTRACT
Rhabdoid tumor cells are typically observed in atypical teratoid/
rhabdoid tumor (AT/RT) but may also be seen in meningioma,
glioma, melanoma, rhabdomyosarcoma and metastatic carcinoma.
We present an astroblastoma with unusual rhabdoid features which
is rarely described in the English literature. Apart from the rhab-
doid tumor cells, all the histopathological features typical for astro-
blastoma are present in this case. These features include pseudo-
papillary arrangement, astroblastic pseudorosettes, perivascular
hyalinization and calcifications, absence of fibrillary background
and a pushing tumor border. The tumor cells display a multilineage
immunohistochemical profile. In addition, diffuse and strong
membranous and cytoplasmic dot-like pattern is appreciated with
epithelial membrane antigen (EMA). The diagnosis of astroblas-
toma is also well supported by the age of presentation, anatomical
location and radiological features of the tumor. We believe that on
top of the above-mentioned unusual tumors with rhabdoid cells,
astroblastoma should also be considered in the list of differential
diagnosis.
Brain Pathology 19 (2009) 337–340