Acta Neuropathol (2005) 110: 6976

DOI 10.1007/s00401-005-1015-7

CASE REPORT

Atsushi Sasaki Hideyuki Kurihara Shogo Ishiuchi

Junko Hirato Nobuhito Saito Yoichi Nakazato

Pediatric embryonal tumor of the cerebellum with rhabdoid cells

and novel intracytoplasmic inclusions: distinction from atypical
teratoid/rhabdoid tumor
Received: 13 December 2004 / Revised: 7 February 2005 / Accepted: 14 February 2005 / Published online: 17 June 2005

Springer-Verlag 2005

Abstract We report a case of embryonal tumor with

novel inclusion bodies occurring in the cerebellum of a
12-year-old girl. The tumor was histopathologically
composed of small undierentiated cells intermingled
with a small number of rhabdoid cells, which had an
ultrastructural feature of intermediate lament whorls.
Immunohistochemically, the neoplasm showed a polyphenotype, including glial brillary acidic protein
(GFAP), S-100, synaptophysin, chromogranin A, cytokeratin, vimentin, smooth muscle actin, and desmin.
However, epithelial membrane antigen (EMA) immunoreactivity was absent. The MIB-1 labeling index was
high (25.6%). Ultrastructurally, there was no evidence
of neuronal or myogenic dierentiation. The small
neoplastic cells contained numerous small intracytoplasmic inclusions stained pink by eosin and red by
Massons trichrome stain. The inclusion body was a
densely packed, granulovesicular structure at the electron microscopic level, and was immunoreactive for vimentin, GFAP, desmin, and actin. Reverse
transcription-PCR and immunohistochemistry showed
the expression of INI1 at the RNA and protein levels,
respectively. In conclusion, this tumor was dierentiated
from atypical teratoid/rhabdoid tumor by the absence of
EMA and the presence of INI1 mRNA and protein, and
diagnosed as an unclassied, embryonal tumor. EosinA. Sasaki (&) J. Hirato Y. Nakazato
Department of Human Pathology,
Gunma University Graduate School of Medicine,
Maebashi, 371-8511 Gunma, Japan
E-mail: achie@med.gunma-u.ac.jp
Tel.: +81-27-2207971
Fax: +81-27-2207978
S. Ishiuchi N. Saito
Department of Neurosurgery,
Gunma University Graduate School of Medicine,
Maebashi, Gunma, Japan
H. Kurihara
Department of Neurosurgery,
Kiryu Kousei Hospital, Kiryu, Gunma, Japan

ophilic, granulovesicular inclusions of the tumor cells

are novel cytoplasmic inclusions in the brain tumor.
Keywords Atypical teratoid/rhabdoid tumor
Immunohistochemistry INI1 Rhabdoid cells
Inclusion body

Introduction
Brain tumors make up 22% of all malignancies in children of up to 14 years of age, and are the leading cause
of death from childhood cancer. The updated 2000
WHO Classication of Tumors of the Nervous System
has for the rst time linked the standard histological
features of the neoplasms with the available molecular
genetic data. However, there are many pediatric tumors
that do not t into the present pathological classication, and these unclassied tumors should be collected
and eventually categorized according to histology,
genetics, and behavior.
Atypical teratoid/rhabdoid tumor (AT/RT) was initially described in 1987 [13] and has been dened in the
updated 2000 WHO classication as a highly aggressive
malignant tumor in children that is composed of
rhabdoid cells, primitive neuroectodermal, epithelial and
mesenchymal components in various cell populations
[20]. The histological and immunophenotypic spectrum
of these neoplasms has been reviewed [1, 5, 7, 16, 19, 20].
However, some cases presented diculties in pathological diagnosis of AT/RT for the following reasons: (1)
typical rhabdoid cells are seen in a minority of AT/RT
cases; (2) the cell population of rhabdoid cells varies
among cases; (3) immunoreactivity of tumor cells varies
except for the almost consistent expression of vimentin
and epithelial membrane antigen (EMA) by rhabdoid
cells; and (4) rhabdoid cells are present in various types
of malignant neoplasms. To date, the histogenesis of AT/
RT is unknown. Molecular testing for the hSNF5/INI1
gene at the DNA, RNA, or protein level now serves as a

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critical and denitive diagnostic adjuvant [2, 9, 21]. The

prognosis for AT/RT is much poorer than that of other
pediatric brain tumors, including primitive neuroectodermal tumor/medulloblastoma (PNET/MB), since most
of patients with AT/RT die within a year after diagnosis.
Thus, dierential diagnosis of AT/RT from other pediatric brain tumors is of clinical signicance [5, 20].
In this report, we present a case of cerebellar
embryonal tumor in a 12-year-old girl mimicking AT/
RT. The tumor could be dierentiated from AT/RT by
performing careful clinicopathological and molecular
studies, including INI1 expression at RNA and protein
levels, although the tumor remains an unclassied
embryonal tumor in the present classication. We also
report histological and ultrastructural features of
numerous intracytoplasmic inclusions in the neoplastic
cells, which have not been described previously.

Case report
A 12-year-old girl was admitted to the hospital due to
progressive headache, vomiting, and disturbance of consciousness that developed over 2 weeks. Magnetic resonance imaging (MRI) revealed a large mass lesion,
approximately 554 cm in size, nonhomogeneously enhanced with gadolinium in the right posterior fossa
(Fig. 1). MRI showed no evidence of tumor outside the
brain. The patient underwent subtotal removal of the
tumor at the Gunma University Hospital. At the time of
operation, the tumor was originated in the cerebellar
hemisphere, and the tumor margin was unclear. After
resection, the patient received irradiation (30 Gy whole
brain, 30 Gy local brain, 30 Gy whole spinal cord) and

chemotherapy (cisplatin and etoposide), but 19 months

later the tumor recurred. She underwent gamma-knife
treatment and chemotherapy (vincristine, actinomycinD, cyclophosphamide, intrathecal methotrexate). At the
age of 15 and 17 years, MRI demonstrated the second and
third recurrence of the tumor, and the tumor resection was
performed. At 5 years 7 months after the initial surgery
(9 months after the last resection), the patient is still alive.

Materials and methods

Histological and immunohistological studies
Formalin-xed, paran-embedded sections of the
tumors extracted in the initial operation and in the
subsequent four resections were subjected to hematoxylin-eosin (HE), periodic acid-Schi (PAS), and Massons
trichrome staining. The paran sections of the tumor
were examined using a panel of antibodies by immunoperoxidase methods. Sources and dilutions of the antibodies used were as follows: vimentin (DAKO, Glostrup,
Denmark, 1:200), EMA (DAKO, 1:100), a-smooth
muscle actin (SMA, BioMakor, Rehovot, Israel, 1:3,200),
glial brillary acidic protein (GFAP, our own, 1:10,000),
keratin (AE1/AE3, Boehringer-Mannheim, Mannheim,
Germany, 1:500), S-100 protein (our own 1:20,000),
neurolament protein 160 kDa (NFP, our own, 1:1,000),
synaptophysin (SY38, DAKO, 1:10), chromogranin A
(CGA, DAKO, 1:1,000), neuron-specic enolase (NSE,
DAKO, 1:200), desmin (D33, DAKO, 1:200), musclespecic actin (HHF-35, DAKO, 1:400), myoglobin
(DAKO, 1:5,000), a-fetoprotein (AFP, DAKO, 1:2,000),
leukocyte common antigen (LCA, DAKO, 1:50), melanoma antigen (HMB45, DAKO, 1:50), MIB-1 (Immunotech, Marseille, France, 1:50), INI1 (BAF47/SNF5,
BD Transduction Labs, San Diego, CA, 1:100), and p53
(DO7, DAKO, 1:50). Prior to incubation with some
antibodies, pretreatment was performed as follows: (1)
autoclaving (121C) for 10 min (MIB-1), and (2) microwave (90C) for 10 min (p53, INI1). The labeling index
(LI) of MIB-1 was dened as the percentage of immunostained cells divided by the total number of neoplastic
cells in the area of maximal labeling.
Ultrastructural study
The biopsy specimens of the initial operation were xed
in 1% buered glutaraldehyde, postxed in 2% osmium
tetroxide, routinely processed, and embedded in Quetol
812. Ultrathin sections were stained with uranyl acetate
and lead citrate, and observed under a JEM transmission electron microscope.

Fig. 1 Preoperative MRI. Axial plane of gadolinium-enhanced T1weighted image. The MRI reveals a 554-cm heterogeneously
enhancing lesion in the right posterior fossa (MRI magnetic
resonance imaging)

Molecular study for the INI1 mRNA expression

Reverse transcription (RT)-PCR was performed on this
case and two control tumors (glioblastoma and

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medulloblastoma) according to established methods

using two pairs of primers to amplify the INI1 cDNA
[2]. RT-PCR of the same cDNA samples for G3PDH
was also examined for comparison.

Results
Light microscopic ndings
Histologically, the neoplasm of the initial operation was
highly cellular and composed of solid and noncohesive
sheets of small to medium-sized cells with hyperchromatic oval-to-round nuclei and scant cytoplasm
(Fig. 2AC). In some areas the tumor cells were intermixed with embracing cells. Spindle-shaped cells arranged in short fascicular pattern were seen as a small
population in the tumor. Specic structures such as
ependymal canals or neural-tube-like structures or rosette formation were absent. No epithelial dierentiation
such as gland-like/papillary structure and squamous
epithelium was observed. Both mitotic gures (56 per 10
high-power elds) and apoptotic bodies were abundant.
A small area of necrosis was present, but pseudopalisading necrosis was not found. At higher magnication,
numerous tumor cells showed small intracytoplasmic
inclusion bodies, which were irregularly shaped and
stained pink by eosin and red by Massons trichrome
stain (Fig. 2D, F), but did not react with PAS. In addition, a small number of rhabdoid cells with eccentric
nuclei and eosinophilic cytoplasm were seen (Fig. 2E).
The large inclusion of rhabdoid cells was light blue on
Massons trichrome stain (Fig. 2F). The histology of the
tumor cells at the recurrences was similar to that of the
small undierentiated cells observed at the initial operation material, although neither rhabdoid cells nor
eosinophilic inclusions were identied in the fourth and
fth resections.
Immunohistochemistry
Immunohistochemistry yielded a polyphenotypic pattern
of expression: the tumor cells were positive for NSE
(Fig. 3A), CGA, synaptophysin, GFAP (Fig. 3B), S-100,
vimentin (Fig. 3C), SMA (Fig. 3D), muscle-specic actin, desmin (Fig. 3E), and keratin (Fig. 3F) in various
quantities. The positivity of SMA and muscle-specic
actin was reminiscent of subsarcolemmal staining. The
neoplastic cells were non-reactive for EMA (Fig. 3G),
NFP, AFP, LCA, HMB-45, Mic2, myoglobin, MyoD1,
and p53. Staining with the INI1 antibody showed nuclear
staining in tumor cells (Fig. 3H). Numerous intracytoplasmic inclusion bodies were stained with antibodies
against vimentin, GFAP, desmin, SMA, and musclespecic actin. The rhabdoid cells showed paranuclear,
globular positivity for vimentin, keratin, and desmin.
The MIB-1 LI was 25.6% at the initial resection, and
ranged from 18% to 37% at the recurrent resections.

Ultrastructure
In typical rhabdoid cells concentrically arranged intermediate lament bundles were formed in the paranuclear cytoplasm (Fig. 4A). Most of the tumor cells were
relatively small with irregular, deeply indented nuclei. In
their cytoplasm, small, oval-shaped intracytoplasmic
inclusion bodies with granulovesicular, faintly lamentous materials were often identied (Fig. 4B). The tumor
cells lacked muscle cell or neuronal dierentiation.
RT-PCR
Using RT-PCR, expression of the 5 (exon 16) and 3
(exon 59) portions of the INI1 cDNA was observed for
this case and for two control tumors (glioblastoma and
medulloblastoma) (Fig. 5).

Discussion
We reported the clinical, histological, immunophenotypical, ultrastructural, and molecular (INI1) ndings
for an unusual tumor occurring in the cerebellum of a
12-year-old girl. The tumor corresponded pathologically
to a poorly dierentiated/undierentiated neoplasm
with rhabdoid cells and immunohistochemical polyphenotype, and mimicked AT/RT. A rhabdoid phenotype
is identied not only in AT/RT, but also in other brain
tumors, such as rhabdoid meningioma, rhabdoid glioblastoma, medullomyoblastoma, and rhabdomyosarcoma [12, 17, 24]. The histology and the presence of the
immunohistochemical polyphenotype eliminated tumors
such as classic medulloblastoma, large cell medulloblastoma, PNET, glioblastoma, and meningioma. In
terms of the presence of rhabdoid cells and desmin
reactivity, our tumor resembled medullomyoblastoma or
rhabdomyosarcoma. However, this case diered from
those tumors as our tumor was negative for other muscle
markers (myoglobin, MyoD1) and showed no well-dened striated muscle. Extensive immunohistochemical
studies and molecular study of the INI1 gene product
were required for the dierential diagnosis of AT/RT.
The presence of rhabdoid cells is the essential and
sole feature in common with AT/RT. Electron microscopic analysis could be presented as additional support
for AT/RT by demonstrating rhabdoid cells with intermediate lament whorls. Histologically, AT/RT is
mitotically active and usually accompanied by eld
necrosis. Ho et al. [7] pointed out that identication of
embracing cells could be helpful in the diagnosis of AT/
RT. Immunohistochemically, AT/RT shows striking
polyphenotypic immunoreactivity, including glial, neuronal, epithelial, and mesenchymal markers. The present
case contained all of these histological, immunophenotypical, and ultrastructural features of AT/RT.
However, this case was unusual in some clinicopathological ndings. In particular, two aspects are

72

Fig. 2 Histological features. A The highly cellular, solid tumor

with numerous mitotic gures and apoptotic bodies is traversed by
brovascular septa. B In the noncohesive area, embracing cells
(arrows) are often observed. C Pale nuclei and clear cytoplasm
show a spongy appearance. DF Higher magnication showed
numerous intracytoplasmic inclusion bodies, which are irregular in

size and shape, stained pink by HE (D), and red by Massons

trichrome stain (F, arrows). A small number of globular inclusion
bodies in rhabdoid cells are pink and pale blue on HE (E) and
Massons trichrome stainings (F, asterisk), respectively (HE
hematoxylin and eosin). AE HE, F Massons trichrome; original
magnications A, C 100; B 200; DF 400

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Fig. 3 Immunohistochemistry demonstrates positivity for neuronspecic enolase (A), glial brillary acidic protein (B), vimentin (C),
SMA (D), desmin (E), and cytokeratin (F), but not for epithelial
membrane antigen (G). Small intracytoplasmin inclusions are

stained with vimentin, SMA (arrows in D), and desmin. Rhabdoid

cells with globular cytoplasmic inclusions contain vimentin (inset in
C) and cytokeratin. Nuclear staining with INI1 is observed (H)
(SMA a-smooth muscle actin). Original magnication AH 200

74
Fig. 4 Ultrastructural features.
A The typical rhabdoid cell
possesses a concentric
arrangement of intermediate
laments with small amounts of
entrapped cytoplasmic
organelles. B Many tumor cells
contain small, oval-shaped
intracytoplasmic inclusion
bodies, which are composed of
densely packed,
granulovesicular, vaguely
lamentous materials (n nuclei).
Bars A 2 lm (i nset 200 nm),
B 1 lm (inset 200 nm)

Fig. 5 INI1 gene expression

demonstrated using RT-PCR in
the present tumor and two
control tumors (glioblastoma
and medulloblastoma). RTPCR of the 5 region and 3
region of the gene amplies a
product in the three tumors.
RT-PCR of the same cDNA
samples for G3PDH is shown
for comparison

75

important: (1) the patient was in the second decade at

diagnosis and alive at the time of this case report,
5.5 years after the initial surgery, and (2) the tumors
were negative for EMA and strikingly positive for desmin with globular staining. Of the 188 AT/RT cases
observed to date, 94% were 5 years of age or less at
diagnosis, and four adult cases have been reported (21
41 years) [18]. A study of 133 AT/RT cases showed that
98 cases (74%) died of local and/or disseminated disease
within 24 months [16]. Some previous studies indicated
that EMA is always expressed [1, 5, 20], although a few
cases that were negative for EMA have been reported [7,
15]. In addition, desmin expression in AT/RT is exceptionally sparse even when present, and is typically conned to the mesenchymal and PNET elds, not in
rhabdoid cells.
The INI1 gene on 22q11.2 has been implicated as a
tumor suppressor gene in AT/RT in the CNS as well as
in rhabdoid tumors in other organs [2, 21, 23]. Homozygous inactivation of INI1 by deletion and/or mutation
in the INI1 gene was demonstrated in most rhabdoid
tumors and AT/RT by cytogenetic and molecular analyses, such as uorescence in situ hybridization and PCRbased sequences [2, 4, 18]. A previous study [2] showed
that among 29 AT/RT, renal, and extrarenal rhabdoid
tumors, 14 tumors had demonstrated mutations, and the
other 15 tumors had homozygous deletions of one or
more exons of the INI1 gene, in which the gene
expression was absent by RT-PCR. Recently, Judkins
et al. [9] showed that immunohistochemistry with an
INI1 antibody correlated with molecular ndings in AT/
RT and might be useful in distinguishing AT/RT from
other tumors with indeterminate histological features or
atypical immunophenotypes. Based on the results of
RT-PCR and immunohistochemistry, it appears that
alterations in INI1 at the RNA and protein levels were
absent in the present tumor. In a previous study of
problematic AT/RT tumors with weak or absent EMA
expression, immunohistochemical staining for INI1
showed nuclear expression in all the tumors, suggesting
that these were not AT/RT [9]. On the basis of clinical,
histological, immunohistochemical, and RT-PCR ndings, this case could be distinguished from AT/RT and
diagnosed as a primitive tumor of unknown histogenesis, dicult to classify in the current classication.
The present tumor contained two dierent types of
intracytoplasmic eosinophilic inclusions: inclusions of
rhabdoid cells and inclusions of undierentiated cells.
The microscopic, immunophenotypic, and ultrastructural ndings of the former inclusion were compatible
with those seen in AT/RT and malignant rhabdoid tumor of the kidney or soft tissues. On routine HE stain,
both inclusions were eosinophilic, but the latter were
more irregular in shape and size. The results of Massons
trichrome stain and electron microscopy were clearly
dierent (light blue vs red, and whorls of intermediate
laments vs granulovesicular materials). To date, the
latter inclusion has not been reported in the literature of
brain tumors, including AT/RT.

Several types of intracytoplasmic eosinophilic inclusions have been reported in ependymoma, meningioma,
and primary intracerebral leiomyoma [8, 10, 11, 14, 22].
In cerebellar medulloblastoma (neuroblastic medulloblastoma), paranuclear aggregates of actin-type microlaments have been observed [3]. Ultrastructurally,
however, all of them lacked the granular materials seen
in our case, and most of them were negative for desmin.
At microscopic and ultrastructural levels, our novel
cytoplasmic inclusions are similar to intracytoplasmic
hyaline globules, which have been observed in carcinoma of various organs (lung, breast, liver) [6]. However, unlike our inclusions, the hyaline globules are PAS
positive. The inclusions in the present case might have
been formed as a part of tumorigenesis, and not as a
reactive process of therapeutic interventions, since they
were present in the initial resection specimen. The
inclusions were immunohistochemically positive for
antibodies against three types of intermediate lament
proteins (vimentin, desmin, GFAP) and actin, and vaguely lamentous at electron microscopic level. Thus,
our inclusion bodies might be products from non-polymerized cytoskeleton proteins. Our inclusions shared
immunohistochemical positivity of vimentin and desmin
with inclusions of rhabdoid cells, and both inclusions
disappeared in the third recurrence. However, either the
relationship between the two inclusions or the mechanism for their formation remains uncertain.
In conclusion, we report a case of cerebellar embryonal tumor in a 12-year-old girl mimicking AT/RT. The
tumor could be dierentiated from AT/RT by the absence of EMA and the presence of INI1 mRNA and
protein. Thus, immunohistochemical staining with EMA
and INI1 antibodies may be useful in cases where the
diagnosis of AT/RT is considered, but equivocal clinicopathological ndings are encountered. Eosinophilic
intracytoplasmic inclusions, containing densely packed
granulovesicular materials at the ultrastructural level,
were characteristic features in this unclassied tumor.
These inclusions should be noted in the pathological
analysis of brain tumors, particularly pediatric primitive
tumors. Additional cases with similar clinicopathological ndings will be needed to dene the spectrum of the
present tumor.
Acknowledgements The authors thank Machiko Yokota and Kohji
Isoda for technical support. This work was supported in part by a
Grant-in-Aid for Scientic Research (B) (no. 15300113) from the
Japanese Ministry of Education, Culture, Sports, Science, and
Technology (to Y.N.).

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