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2014, Volume 30, Number 1, Page(s)
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DOI: 10.5146/tjpath.2013.01213 |
Atypical Teratoid Rhabdoid Tumor in Childhood, 15 Cases of a Single Institute Experience |
Maysa AL-HUSSAINI1, Noreen DISSI1, Usama AL-JUMAILY2, Maisa SWAIDAN3 |
1Departments of Pathology and Laboratory Medicine, King Hussein Cancer Center, AMMAN, JORDAN 2Departments of Pediatric Oncology, King Hussein Cancer Center, AMMAN, JORDAN 3Departments of Radiology, King Hussein Cancer Center, AMMAN, JORDAN |
Keywords: Central nervous system neoplasms, Medulloblastoma, Immunohistochemistry |
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Objective: Atypical teratoid rhabdoid tumor is a rare neoplasm with
a distinct cytogenetic profile that predominates in infancy. Many
cases show predominance of small cells with scanty rhabdoid cells,
making recognition of this tumor difficult. We aim at describing our
experience with atypical teratoid rhabdoid tumor cases diagnosed
over a 6-year period.
Material and Method: Clinicopathologic features and immunohistochemical
staining of atypical teratoid rhabdoid tumor cases diagnosed
between 2006 and 2011 are presented.
Results: Fifteen cases were identified including 9 males with a
median age of 26 months. The most common presenting symptom
was recurrent vomiting with a mean duration of 6 weeks. Nine cases
(60%) were infratentorial and cerebrospinal fluid was positive in 2
cases (13.3%) at time of diagnosis. The median overall survival of
the group was 9.5 months. All cases except one showed admixture of
rhabdoid and/or small round blue cells in variable proportions. Only
5 out of fourteen referred cases (35.7%) were correctly diagnosed.
Three cases showed unusual growth patterns. In 2 cases, nodular
medulloblastoma-like growth pattern predominated, with loss of
INI-1/BAF47 staining both within the nodules and the inter-nodular
areas. The third case contained scattered individual and small groups
of large cells with abundant acidophilic cytoplasm and eccentric
nuclei, reminiscent of rhabdomyoblasts that were positive for
GFAP and desmin, and retained nuclear staining for INI-1/BAF47,
consistent with reactive gemistocytes.
Conclusion: Pathologists should be aware of the various, and unusual
histopathologic patterns of atypical teratoid rhabdoid tumor. INI-1/
BAF47 immunostain should be performed on all central nervous
system embryonal tumors, especially in infants and young children. |
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Atypical teratoid rhabdoid tumor (AT/RT) is a rare highly
aggressive neoplasm of uncertain origin. It accounts
for 1-2% of all brain tumors, and at least 10% of central
nervous system (CNS) tumors in infants, with a slight
male predominance 1. It occurs in supratentorial and
infratentorial locations, but predominates infratentorially 2. Occasional examples can be multifocal 2 or originate
within the ventricles 3. It was first described in 1987 as
a tumor with a distinctive morphology characterized by
proliferation of monomorphic cells with rhabdoid features
hence was called malignant rhabdoid tumor of the central
nervous system 4. Epithelial and mesenchymal elements
were later recognized along with the frequent presence of
the rhabdoid component, so the name was changed into
“Atypical teratoid rhabdoid tumor” 5. The presence of
a small cell component with the potential confusion with
medulloblastoma and primitive neuroectodermal tumor
(PNET ) was appreciated later in several studies 2,6.
Eventually, in 1996 it was defined as a separate entity and
was recognized in the 2000 WHO classification 7.
In 1999 AT/RT was linked to a highly specific genetic
alteration i.e. the inactivation of hSNF5/INI-1 gene
located on chromosome 228. The introduction of an
immunohistochemical antibody few years later that targets
this mutation has resulted in the increasing recognition
and reporting of this tumor9. Recently however, Tsai et
al. demonstrated that not all tumors with absence of INI-
1protein on immunostaining carry a mutation in the INI-1
gene, pointing towards other possible posttranscriptional
regulatory mechanisms for INI-1 protein synthesis10.
AT/RT cases showing SMARCA4/BRG1 mutation have
retained INI-1 expression11.
The overall prognosis of AT/RT is dismal, and the reported
median survival is less than one year2. Separation of
this tumor from mimickers including medulloblastoma
and CNS-PNET is important due to the apparent poorer
prognosis12.
This study aims at summarizing our experience with cases
of AT/RT diagnosed at the center over a 6-year period.
Description of the clinical and pathological features is
provided with emphasis on unique morphological patterns. |
Top
Abstract
Introduction
Methods
Results
Disscussion
References
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After obtaining the approval from the local institutional
review board/research ethics committee, a retrospective
search in the pathology files for cases diagnosed as AT/
RT over a six-year period (2006-2011) was performed. Clinical data collected included age at presentation, gender,
nationality, presenting symptoms and their duration, tumor
location, radiological findings, presence or absence of
cranio-spinal metastasis at time of diagnosis, modalities of
treatment offered including surgery, chemotherapy (CTH)
and radiotherapy (RTH), and date of last follow up. The
outcome of patients was determined as dead versus alive at
the last available follow up.
Pathologic material available from all cases was
reviewed including hematoxylin-eosin (H&E) slides,
immunohistochemical stains, and cerebrospinal fluid
slides. Pathologic features assessed included the growth
patterns (diffuse, reticular, nodular, papillary), the types
of cells present including rhabdoid cells (dyscohesive cells
with vesicular nuclei, prominent nucleoli, and spherical
cytoplasmic filamentous inclusions), pale cells (cells
with vesicular nuclei, prominent nucleoli, and granular
vacuolated wispy or water clear cytoplasm lacking intracytoplasmic
inclusions), and small cells (small round blue
cells with high nuclear to cytoplasmic ratio reminiscent of
cells in medulloblastoma/PNET ). The proportion of cell
types was then assessed semi-quantitatively as focal or
diffuse if occupying <50% or ≥50% of the area of the slides
examined; respectively. Moreover, the existence and the
type of mesenchymal (myxoid, cartilaginous, lipomatous,
osteoid, etc.) and epithelial (glandular, squamous, etc.)
differentiation were determined. Finally necrosis and
calcifications were also evaluated as present or absent. All
immunohistochemical stains used are listed in Table I.
Positive control was run for each antibody. Diffuse positive
staining was defined as reactivity in ≥50% of tumor cells
and otherwise the stain was considered focal.
All data were entered into an Excel sheet. Descriptive
statistics including counts and percentages as well as
medians and ranges were calculated for variables as
applicable. |
Top
Abstract
Introduction
Methods
Results
Disscussion
References
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A total of fifteen cases were identified, 9 males and 6 females,
with a male to female ratio of 1.5:1. The median age at
diagnosis was 26 months (mean 28 months and range 6-61
months), with 7 (46.7%) cases younger than 24 months. Nine
(64%) cases were Jordanian. The clinical symptoms were
related to tumor location. The most common presenting
symptoms were recurrent vomiting (n=11; 73.3%), squint
(n=4; 26.6%), unsteady gait (n=3; 20%), and 6th nerve
palsy (n=2; 13.3%). The median duration of the symptoms
was 6 weeks. The tumor was incidentally discovered in a
single patient after a head trauma accident. One patient
was the result of an in- vitro fertilization (IVF) pregnancy. Abdominal ultrasound was negative for renal masses in the
patients investigated.
Nine (60%) cases were located infratentorially and six (40%)
were supratentorial. In two cases the tumors were intraventricular
occupying the 4th and left lateral ventricles.
Initial magnetic resonance imaging (MRI) and/or computerized
tomography (CT) scans were available for review in
fourteen cases. In all cases the tumors were large, lobulated
and heterogeneous. They showed contrast enhancement
(n=10; 71.4%), a partially cystic appearance (n=9; 64.3%),
mild edema (n=6; 46.8%), and calcifications (n=4; 28.5%).
Cranio-spinal metastases were present at the time of diagnosis
in 2 (14.3%) cases and developed later during the
course of the disease in an additional 5 (35.7%).
In the cases with available data on the extent of surgical
resection, gross total resection (GTR) was performed in 5
(38.5%), subtotal resection (STR) in 3 (23.0%), and partial
resection (PR) in 5 (38.5%). The extent of resection was not
documented in two (13.3%) cases. Chemotherapy (CTH)
was offered to 10 (66.6%) cases, including four who also
received radiotherapy (RTH), while RTH alone was offered
for a single (7.1%) case. No documented further treatment
was offered to the 4 remaining cases.
At time of data collection, eleven of twelve (91.7%) cases
with available follow up data were dead of local and/ or disseminated disease. One case was still alive without
evidence of disease 86 months following diagnosis. The
median progression free survival and overall survival
were less than a year being 7.75 months and 9.5 months,
respectively.
Table II summarizes the patients’ demographics, clinical
findings and outcome.
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Table II: Patient’s demographics, clinical and radiological features and outcome |
Pathologic and Immunohistochemical Findings:
Table III summarizes the morphological and immunohistochemical
characteristics of tumors.
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Table III: Morphological and immunohistochemical characteristics of tumors |
Growth pattern was predominantly diffuse in nine cases
(60%) with nodular, reticular and papillary patterns noted
in 2 cases each (13.3%). Common cellular components
included rhabdoid cells (n=12; 80.0%) (Figure 1A), large pale
cells (n=12; 80.0%) and small round blue cells (n=13; 86.6%)
(Figure 1B). Admixture of all the three cell components was
noted in most cases (n=9; 60%). None of the fifteen cases
examined was composed exclusively of rhabdoid cells. On
the contrary small cells were the only cells identified in a
single case and were diffusely identified in 2 (13.3%) other
cases. Variable mesenchymal components were recognized
focally including myxoid/fibromyxoid changes in six cases,
while chondroid (Figure 1C), lipoblastic (Figure 1D) and
spindle cells elements were noted in a single case each. No
glandular component was seen in any of the cases, although papillary structures were seen in two (13.3%) (Figure 1E).
Necrosis was evident in all (100%) cases, while dystrophic
calcifications were noted in three (20%).
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Figure 1: A) Rhabdoid dyscohesive cells with vesicular nuclei, prominent nucleoli, and spherical cytoplasmic filamentous inclusions
(H&E, x200). B) Small hyperchromatic cells similar to PNET /medulloblastoma showing dense nuclei, dispersed chromatin, small to
inconspicuous nucleoli, with scant cytoplasm and indistinct cell borders, arranged in a solid pattern (H&E x200). C) Areas of chondroid
differentiation arranged in a reticular and cord like patterns (H&E x200). D) Lipoblastic-like cells with clear vacuolated cytoplasm and
eccentric nuclei (H&E x200). E) AT/RT displaying an epithelial component with a papillary architecture (H&E x200). F) CSF smear
displaying a single tumor aggregate with rhabdoid features (Diff Quick stain x200). |
Cerebrospinal fluid analysis was performed at presentation
in ten out of fifteen cases. Malignant cells were identified
in 2 (20%), both with positive leptomeningeal seeding by
MRI studies. Cytologically, the smears showed clusters and
individual large cells with abundant eosinophilic cytoplasm
and eccentric nuclei (Figure 1F).
The diagnosis of AT/RT was confirmed in all cases
through demonstrating total loss of INI-1/BAF47 nuclear
immunostain in all kinds of tumor cells present in all cases with appropriate positive normal endothelial and
mononuclear cell control in the examined slides (Figure
2A). Where available rhabdoid and non- rhabdoid cells
showed variable positivity for EMA in twelve of thirteen
cases (Figure 2B), being diffusely positive in four (33.3%).
Synaptophysin displayed positivity in all ten cases
examined, being diffusely positivity in two (20%) (Figure
2C). Eight out of eight cases stained for glial fibrillary acidic
protein (GFAP), being diffuse in two (18.1%) (Figure 2D).
Vimentin was diffusely positive in five of five cases (100%).
Smooth muscle actin (SMA) was only focally positive in
three of four cases (75%) (Figure 2E) and pan-cytokeratin
was positive in six out of ten cases, being diffusely positive in three (50%) (Figure 2F). Desmin (n=4), CD99 (n=3), and
transthyretin (n=2), were all negative. The Ki67 labelling
index ranged between 30-50%.
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Figure 2: The variable immunostaining profile for AT/RT. A) INI-1/BAF47 immunostain is lost in tumor cells nuclei, but is retained
in the endothelial cells. B) EMA shows strong positivity in tumor cells. C) Synaptophysin is diffusely moderately to strongly positive in
tumor cells. D) GFAP shows focal strong positivity. E) Smooth muscle actin is diffusely weakly positive and F) pan-cytokeratin (MNF) is
diffusely strongly positive. (All stains are using 20 objective: x200). |
Cases with distinct morphological patterns
In two cases (cases number 7 and 8), a nodular
medulloblastoma-like growth pattern predominated. There
were pale nodules with distinct neuropil containing slightly
mature cells lacking mitosis (Figure 3A). The inter-nodular
areas showed a more compact proliferation of small round
blue cells. In both cases scattered rhabdoid cells were noted
after a more meticulous search. Synaptophysin (Figure
3B) was focally positive inside the nodules in both cases
while chromogranin (Figure 3C) was only focally weakly
positive inside the nodules in a single case. Reticulin stain was negative in both cases with no evidence of desmoplasia.
Focal positivity for epithelial membrane antigen (EMA)
(Figure 3D) and diffuse positivity for vimentin in the internodular
but not the intra-nodular areas (Figure 3E) was
noted in one of the cases. However the total absence of INI-
1/BAF47 immune-expression was seen in both the intra and
inter-nodular areas (Figure 3F) in both cases. Both cases
developed leptomeningeal metastases, and died of disease
progression 25 and 11 months after the initial diagnosis.
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Figure 3: AT/RT mimicking nodular medulloblastoma. A) Small undifferentiated cells with pale islands showing marked neuronal
differentiation (arrow, H&E x200). B) Synaptophysin is positive in areas of neuronal differentiation (Synaptophysin x200).
C)Chromogranin is weakly positive in the same areas (Chromogranin x200). D) EMA is focally positive (EMA x200). E) Vimentin is
negative in the differentiated pale areas and positive in the inter-nodular cellular areas (Vimentin x200). F) Loss of INI-1 nuclear staining
is visible in both inter- and intra-nodular areas with appropriate positive control of endothelial cells (INI-1/BAF47 x200), excluding the
possibility of entrapped normal nodular parenchyma. |
A single case (case number 6) harboured many single and
small aggregates of large cells with abundant acidophilic
cytoplasm that were closely related to the blood vessels
(Figure 4A). These cells were strongly positive for GFAP
(Figure 4B), vimentin and desmin (Figure 4C), but were negative for myogenin (not shown) and smooth muscle actin
(Figure 4D) ruling out the possibility of rhabdomyoblastic
and smooth muscle differentiation, respectively. Although
the tumor cells were focally positive for pan-cytokeratin
the large cells were negative (Figure 4E), and they retained
nuclear stain for INI-1/BAF47 (Figure 4F) against the
negative tumor cells. They were consistent with aggregates
of reactive gemistocytic astrocytes.
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Figure 4: Small aggregates of reactive gemistocytes in a case of AT/RT. A) Gemistocytic-like cells showing abundant glassy eosinophilic
cytoplasm, eccentric vesicular nuclei, and small prominent nucleoli, arranged predominantly around blood vessels (H&E x400).
B) GFAP is strongly positive in these cells (GFAP x200). C) Desmin is positive (Desmin x400), D) whereas smooth muscle actin is
negative (arrow points at one of the cells, SMA x400). E)They were negative for cytokeratin, although the surrounding tumor cells were
positive (arrows point at the cells, CK x200). F) INI-1/BAF47 nuclear stain is retained in these cells (arrow) confirming that they are not
an integral part of the tumor (INI-1/BAF47 x400). |
Referral cases
All cases were initially operated on and diagnosed outside
our center. The original diagnosis was not documented in a
single case. In five (35.7%) cases the diagnosis was consistent
with AT/RT and nine (64.3%) discordant cases were identified, as listed in Table II. The most frequent diagnosis
assigned by the original pathologists was medulloblastoma
(n=7; 77.7%) and anaplastic ependymoma (n=2; 22.2%).
Both cases with nodular medulloblastoma-like growth
pattern were misdiagnosed as medulloblastoma. INI-1/
BAF47 was not ordered on any of the discordant cases,
while it was performed on all concordant ones. Notably; the
percentage of discordant cases decreased after 2010. Four
out of the 7 (57.1%) referral cases were correctly diagnosed
as AT/RT since 2010, versus 1/7 (14.3%) cases diagnosed
before 2010.
Long term survival case
A single long-term survival was identified (case number 1). This patient presented initially at age of 23 months to
another institution with unsteady gait, vomiting and squint
of 16 weeks duration. Physical examination at diagnosis was
unremarkable. Brain imaging showed a lobulated, complex
partly solid partly cystic mass lesion involving the left
cerebellar hemisphere and the vermis. It measured about
3.8x4.3 cm and showed extension into the 4th ventricle. It also
extended through the left foramen of Luscka. He underwent
GTR, with ventriculo-peritoneal (VP) shunt insertion. The
referral diagnosis was medulloblastoma. At our institution,
the case was diagnosed as AT/RT on the basis of total loss
of INI-1/BAF47 immunostain in the tumor cells. Cerebrospinal
fluid (CSF) cytology, bone marrow examination and abdominal ultra-sound for renal tumors were all negative.
He was started on chemotherapy and received cranio-spinal
irradiation of 180grays/28 sessions. He is now 9 years and
5 months old with no evidence of disease 86 months after
diagnosis. |
Top
Abstract
Introduction
Methods
Results
Disscussion
References
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We have summarized our experience with AT/RT cases
diagnosed and managed at our center. The findings are at
large compatible with the international literatüre 13,14.
Our patients had a median age of 26 months at presentation,
a predominance of males with sixty percent occurring in
the posterior fossa. A single case was the product of IVF, (case number 7), who presented at 29 months of age, a
rarely reported association 15.
Radiologically findings are at large non-specific. The
tumors are usually non homogeneous secondary to the
heterogeneous cellular populations as well as the frequent
presence of necrosis and hemorrhage. Nine of our cases
contained a cystic component which is higher than the
occasional eccentric cyst formation described in some
reports16. In contrast to medulloblastoma (MB),
infratentorial AT/RT tend more often to be extra-axial
involving the cerebello-pontine (CP) angle, and to be
associated with intra-tumoral hemorrhage, which might
help in suggesting the diagnosis radiologically17. One of
our cases originated in the left CP angle and was one of the
five cases with concordant referral diagnosis. More recently;
the MR-based apparent diffusion coefficient histogram was
able to separate all cases of supratentorial PNET from AT/
RT cases18. Pure intra-ventricular examples included
2 cases in the left lateral and 4th ventricles, a rare but well
documented origin of AT/RT. In the original article by
Meyers S.P on 17 cases a single case of intraventricular
tumor involving the septum pellucidum was reported3,
while Donovan et al. reported a midline lateral ventricular
tumor which presented with intra-ventricular hemorrhage19. None of our intra-ventricular cases presented with
hemorrhage. A fourth ventricular origin of AT/RT has
been reported by Inenega C et al. in an infant with a dismal
outcome20. The outcome in both of our cases was
poor and did not differ from the rest of patients despite
of GTR of the 4th ventricular tumor. The intra-ventricular
origin of a small subset of AT/RT cases brings choroid
plexus carcinoma (CPC) into the differential diagnosis,
especially that both tumors share predominance in infancy
and young children. Immunostaining reveals epithelial
markers, especially cytokeratin and less frequently EMA,
to be positive in CPC. Other less frequent positive markers
include synaptophysin and GFAP. INI-1/BAF 47 has been
described in cases of choroid plexus carcinoma21f. This
has led to the speculation that AT/RT and CPC might be
related tumors especially that the origin of AT/RT is not
yet resolved. A finding that has been recently supported
by evidence of choroid plexus epithelial differentiation in
2 AT/RT cases exhibiting membranous staining by Kir7.122. However; we believe that our cases are AT/RT rather
than CPC due to the immature/PNET like nature of the
tumor cells, the presence of myxoid/mesenchymal features,
the absence of papillae, eosinophilic globules, as well as the
diffuse absence of INI-1/BAF 47 immunostain despite of
the positive staining for cytokeratin in one of the tumors.
None of our tumors was predominantly composed of
rhabdoid cells. On the contrary; small round blue cells
component was seen in 86.6% of our cases and was the
only component in a single case and the predominant
component in 2 others. This finding is well described
in literature with a more aggressive behavior than other
primitive tumors12,23. In addition; various mesenchymal
components can be seen including chondroid, myxoid and
spindle cell sarcoma, which can occasionally predominate24. Epithelial components in the form of papillary
structures, cohesive cell nests, tubule-glandular patterns
and keratinizing squamous islands have all been previously
reported25. This variable cellular and tissue patterns are
reflected in the variable immunostaining profile of AT/
RT26. Loss of INI-1 expression from tumor cell nuclei
remains currently the best available stain forming the basis
of the diagnosis of AT/RT in most cases. Caution should be
experienced in misinterpretation of irrelevant cytoplasmic
brush staining as positive staining. Also pathologists
should be aware of the occasional cases of AT/RT with
retained nuclear staining27. Staining for other markers
including insulin-like growth factor II, insulin-like growth
factor receptor type I, and cathepsin have been reported28. Interestingly; the embryonal/germ cell tumor marker
SALL4 has been recently described in cases of AT/RT29.
Immune-expression of claudin-6 has been proposed as a
specific positive diagnostic marker30, which was linked
to poor prognosis in some23,27 but not all studies31,32. The divergent differentiation characteristic of AT/RT
has been attributed by some to the presence of CD133+
stem cells33.
Two unusual morphological patterns were encountered in
three of our cases carrying a potential for misdiagnosis. AT/
RT mimicking nodular medulloblastoma was reported by
Varlet el al34. This was further confirmed at the somatic
level by detection of hemizygous deletion of the whole
hSNF5/INI-1 gene and point mutations in exon 2 of the
remaining allele. Haberler et al. described pale nodules in
one of their cases at post mortem examination. Unlike our
cases however, these nodules consisted of rhabdoid cells
on high power examination and did not show neuropil or
neuronal differentiation12.
The reactive gemistocytes in the third case exhibited
abundant acidophilic cytoplasm, and together with the
unexpected positivity for desmin brought resemblance to
rhabdomyoblasts. A case with similar findings was reported
by Fleming et al., which was initially misdiagnosed as
gliosarcoma23. Positivity for desmin in CNS tumors
should be interpreted with caution as certain clones particularly DE -R-11 is reported to show cross immunereactivity
in normal and tumor astrocytes35.
AT/RT is gaining more recognition by the reporting
pathologists over the last few years. Many of our cases
were initially misdiagnosed as other tumors, especially
medulloblastoma. Although at least focal rhabdoid cells
were seen on review in all but one case, these cells were
overlooked by the initial reporting pathologist. In addition,
INI-1 /BAF47 was not performed on most of the cases prior
to referral, since AT/RT was not suspected. In their series
on 12 cases Fleming et al. recognized retrospectively 10
cases23, while Woehner et al. on their review of Austrian
Brain Tumor Registry identified 52.6% cases that were
retrospectively diagnosed as AT/RT36. In our series the
number of correctly diagnosed referrals of AT/RT increased
since 2010, probably a reflection of the growing recognition
of the entity.
It is possible that inactivation of INI-1 may be associated
with a poor prognosis, even if the rhabdoid component
of the tumor is not apparent by histological or immunohistochemical
studies8. However; this has been recently
challenged with the description of a new entity, cribriform
neuroepithelial tumor (CRINET ), which despite of showing
loss of INI-1 /BAF47 immunostain, it carries a relatively
favourable prognosis37.
The lack of therapeutic response and dismal prognosis necessitates
separating AT/RT from other CNS embryonal
tumors including CNS-PNET , medulloblastoma, ependymoma
and others38, especially in infants and children
younger than 3 years of age at diagnosis and children with
metastatic disease39. The use of aggressive therapy modalities
including a combination of surgery, chemotherapy
with or without stem cell rescue, intra-thecal chemotherapy
and radiotherapy has resulted in some improvement in the
natural history of the tumor40. Gross total resection including
a second look surgery is the strongest factor associated
with survival13,14. The only long-term survival in
our series underwent GTR. However; all other 4 patients
with GTR have died as a result of the disease suggesting that
other factors are linked to outcome. The use of Head-Start-I
(HS I), which is the standard modality of treatment at our
center, or Head- Start-II (HS II) regimens, has resulted in
long-term survivors of up to 67 months following diagnosis41. Intra-thecal chemotherapy seems to decrease the
risk of cranio-spinal metastasis and improves the overall
survival42. Initial radiotherapy might be associated with
improved survival in a subset of patients43-46, despite of possible devastating neuro-cognitive outcome in the very
young.
In conclusion, we herein have presented fifteen cases of
AT/RT that we have encountered at our center, including
2 cases with exceptional intraventricular location, and 3
cases with unusual growth patterns. INI-1 immunostain
should be performed on all CNS embryonal tumors
encountered in infants and young children less than 5 years
of age, showing malignant small cells simulating CNSPNET
/ medulloblatoma, including lesions with favourable
histopathologic features such as nodular medulloblastoma,
even in the absence of detectable rhabdoid cells. The
proper diagnosis of AT/RT is important for providing the
appropriate intensive treatment for these patients. |
Top
Abstract
Introduction
Methods
Results
Discussion
References
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Top
Abstract
Introduction
Methods
Results
Discussion
References
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