Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Polymorphous oligodendroglioma of Zülch revisited: a genetically heterogeneous group of anaplastic gliomas including tumors of bona fide oligodendroglial differentiation.
[alpha-thalassemia]
A
polymorphous
variant
of
oligodendroglioma
was
described
by
K
.
J
.
Zülch
half
a
century
ago
,
and
is
only
very
sporadically
referred
to
in
the
subsequent
literature
.
In
particular
,
no
comprehensive
analysis
with
respect
to
clinical
or
genetic
features
of
these
tumors
is
available
.
From
a
current
perspective
,
the
term
polymorphous
oligodendroglioma
(
pO
)
may
appear
as
contradictory
in
terms
,
as
nuclear
monotony
is
a
histomorphological
hallmark
of
oligodendrogliomas
.
For
the
purpose
of
this
study
,
we
defined
pO
as
diffusely
infiltrating
gliomas
felt
to
be
of
oligodendroglial
rather
than
astrocytic
differentiation
and
characterized
by
the
presence
of
multinucleate
tumor
giant
cells
and
/
or
nuclear
pleomorphism
.
In
a
total
of
nine
patients
,
we
identified
tumors
consistent
with
this
working
definition
.
All
tumors
were
high
-grade
.
We
characterized
these
with
respect
to
clinical
,
histomorphological
and
genetic
features
.
Despite
clinical
and
genetic
heterogeneity
,
we
identified
a
subset
of
tumors
of
bona
fide
oligodendroglial
differentiation
as
characterized
by
combined
loss
of
heterozygosity
of
chromosome
arms
1
p
and
19
q
(
LOH
1
p
19
q
)
.
Those
tumors
that
lacked
LOH
1
p
19
q
showed
a
high
frequency
of
IDH
1
mutations
and
loss
of
alpha
thalassemia
/
mental
retardation
syndrome
X-
linked
gene
(
ATRX
)
immunoreactivity
,
indicating
a
possible
phenotypic
convergence
of
true
oligodendrogliomas
and
gliomas
of
the
alternative
lengthening
of
telomeres
(
ALT
)
pathway
.
p
53
alterations
were
common
irrespective
of
the
1
p
19
q
status
.
Histomorphologically
,
the
tumors
featured
interspersed
bizarre
multinucleate
giant
tumor
cells
,
while
the
background
population
varied
from
monotonous
to
significantly
pleomorphic
.
Our
findings
indicate
,
that
a
rare
polymorphous
-
or
"
giant
cell
"
-
variant
of
oligodendroglioma
does
indeed
exist
.
Diseases
Validation
Diseases presenting
"tumor cells"
symptom
alpha-thalassemia
carcinoma of the gallbladder
cholangiocarcinoma
cushing syndrome
dedifferentiated liposarcoma
dentin dysplasia
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
junctional epidermolysis bullosa
kindler syndrome
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary effusion lymphoma
severe combined immunodeficiency
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom