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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
"high frequency"
symptom
alpha-thalassemia
canavan disease
classical phenylketonuria
cohen syndrome
cowden syndrome
cystinuria
erdheim-chester disease
familial hypocalciuric hypercalcemia
familial mediterranean fever
gm1 gangliosidosis
heparin-induced thrombocytopenia
hirschsprung disease
homocystinuria without methylmalonic aciduria
inclusion body myositis
kabuki syndrome
kallmann syndrome
krabbe disease
lymphangioleiomyomatosis
neonatal adrenoleukodystrophy
papillon-lefèvre syndrome
pyruvate dehydrogenase deficiency
waldenström macroglobulinemia
x-linked adrenoleukodystrophy
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