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VHL-deficient vasculogenesis in hemangioblastoma.
[von hippel-lindau disease]
Hemangioblasts
are
capable
of
differentiation
into
vascular
structures
and
blood
.
Patients
with
von
Hippel-
Lindau
(
VHL
)
disease
develop
hemangioblastomas
which
are
composed
of
VHL
-
deficient
tumor
cells
with
protracted
hemangioblastic
differentiation
potential
.
In
a
subset
of
these
tumors
,
hemangioblastic
differentiation
is
characterized
by
different
stages
of
red
blood
cell
formation
.
It
has
remained
controversial
,
however
,
whether
VHL
-
deficient
hemangioblastic
cells
are
similarly
capable
of
differentiating
into
vascular
cells
and
functioning
vascular
structures
in
vivo
.
By
histologic
,
immunohistologic
and
microdissection-based
genetic
analysis
of
60
VHL
disease-associated
hemangioblastomas
,
we
re
-examined
the
controversial
question
whether
VHL
-
deficient
neoplastic
hemangioblastic
cells
are
capable
of
vascular
differentiation
(
vasculogenesis
)
.
In
most
tumors
(
n
=
47
)
,
there
was
no
evidence
of
either
vasculogenesis
or
hematopoiesis
;
tumor
cells
were
either
scattered
between
reactive
angiogenetic
vascular
structures
or
arranged
in
solid
clusters
.
A
subset
of
tumors
(
n
=
13
)
,
however
,
revealed
vaculogenetic
structures
that
were
composed
of
cuboidal
or
flat
cells
and
frequently
contained
red
blood
cell
precursors
or
mature
red
blood
cells
.
Microdissection-based
deletion
analysis
of
epithelial
cells
confirmed
them
to
be
VHL
-
deficient
tumor
cells
.
Immunohistochemistry
for
CD
31
was
consistently
negative
in
these
structures
,
and
no
evidence
could
be
obtained
for
connectivity
with
reactive
vasculature
.
We
demonstrate
that
hemangioblastic
differentiation
capacity
of
VHL
-
deficient
hemangioblastic
cells
includes
not
only
erythropoiesis
,
but
also
differentiation
into
primitive
vasculogenetic
structures
.
Tumor
cells
,
however
,
do
not
appear
to
have
the
potential
of
terminal
differentiation
into
mature
and
functional
vascular
structures
.
Diseases
Validation
Diseases presenting
"vascular differentiation"
symptom
von hippel-lindau disease
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