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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
"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
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