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Von hippel-lindau disease associated pulmonary carcinoid with cranial metastasis.
[von hippel-lindau disease]
Carcinoids
have
rarely
been
described
in
von
Hippel-
Lindau
(
VHL
)
disease
.
We
describe
the
first
reported
case
of
a
patient
with
VHL
who
developed
a
pulmonary
carcinoid
that
subsequently
metastasized
to
a
pre-existent
cranial
hemangioblastoma
.
Histological
and
immunohistochemical
features
of
the
metastatic
lesion
were
similar
to
the
primary
carcinoid
.
Both
lesions
demonstrated
heterozygous
VHL
gene
deletions
with
fluorescence
in
situ
hybridization
analysis
.
This
case
provides
direct
molecular
genetic
evidence
of
an
association
between
VHL
and
carcinoids
.
