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[Local recurrence of pheochromocytoma associated with von Hippel-Lindau disease 26 years after bilateral adrenalectomy : a case report].
[von hippel-lindau disease]
A
60
-
year
-old
man
who
had
undergone
left
adrenalectomy
and
right
partial
adrenalectomy
for
bilateral
pheochromocytoma
26
years
ago
was
found
to
have
an
elevated
serum
noradrenaline
(
NA
)
and
dopamine
(
DA
)
during
a
long
-term
follow-up
.
At
the
time
of
right
partial
adrenalectomy
,
the
normal
part
of
the
right
adrenal
gland
was
preserved
.
His
cousin
and
second
cousin
had
pheochromocytoma
associated
with
von
Hippel-
Lindau
(
VHL
)
disease
.
His
eldest
daughter
had
cerebellar
hemangioblastoma
.
Computed
tomography
and
magnetic
resonance
imaging
revealed
a
tumor
which
was
17
mm
in
diameter
with
contrast
enhancement
in
the
vicinity
of
the
S
6
region
in
the
liver
.
123
I
-
metaiodobenzylguanidine
(
MIBG
)
scintigraphy
showed
an
abnormal
accumulation
in
the
same
area
.
The
tumor
was
surgically
removed
under
the
diagnosis
of
recurrence
of
pheochromocytoma
.
Histopathological
examination
revealed
findings
consistent
with
recurrent
pheochromocytoma
.
After
operation
,
serum
NA
and
DA
returned
to
normal
range
and
the
abnormal
up-take
on
12
3
I
-MIBG
scintigraphy
disappeared
.
Genetic
testing
revealed
that
the
patient
and
his
daughter
had
VHL
disease
.
An
extensive
genetic
examination
and
long
-term
follow-up
should
be
considered
for
the
present
family
.
