Rare Diseases Symptoms Automatic Extraction
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ACTH-secreting neuroendocrine pancreatic tumor: a case report.
[cushing syndrome]
Incidence
of
neuroendocrine
tumor
(
NET
)
is
increased
in
the
last
thirty
years
from
1
.
1
to
5
.
2
cases
per
100
,
000
people
in
the
United
States
.
They
can
originate
from
the
pancreatic
gland
and
for
the
majority
of
cases
are
not
functioning
(
80
%
)
.
A
small
percentage
of
functioning
may
produce
adrenocorticotropic
hormone
(
ACTH
)
and
lead
to
ectopic
ACTH
Syndrome
(
EAS
)
,
responsible
of
Cushing-
Syndrome
.
We
present
a
case
of
a
30
year
-old
woman
suffering
from
EAS
due
to
a
neoformation
of
the
pancreatic
tail
of
the
maximum
diameter
of
4
Â
cm
.
The
lesion
was
resectable
at
preoperatory
imaging
.
The
patient
was
subjected
to
distal
splenopancreasectomy
.
Histological
examination
showed
a
well-differentiated
neuroendocrine
carcinoma
pT
3
N
0
.
The
postoperative
course
was
regular
.
At
two
years
of
follow-up
patient
is
almost
completely
asymptomatic
for
Cushing
's
but
she
has
developed
multiple
liver
metastases
,
for
which
she
began
chemotherapy
.
p
-
NET
responsible
for
EAS
is
usually
malignant
and
the
radical
treatment
of
excision
of
the
lesion
is
not
possible
because
they
occur
at
the
time
of
diagnosis
with
liver
metastases
or
unresectable
.
Our
patient
had
a
mass
at
the
time
of
diagnosis
resectable
but
despite
radical
surgery
,
she
has
developed
multiple
liver
metastases
at
two
years
and
she
was
undergoing
chemotherapy
.
In
agreement
with
previous
literature
we
confirm
the
aggressive
nature
of
pancreatic
tumors
secreting
ACTH
,
despite
radical
surgery
.
Conversely
,
surgical
treatment
is
effective
on
the
resolution
of
clinical
symptoms
.
