Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
ACTH-secreting Pancreatic Neoplasms Associated With Cushing Syndrome: Clinicopathologic Study of 11 Cases and Review of the Literature.
[cushing syndrome]
Adrenocorticotropic
hormone
(
ACTH
)
-
secreting
pancreatic
neuroendocrine
tumors
(
PanNETs
)
,
although
rare
,
are
responsible
for
about
15
%
of
ectopic
Cushing
syndrome
(
CS
)
.
They
represent
a
challenging
entity
because
their
preoperatory
diagnosis
is
frequently
difficult
,
and
clear-cut
morphologic
criteria
useful
to
differentiate
them
from
other
types
of
PanNETs
have
not
been
defined
.
Ectopic
ACTH
secretion
associated
with
CS
can
also
be
rarely
due
to
pancreatic
acinar
cell
carcinoma
(
ACC
)
and
pancreatoblastoma
,
rare
tumor
types
with
morphologic
features
sometimes
overlapping
those
of
PanNETs
and
,
for
this
reason
,
representing
a
diagnostic
challenge
for
pathologists
.
We
herein
describe
the
clinicopathologic
and
immunohistochemical
features
of
10
PanNETs
and
1
ACC
secreting
ACTH
and
associated
with
CS
together
with
an
extensive
review
of
the
literature
to
give
the
reader
a
comprehensive
overview
on
ACTH-producing
pancreatic
neoplasms
.
ACTH-secreting
PanNETs
are
aggressive
neoplasms
with
an
immunohistochemical
profile
that
partially
overlaps
that
of
pituitary
corticotroph
adenomas
.
They
are
generally
large
and
well-differentiated
neoplasms
without
distinctive
histologic
features
but
with
signs
of
aggressiveness
including
vascular
and
perineural
invasion
.
They
are
more
frequent
in
female
individuals
with
a
mean
age
of
42
years
.
At
5
and
10
years
after
diagnosis
,
35
%
and
16
.
2
%
of
patients
,
respectively
,
were
alive
.
ACTH-secreting
ACCs
and
pancreatoblastomas
are
very
aggressive
pediatric
tumors
with
a
poor
prognosis
.
Using
an
appropriate
immunohistochemical
panel
including
ACTH
,
β-endorphin
,
trypsin
,
and
BCL
10
it
is
possible
to
recognize
ACTH-secreting
PanNETs
and
to
distinguish
them
from
the
very
aggressive
ACTH-secreting
ACCs
.
Diseases
Validation
Diseases presenting
"rare tumor types with morphologic features sometimes overlapping those of pannets and"
symptom
cushing syndrome
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom