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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
"distinctive histologic features"
symptom
cushing syndrome
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