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Prognostic role of overt hypercortisolism in completely operated patients with adrenocortical cancer.
[cushing syndrome]
Although
prognostic
parameters
are
important
to
guide
adjuvant
treatment
,
very
few
have
been
identified
in
patients
with
completely
resected
adrenocortical
carcinoma
(
ACC
)
.
To
assess
the
prognostic
role
of
clinical
symptoms
of
hypercortisolism
in
a
large
series
of
patients
with
completely
resected
ACC
.
A
total
of
524
patients
followed
at
referral
centers
for
ACC
in
Europe
and
the
United
States
entered
the
study
.
Inclusion
criteria
were
≥
18
yr
of
age
,
a
histologic
diagnosis
of
ACC
,
and
complete
surgery
(
R
0
)
.
Exclusion
criteria
were
a
history
of
other
malignancies
and
adjuvant
systemic
therapies
other
than
mitotane
.
A
ll
ACC
patients
were
completely
resected
,
and
adjuvant
mitotane
therapy
was
prescribed
at
the
discretion
of
the
investigators
.
The
primary
end
point
was
overall
survival
(
OS
)
.
The
secondary
end
points
were
recurrence-free
survival
(
RFS
)
and
the
efficacy
of
adjuvant
mitotane
therapy
according
to
cortisol
secretion
.
Overt
hypercortisolism
was
observed
in
197
patients
(
37
.
6
%
)
.
Patients
with
cortisol
excess
were
younger
(
p
=
0
.
002
)
;
no
difference
according
to
sex
and
tumor
stage
was
observed
.
The
median
follow-up
of
the
series
was
50
mo
.
After
adjustment
for
sex
,
age
,
tumor
stage
,
and
mitotane
treatment
,
the
prognostic
significance
of
cortisol
excess
was
highly
significant
for
both
RFS
(
hazard
ratio
[
HR
]
:
1
.
30
;
95
%
confidence
interval
[
CI
]
,
1
.
04
-
2
.
62
;
p
=
0
.
02
)
and
OS
(
HR
:
1
.
55
;
95
%
CI
,
1
.
15
-
2
.
09
;
p
=
0
.
004
)
.
Mitotane
administration
was
associated
with
a
reduction
of
disease
progression
(
adjusted
HR
:
0
.
65
;
95
%
CI
,
0
.
49
-
0
.
86
;
p
=
0
.
003
)
that
did
not
differ
according
to
the
patient
's
secretory
status
.
A
major
limitation
is
that
only
symptomatic
patients
were
considered
as
having
hypercortisolism
,
thus
excluding
information
on
the
prognostic
role
of
elevated
cortisol
levels
in
the
absence
of
a
clinical
syndrome
.
Clinically
relevant
hypercortisolism
is
a
new
prognostic
factor
in
patients
with
completely
resected
ACC
.
The
efficacy
of
adjuvant
mitotane
does
not
seem
to
be
influenced
by
overt
hypercortisolism
.
Diseases
Validation
Diseases presenting
"factor in patients"
symptom
cushing syndrome
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
liposarcoma
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