The Journal of Clinical Endocrinology & Metabolism,
Journal Year:
2023,
Volume and Issue:
108(12), P. 3214 - 3222
Published: June 15, 2023
The
MPOWERED
core
trial
(NCT02685709)
and
open-label
extension
(OLE)
phase
investigated
long-term
efficacy
safety
of
oral
octreotide
capsules
(OOC)
in
patients
with
acromegaly.
Core
primary
endpoint
data
demonstrated
noninferiority
to
injectable
somatostatin
receptor
ligands
(iSRLs).
completers
were
invited
participate
the
OLE
phase.To
assess
OOC
acromegaly
who
previously
responded
tolerated
both
octreotide/lanreotide
completed
phase.The
unique
study
design
transitioning
between
iSRLs
allowed
within-patient
evaluations.
proportion
biochemical
responders
(insulin-like
growth
factor
I
<
1.3
×
upper
limit
normal)
at
end
each
year
entered
that
as
was
main
outcome
measure.At
1
end,
52/58
from
monotherapy
combination
therapy
groups
(89.7%;
95%
CI
78.8-96.1),
36/41
(87.8%;
73.8-95.9)
2,
29/31
(93.5%;
78.6-99.2)
3.
No
new
or
unexpected
signals
detected;
patient
withdrew
owing
treatment
failure.
Patients
transitioned
reported
improved
convenience/satisfaction
symptom
control.Patient-reported
support
for
first
time
randomized
iSRL
(who
iSRLs)
back
had
a
significant
effect
on
patients'
symptoms
score
prospective
cohort.
showed
maintenance
response
sustained
OOC.
JAMA,
Journal Year:
2023,
Volume and Issue:
329(16), P. 1386 - 1386
Published: April 25, 2023
Importance
Pituitary
adenomas
are
neoplasms
of
the
pituitary
adenohypophyseal
cell
lineage
and
include
functioning
tumors,
characterized
by
secretion
hormones,
nonfunctioning
tumors.
Clinically
evident
occur
in
approximately
1
1100
persons.
Observations
classified
as
either
macroadenomas
(≥10
mm)
(48%
tumors)
or
microadenomas
(&lt;10
mm).
Macroadenomas
may
cause
mass
effect,
such
visual
field
defects,
headache,
and/or
hypopituitarism,
which
about
18%
to
78%,
17%
75%,
34%
89%
patients,
respectively.
Thirty
percent
adenomas,
do
not
produce
hormones.
Functioning
tumors
those
that
an
excess
normally
produced
hormones
prolactinomas,
somatotropinomas,
corticotropinomas,
thyrotropinomas,
prolactin,
growth
hormone,
corticotropin,
thyrotropin,
Approximately
53%
can
hypogonadism,
infertility,
galactorrhea.
Twelve
acromegaly
adults
gigantism
children,
4%
secrete
corticotropin
autonomously,
resulting
hypercortisolemia
Cushing
disease.
All
patients
with
require
endocrine
evaluation
for
hormone
hypersecretion.
Patients
additionally
compressing
optic
chiasm
should
be
referred
ophthalmologist
formal
testing.
For
requiring
treatment,
first-line
therapy
is
usually
transsphenoidal
surgery,
except
medical
therapy,
bromocriptine
cabergoline,
first
line.
Conclusions
Relevance
manifest
affect
people
complicated
syndromes
well
defects
hypopituitarism
from
effect
larger
First-line
prolactinomas
consists
surgery
other
treatment.
JAMA,
Journal Year:
2023,
Volume and Issue:
330(2), P. 170 - 170
Published: July 11, 2023
Importance
Cushing
syndrome
is
defined
as
a
prolonged
increase
in
plasma
cortisol
levels
that
not
due
to
physiological
etiology.
Although
the
most
frequent
cause
of
exogenous
steroid
use,
estimated
incidence
endogenous
overproduction
ranges
from
2
8
per
million
people
annually.
associated
with
hyperglycemia,
protein
catabolism,
immunosuppression,
hypertension,
weight
gain,
neurocognitive
changes,
and
mood
disorders.
Observations
characteristically
presents
skin
changes
such
facial
plethora,
easy
bruising,
purple
striae
metabolic
manifestations
excess
fat
deposition
face,
back
neck,
visceral
organs.
disease,
which
corticotropin
produced
by
benign
pituitary
tumor,
occurs
approximately
60%
70%
patients
production.
Evaluation
possible
begins
ruling
out
use.
Screening
for
elevated
performed
24-hour
urinary
free
test
or
late-night
salivary
evaluating
whether
suppressed
morning
after
an
evening
dexamethasone
dose.
Plasma
can
help
distinguish
between
adrenal
causes
hypercortisolism
(suppressed
corticotropin)
corticotropin-dependent
forms
(midnormal
levels).
Pituitary
magnetic
resonance
imaging,
bilateral
inferior
petrosal
sinus
sampling,
whole-body
imaging
identify
tumor
sources
hypercortisolism.
Management
surgery
remove
source
production
followed
medication
includes
steroidogenesis
inhibitors,
pituitary-targeted
drugs,
glucocorticoid
receptor
blockers.
For
responsive
medication,
radiation
therapy
adrenalectomy
may
be
appropriate.
Conclusions
Relevance
The
First-line
causative
tumor.
Many
will
require
additional
treatment
medications,
radiation,
adrenalectomy.
Pituitary,
Journal Year:
2023,
Volume and Issue:
unknown
Published: Nov. 3, 2023
Abstract
Purpose
The
14th
Acromegaly
Consensus
Conference
was
convened
to
consider
biochemical
criteria
for
acromegaly
diagnosis
and
evaluation
of
therapeutic
efficacy.
Methods
Fifty-six
experts
from
16
countries
reviewed
discussed
current
evidence
focused
on
assays;
the
role
imaging,
pathology,
clinical
assessments;
consequences
diagnostic
delay;
remission
recommendations
follow
up;
value
assessment
monitoring
in
defining
disease
progression,
selecting
appropriate
treatments,
maximizing
patient
outcomes.
Results
In
a
with
typical
features,
insulin-like
growth
factor
(IGF)-I
>
1.3
times
upper
limit
normal
age
confirms
diagnosis.
Random
hormone
(GH)
measured
after
overnight
fasting
may
be
useful
informing
prognosis,
but
is
not
required
For
patients
equivocal
results,
IGF-I
measurements
using
same
validated
assay
can
repeated,
oral
glucose
tolerance
testing
might
also
useful.
Although
primary
treatment
outcome,
findings
should
interpreted
within
context
acromegaly.
Follow
up
assessments
effectiveness,
imaging
studies
evaluating
residual/recurrent
adenoma
mass,
signs
symptoms
acromegaly,
its
complications,
comorbidities.
Referral
multidisciplinary
pituitary
center
considered
biochemical,
or
at
diagnosis,
insufficiently
responsive
standard
approaches.
Conclusion
highlight
new
understandings
disordered
GH
importance
expert
management
this
rare
disease.
Pituitary,
Journal Year:
2023,
Volume and Issue:
26(5), P. 583 - 596
Published: Aug. 28, 2023
The
Pituitary
Society
established
the
concept
and
mostly
qualitative
parameters
for
defining
uniform
criteria
Tumor
Centers
of
Excellence
(PTCOEs)
based
on
expert
consensus.
Aim
study
was
to
validate
those
previously
proposed
through
collection
evaluation
self-reported
activity
several
internationally-recognized
tertiary
pituitary
centers,
thereby
transforming
2017
definition
into
a
validated
quantitative
one,
which
could
serve
as
basis
future
objective
PTCOE
accreditation.An
ad
hoc
prepared
database
distributed
nine
chosen
by
Project
Scientific
Committee
comprising
worldwide
repute,
agreed
provide
information
derived
from
registries
related
years
2018-2020
completing
within
60
days.
database,
provided
each
center
composed
Excel®
spreadsheets
with
requested
specific
leading
supporting
teams,
reviewed
two
blinded
referees
all
9
candidate
centers
satisfied
overall
definition,
according
referees'
evaluations.
To
obtain
numerical
criteria,
median
values
activity/parameter
were
considered
preferred
target,
whereas
low
limit
range
selected
acceptable
target
respective
parameter.Three
dedicated
neurosurgeons
are
preferred,
one
surgeon
is
acceptable.
Moreover,
100
surgical
procedures
per
year
while
results
indicated
that
50
surgeries
Acute
post-surgery
complications,
including
mortality
readmission
rates,
should
preferably
be
negligible
or
nonexistent,
but
criterion
rate
lower
than
10%
patients
complications
requiring
30
days
after
surgery.
Four
endocrinologists
devoted
diseases
in
total
population
followed
not
less
850.
It
appears
at
least
dedicated/expert
present
neuroradiology,
pathology,
ophthalmology
groups,
radiation
oncologists
needed.This
is,
our
knowledge,
first
survey
evaluate
relevant
number
high-volume
field.
This
effort,
internally
reviewers,
allowed
transformation
formulated
theoretical
precise
definitions
real-life
evidence.
application
synopsis
used
independent
bodies
accreditation
PTCOEs.
Cell Reports Medicine,
Journal Year:
2023,
Volume and Issue:
4(2), P. 100934 - 100934
Published: Feb. 1, 2023
Pituitary
neuroendocrine
tumor
(PitNET)
is
one
of
the
most
common
intracranial
tumors
with
variable
recurrence
rate.
Currently,
prediction
unsatisfying
and
can
be
improved
by
understanding
cellular
origins
differentiation
status.
Here,
to
comprehensively
reveal
origin
PitNET,
we
perform
comparative
analysis
single-cell
RNA
sequencing
data
from
3
anterior
pituitary
glands
21
PitNETs.
We
identify
distinct
genes
representing
major
subtypes
well
poorly
differentiated
PitNETs
in
each
lineage.
To
further
verify
predictive
value
biomarkers,
include
an
independent
cohort
800
patients
average
follow-up
7.2
years.
In
both
PIT1
TPIT
lineages,
groups
show
significantly
higher
rates
while
well-differentiated
SF1
Our
findings
possible
status
PitNET
based
on
which
new
classification
proposed
verified
predict
recurrence.
Pituitary,
Journal Year:
2024,
Volume and Issue:
27(4), P. 381 - 388
Published: June 4, 2024
Abstract
Purpose
A
series
of
consensus
guidelines
on
medical
treatment
acromegaly
have
been
produced
in
the
last
two
decades.
However,
little
information
is
available
their
application
clinical
practice.
Furthermore,
international
standards
care
not
published.
The
aim
our
study
was
to
report
current
for
therapy
acromegaly,
using
results
collected
through
an
audit
performed
validate
criteria
definition
Pituitary
Tumor
Centers
Excellence
(PTCOE).
Methods
Details
approaches
were
voluntarily
provided
by
nine
renowned
centers
that
participated
this
audit.
For
period
2018–2020,
we
assessed
overall
number
patients
under
treatment,
distribution
different
modalities,
biochemical
control
rate
with
therapy,
and
specific
rates
options.
Results
Median
total
median
new
managed
annually
endocrinology
units
206
16.3,
respectively.
percentage
48.9%.
Among
first-generation
somatostatin
receptor
ligand
(SRL)
monotherapy
used
a
48.7%,
followed
combination
therapies
29.3%.
Cabergoline
6.9%
patients.
Pegvisomant
7
pasireotide
5
centers,
7.9%
6.3%,
Conclusions
Current
PTCOEs
include
use
SRLs
as
first
option
about
50%
patients,
recommended
guidelines.
some
are
kept
despite
inadequate
suggesting
cost-effectiveness,
availability,
patient
preference,
side
effects,
therapeutic
inertia
may
play
possible
role
also
PTCOE.
Moreover,
at
odds
guidelines,
other
monotherapies
appear
marginal
compared
extrapolated
from
PTCOE
practice
data.
Presence
uncontrolled
each
category
suggest
further
optimization
well
tools
such
radiosurgery
be
needed.