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Metabolomic profile of the adrenal gland: from physiology to pathological conditions.
[von hippel-lindau disease]
In
this
study
,
we
i
)
assessed
the
metabolic
profile
of
the
normal
adrenal
cortex
and
medulla
of
adult
human
subjects
by
means
of
(
1
)
H-
high
-resolution
magic-angle
spinning
nuclear
magnetic
resonance
(
HRMAS
NMR
)
spectroscopy
;
ii
)
compared
the
biochemical
profile
of
adenoma
(
Ad
)
,
adrenal
cortical
carcinoma
(
ACC
)
,
and
pheochromocytoma
(
PCC
)
samples
with
that
of
healthy
adrenal
tissue
samples
;
and
iii
)
investigated
the
metabolic
differences
between
ACCs
and
Ads
as
well
as
between
ACCs
and
PCCs
.
Sixty
-
six
tissue
samples
(
13
adrenal
cortical
tissue
,
eight
medullary
tissue
,
13
Ad
,
12
ACC
,
and
20
PCC
samples
)
were
analyzed
.
Adrenaline
and
noradrenaline
were
undetectable
in
cortical
samples
representing
the
metabolic
signature
of
the
tissue
derived
from
neural
crest
.
Similarity
between
the
metabolic
profile
of
Ads
and
that
of
the
normal
adrenal
cortex
was
shown
.
Inversely
,
ACC
samples
clearly
made
up
a
detached
group
exhibiting
the
typical
stigmata
of
neoplastic
tissue
such
as
choline-containing
compounds
,
biochemical
markers
of
anaerobic
processes
,
and
increased
glycolysis
.
Significantly
higher
levels
of
lactate
,
acetate
,
and
total
choline-containing
compounds
played
a
major
role
in
the
differentiation
of
ACCs
from
Ads
.
Moreover
,
the
high
fatty
acid
content
of
ACCs
contributed
to
the
cluster
identification
of
ACCs
.
Of
the
14
sporadic
PCC
samples
,
12
exhibited
predominant
or
exclusive
noradrenaline
secretion
.
The
noradrenaline
:
adrenaline
ratio
was
inverted
in
the
normal
medullary
tissue
samples
.
Multiple
endocrine
neoplasia
type
2
-
and
NF
1
-
related
PCC
samples
exhibited
both
adrenaline
and
noradrenaline
secretion
.
In
the
von
Hippel-
Lindau
disease
-related
PCC
samples
,
only
noradrenaline
secretion
was
detected
by
HRMAS
NMR
spectroscopy
.
This
study
is
one
of
the
first
applications
of
metabolomics
to
adrenal
pathophysiology
and
it
is
the
largest
study
to
report
HRMAS
NMR
data
related
to
the
adrenal
cortex
and
adrenal
cortical
tumors
.
Diseases
Validation
Diseases presenting
"total choline-containing compounds"
symptom
von hippel-lindau disease
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