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Cushing syndrome secondary to CRH-producing Wilms tumor in a 6 year old.
[cushing syndrome]
Abstract
Cushing
syndrome
is
caused
by
prolonged
exposure
to
elevated
serum
cortisol
.
It
is
uncommon
in
children
,
and
etiology
includes
pituitary
adenoma
,
adrenal
tumor
,
and
exogenous
glucocorticoid
administration
.
Rarely
,
it
is
paraneoplastic
in
origin
.
We
present
a
case
of
paraneoplastic
Cushing
syndrome
due
to
Wilms
tumor
that
secreted
corticotropin-releasing
hormone
(
CRH
)
.
A
6
-
year
-old
male
presented
with
polyphagia
and
weight
gain
.
He
showed
Cushingoid
appearance
,
hypertension
,
and
palpable
left
flank
mass
.
Serum
cortisol
and
adrenocorticotropic
hormone
(
ACTH
)
levels
were
elevated
.
Computed
tomography
showed
a
neoplasm
originating
from
the
left
kidney
.
Pathologic
diagnosis
of
Wilms
tumor
was
made
upon
nephroureterectomy
.
Immunohistochemical
staining
was
positive
for
CRH
and
negative
for
ACTH
.
All
features
of
Cushing
syndrome
disappeared
after
surgery
.
This
represents
a
rare
case
of
Cushing
syndrome
secondary
to
Wilms
tumor
in
which
CRH
production
has
been
demonstrated
.