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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
.
Diseases
Validation
Diseases presenting
"neoplasm"
symptom
adrenal incidentaloma
alexander disease
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
erdheim-chester disease
esophageal adenocarcinoma
esophageal carcinoma
familial hypocalciuric hypercalcemia
focal myositis
hodgkin lymphoma, classical
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kabuki syndrome
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary effusion lymphoma
proteus syndrome
pyomyositis
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wolf-hirschhorn syndrome
This symptom has already been validated