Learning Objectives: (1) Recognize the clinical
features and establish the diagnosis of Cushing’s syndrome,
(2) Localize ectopic ACTH-secreting tumors in Cushing’s
syndrome with the use of In-Pentetreotide (octreotide)
scintigraphy.
Case: A 39-year-old woman is admitted with
generalized weakness and cramps, and a potassium level 1.7
mmol/L. The patient also complained of two months of facial
fullness, hirsutism, acne, hair thinning, dysesthesia,
irregular menstruation, hyperpigmentation of her face and
upper trunk, and dizziness. The patient has no known past
history of diabetes of hypertension, but was found to have a
random blood sugar of 430 mg/dL and had developed
hypertension.
Labs: morning plasma cortisol level 63 m
/dL (normal 5-25 m g/dL), free
urinary cortisol level 3100 m /24
hours (normal 20-100 m gh/24-4 and
3400 m g/24-4 after 2.0 mg and 8.0
mg dexamethasone respectively. Fasting morning ACTH level
181.1 pg/ml (normal <60 pg/ml), repeat level after 8.0 mg
dexamethasone 303 pg/ml. Urinary 17-ketosteroids 47 mg/24-h
(normal 5-15 mg/24-h).
MRI of the head and pituitary ruled out pituitary
microadenoma. CT of the chest & abdomen were performed to
evaluate the adrenal glands and rule out tumor. The results
showed bilateral adrenal enlargement and multiple hepatic
"hemangiomas." Tumor localization SPECT imaging
using In-Pentetreotide (octreotide) scintigraphy was then
obtained to evaluate for ectopic-ACTH secreting lesion. This
revealed abnormal accumulation of tracer in a mass anterior to
the aorta adjacent to the uncal process of the pancreas. Upon
retrospective evaluation of the abdominal CT, a small mass was
observed.
She underwent surgery which revealed unresectable islet
cell carcinoma and multiple hepatic metastases. Bilateral
adrenalectomy was performed to control the Cushing’s
syndrome.
Discussion: 5-10% of all cases of Cushing’s
syndrome are caused by ectopic ACTH secretion. The efficacy of
In-Pentetreotide scintigraphy in localizing ectopic
ACTH-secreting tumors in Cushing’s syndrome is
controversial. We present a case of the usefulness of
somatostatin receptor scintigraphy in localizing this patient’s
lesion.