Adrenal disorders 2

Adrenal Disorders - 2
Prof. Tariq Waseem
Prof. Tariq Waseem 16/2/2015

6/2/2015 Prof. Tariq Waseem 2
Mont Nebo
Jordan

CASE SCENARIO
 A 52 years old male was investigated
for obesity of recent onset, polydipsia,
and polyuria.
 On examination, he had a B.P of
180/105mmhg and was obese, having
purplish striae over the trunk and flanks.

Diagnosis?
Cushing’s Syndrome

Definition
 A constellation of clinical abnormalities
due to chronic exposure to excess of
cortisol or related corticosteroid

Clinical Feature
Hypercotisolism
Lipid mobilization 
Lipid catabolism 
Lipid redistribution
Moon-face
buffalo hump
truncal obesity
Violaceous striae
Hepatic glucose
production
Insulin resistance
Glucose intolerance
protein metabolism negative
nitrogen balance
disruption of water and
electrocytes metabolism
Proximal muscle
weaknessDependent edema
Hypertension
Hypokalemic metabolic
alkalosis

CLINICAL FEATURES OF GLUCOCORTICOID EXCESS
Frequency(%)
Weight gain 90
“Moon facies” 75
Hypertension 75
Violaceous striae 65
Hirsutism 65
Glucose intolerance 65
Proximal muscle weakness 60
Plethora 60
Menstrual dysfunction 60
Acne 40
Easy bruising 40
Osteopenia 40
Dependent edema 40
Hyperpigmentation 20
Hypokalemic metabolic alkalosis 15Prof. Tariq Waseem 86/2/2015

FIGURE . Multiple wide striae on the abdomen of a patient
with Cushing's disease.

When stimulated by ACTH, the adrenal gland secretes cortisol and other steroid hormones.
ACTH is produced by the pituitary gland and released into the petrosal venous sinuses in
response to stimulation by corticotropin-releasing hormone (CRH) from the hypothalamus

Etiology and Pathophysiology
ACTH-dependent causes
ACTH-secreting pituitary tumor ( Cushing’ s disease )
Pituitary CRH-secreting neoplasm ( ectopic CRP syndrome )
Nonpituitary ACTH-secreting neoplasm ( ectopic ACTH syndrome )

ACTH-independent causes
Adrenal adenoma
Adrenal carcinoma
Micronodular adrenal disease
McCune-Albright syndrome
Massive macronodular adrenal diease

Pseudo-cushing Syndrome
Factitious or surreptitious glucocorticoid
administration

COMMON CAUSES OF ECTOPIC ACTH SECRETION
Small cell carcinoma of the lung 50%
Endocrine tumors of foregut origin 35%
Thymic carcinoid
Islet cell tumor
Medullary carcinoma thyroid
Bronchial carcinoid
Pheochromocytoma 5%
Ovarian tumors 2%

Diagnosis
 Clinical manifestations
 Lab findings
– Plasma cortisol and rhythm (RIA)
– Urinary free cortisol
17-hydroxycortisteriod
17-ketosteriods
– Plasma ACTH

Suppression tests
 Screening test
– 1mg DX P.O at midnight
– Plasma cortisol (PF) at 7-8 am next day
– PF suppressed: Normal
– PF NOT suppressed: Cushing’ s Syndrome

Suppression tests
 Low dose DX suppression test
– DX 0.5 mg q6h P.O 2 days
– Urinary free cortisol decreased: Normal
– Urinary free cortisol NOT decreased:
Cushing’ s Syndrome

Suppression tests
 Large dose DX suppression test
D.X 2mg q6h P.O 2 days
Urinary free cortisol reduced 50%:
Cushing’s disease (Pituitary adenoma)
Urinary free cortisol NOT reduced 50%:
Adrenal tumor,
Carcinoma,
Eectopic ACTH Syndrome

ACTH Stimulation test
 ACTH 25u intravenously 8h
 2-5 fold increase in urinary free cortisol
in Cushing’ s disease
 Plasma cortisol and urinary free cortisol
increase in half of adrenal adenoma
patients
 No response in adrenal carcinoma

CRH stimulation test
 Etiology diagnose (especially for pituitary ACTH-
dependent or ectopic ACTH syndrome)
 A newer approach is to combine a CRH stimulation test
with a dexamethasone suppression test(4mg ).
 method :
1 µg / kg of CRH is administered intravenously.
ACTH and cortisol levels are measured before CRH
injection and 15, 30, 45, 60, 90 and 120 minutes after
injection.
 A rise in the cortisol value of 20 percent or more above
basal level or a rise in the ACTH value of at least 50
percent above basal level is considered evidence for an
ACTH-dependent lesionProf. Tariq Waseem 226/2/2015

Metyrapone Test
 Etiology diagnose (especially for pituitary or adrenal)
– Metyrapone 2-3g (30mg/kg) P.O at midnight
– Urinary 17-OHCS, Plasma ACTH,11-deoxycortisol
more above basal level : Cushing’s disease
(Pituitary adenoma)
– No response in adrenal carcinoma , tumor, ectopic
ACTH Syndrome

Imaging diagnosis
 Pituitary CT has a sensitivity of about 50%
for identifying microadenomas
 MRI has increased sensitivity but is not 100%
predictive

Imaging diagnosis
 If diagnostic doubt need bilateral inferior
petrosal sinus sampling for ACTH

Imaging diagnosis
Adrenal ultrasonography---first choice
Abdominal CT will allow identification of adrenal
pathology

Imaging diagnosis
 Somatostatin scintigraphy to identify
sites of ectopic hormone production

Etiological diagnosis
 Cushing’ s disease:
 Adrenal adenoma:
 Adrenal carcinoma:
 Ectopic ACTH
Syndrome:
 Chronic, moderate clinical
features can be suppressed by
large dose test
 Shorter course , mild features
can NOT be suppressed by large
dose test
 Acute onset, progressive course,
hyperandrogenic effect
predominate, palpable mass, low
ACTH
 Appear suddenly, progress
rapidly, not typical manifestation
of Cushing’s syndrome,
hyperpigmentation, hypokalemia,
high ACTH

Differential diagnosis
 Simple obesity
– General obesity, long history, over nourished
– Narrow and short striae
– Urinary free cortisol can be suppressed by screening ( overnight )
test and/or low-dose DX suppression test
– Normal diurnal rhythm, almost normal plasma cortisol
 Type 2 DM
– Normal plasma cortisol and rhythm
– Once blood glucose controlled, urinary free cortisol turns to normal
 Alcoholic Cushingnoid Syndrome
– No drinking for one week, plasma cortisol and urinary free cortisol
become normal
 Depression
– Lack of clinical manifestation of Cushing’s Syndrome

Treatment
 Cushing’s disease
– Transsphenoidal microadenomectomy
– Pituitary radiation
– Bilateral total adrenolectomy
– Drugs
 Adrenal adenoma and carcinoma
– Surgical removal
– Drugs ( mitotane, metyrapone, ketoconazole ) for
nonresectable or metastatic carcinoma
 Ectopic ACTH Syndrome
– Surgical removal of the ectopic tumor
– Chemotherapy, radiotherapy
– Drugs ( mitotane, metyrapone, ketoconazloe )

MANAGEMENT
 CUSHING’s DISEASE :
 Surgical treatment :
 Trans –sphenoidal surgery for elective
removal of pituitary adenoma

CONTINUED
 BILATERAL ADRENALECTOMY :
 If bilateral adrenalectomy is done for
pituitary adenoma dependant cushings
syndrome it may lead to excessive
growth of the adenoma due to lack of
negative feed back provided by raised
cortisol this may lead to nelson’s
syndrome

NELSON ‘s SYNDROME
 Aggressive pituitary macroadenoma
and very high ACTH levels causing
pigmentation
 Nelson ‘s syndrome can be
prevented by pituitary irradiation

ADRENAL TUMOURS
 ADRENAL ADENOMA :
 Laproscopic removal
 ADRENAL CARCINOMA :
 Resection and irradiation of the
carcinoma followed by cytotoxic
chemotherapy

ECTOPIC ACTH SYNDROME
 Localized tumours causing this
syndrome should be removed for
example bronchial carcinoid

Medical therapy of Cushing’ s Disease
 Purpose
– Correct metabolic abnormalities before
attempted surgical cure
– Palliate surgically noncurable disease
– Achieve remission in patients for whom
surgery is unlikely to achieve satisfactory
long term results

 Steroidogenic inhibition
– Mitotane ( OP’-DDD
– Metyrapone
– Aminoglutethimide
– Ketoconazole
 Neuromodulatory treatment
– Bromocriptine
– Cyproheptadin
– Valproic acid
– Octreotide
– Glucocorticoid receptor antagonist
– RU486

CASE SCENARIO No.2
 A 34 years old female presented to the
opd with the complains of increasing
body weight over the past few months
along with complains of headache over
past 1 month
 On examination she is found to be
hypertensive ,and some black velvety
discoloration was noticed around her
neck and also in her armpits.

DIAGNOSIS
?

CASE SCENARIO No.3
 A 36 years old female was referred with
oligomenhorrea and hirsitism and
weight gain. She has also noticed
excessive hair on her face,arms and
legs. She has been on Hakim
medication for Asthma.
 On examinaion she has excessive acne
and facial hair.
 Her BP is 160/100 mmhg.Prof. Tariq Waseem 426/2/2015

DIAGNOSIS
?

Dead Sea
Jordan

Adrenal disorders 2

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