HIMAWAN SANUSI

Adrenal disorders
Cortex Hypersecretion

Medulla
insufficiency
 ADRENAL GLAND

ADRENAL CORTEX
Glucocorticoid (C21) Cortisol
Mineralocorticoid Aldosterone
Adrenal Androgen

ADRENAL MEDULLA
Epinephrine / norepinephrine
 Disorders of adrenocortical
function
Adrenocortical hyperfunction
Glucocorticoids Cushings syndrome
Aldosteronism Conns disease

Adrenocortical hypofunction
Hypopituitarism Glucocorticoids Addisons
Hypoaldosteronism Mineralocorticoids disease
Hypopituitarism Androgens
ADRENAL HYPERFUNCTION

Cushings Syndrome
 CUSHINGS SYNDROME
Chronic glucocorticoid excess symptoms &
physical features Cushings Syndrome

Etiology :
o Iatrogenic CS : Chronic glucocorticoid therapy
(most commonly)
o Spontaneous CS :
Pituitary (Cushing disease)
Adrenal adenoma
ACTH secrt non pituitary tumor (ectopic ACTH Syndrome)
Etiology

ACTH-independent
- adrenal tumor
- exogenous
glucocorticoid

ACTH-dependent
- pituitary adenoma
- Ectopic tumor secrt
ACTH
 CUSHINGS SYNDROME
Classification

ACTH-dependent
Pituitary adenoma (Cushing disease) 70%
Nonpituitary neoplasma (ectopic ACTH)

ACTH-independent
Iatrogenic (glucocorticoid, megestrol acetat)
Adrenal neoplasma (adenoma, carcinoma)
Hyperplasia adrenal
Factitious
Indicative of
Cushing
Syndrome
Cushing disease

Tumor producing
ACTH
small cell (oat cell)
bronchogenic carcinoma ,
carcinoid tumors of the
thymus, pancreas, or ovary;
thyroid medullary carcinoma
bronchial adenomas

Adrenal tumor
 ETIOLOGY DIAGNOSIS OF
CUSHINGS SYNDROME
Cushings syndrome established
ACTH IRMA

<5 pg/mL >10 ug/mL

CT adrenals MRI pituitary

Unilateral mass Bilateral enlargement IPSS Normal Abnormal

CRH test

Peak ACTH Peak ACTH IPS:P<1.8 IPS:P>2.0

<10pg/ml >20 ug/dL

Adrenal surgery Ectopic ACTH Pituitary

Surgery
 CUSHINGSSYNDROME
CUSHINGS SYNDROME
Treatment :
Cushings disease
Microsurgery, Radiation therapy
Adrenal tumors
Unilateral adrenalectomy
Ectopic ACTH syndrome
Benign surgical treatment
Malignant Ketokonazole, metyrapone
Iatrogenic (Cushingoid)
Tapering off
Alternate day regimen
PSEUDO CUSHINGS SYNDROME

Obesity
Chronic alcoholism
Depression
Hirsutism Post adrenalectomy
 Adrenal Hyperfunction

Hyperaldosteronism
 ACTH

Aldosterone

K Angiotensin II

Angiotensin I
EBV
Renin

Renin substrate

Major factors regulating aldosterone secretion; EBF(Effective Blood Volume)

Primary Hyperaldosteronism
Accounts for about 0.7% of cases of hypertension
Insidens : Women >>
Etiology :
Unilateral adrenocortical adenoma (Conns diss -
73%)
Bilateral (27%)
Hyperaldosteronism :
DIASTOLIC HYPERTENSION
HYPOKALEMIA
METABOLIC ALKALOSIS
 Primary Hyperaldosteronism
Clinical finding : Hypertension, muscular
weakness, paresthesias, headache,
polydipsia, polyuria, moderate hypertension
(malignant is rare)

Laboratory finding : Serum potassium low,

24 hours urine collection aldosterone

Imaging : CT-scan
Primary Hyperaldosteronism

Treatment :
Laparoscopic adrenalectomy
Pharmaco : spironolactone and antihyper
tensive agent
Complication : Renal damage
Prognosis :
Improved by early diagnosis and treatment,
only 2% malignant
 Secondary Hyperaldosteronism
Definition : an appropriately increased production
of aldosterone in response to activation of the
renin-angiotensin system

Characterized by :
- hypokalemic alkalosis, hypertension
- moderate to severe increases in plasma renin
- moderate to marked increases in aldosterone levels
Secondary Hyperaldosteronism
Cause :
Stenosis artery renalis
Congestive heart failure
Nephrotic syndrome
Cirrhocis hepatic

Production rate of aldosterone is often higher

in patients with secondary than in those with
primary aldosteronism
Disorders of adrenocortical insufficiency

Deficient adrenal production of glucocorticoid

and mineralocorticoid Adrenocortical
insufficiency
@ Primary adrenocortical insufficiency damage
of adrenal (Addisons disease)
@ Secondary - deficient pituitary ACTH secretion,
glucocorticoid therapy (most common)
@ Tertiary adrenal insufficiency

hypothalamic disfunction (very rare)

 Adrenal Insuffiency
Etiologi :
Tbc (prior 1920)
Autoimmune adrenalitis adrenal atrophy (80%)
Associated other immunologic and autoimmune endocrine
disorders
AIDS, malignant disease
Incidens :
5 in 10,000 general population.
Hypothalamic-pituitary : 3 in 10,000
Primary adrenal insufficiency : 2 in 10,000
female >>, 30-50 years
Addisons Disease
Secondary - Deficient pituitary ACTH
secretion

Patients receiving long-term steroid

therapy, despite physical findings of
Cushings syndrome Adrenal
insufficiency
Prolonged pituitary - hypothalamic
suppression + adrenal atrophy secondary
to the loss of endogenous ACTH
 Addisons Disease
Laboratorium :
Hiponatremia- hiperkalemia (classic)
Radiologis /CT Scan
Diagnosis
Basal adrenokortical steroid Normal
Rapid ACTH stimulation test
ACTH plasma
Treatment:
Replacement therapy cortisol + fluid
replacement
Diseases of adrenal medulla

Pheochromacytoma
 Pheochromocytoma
o Rare (< 0,2% of hypertensive)
o Cathecolamine-producing tumor of neuro
chromaffin cells.
o Incidence 3-4th decades
o autosomal dominat hereditary
o malignant 10-15% cases
o Hypertension is caused by excessive plasma level
epinephrine by tumor located unilateral or both
adrenals & anywhere along sympathetic nervus
chain ( 90% adrenal)
 Pheochromocytoma
Symptoms and Signs
Usually lethal unless diagnosed and treated
severe headache, perspiration, palpitation,
anxiety, tremor, tachycardia, severe
hypertension
Attack cyanosis, facial pallor
Classical symptomatic triad : headache,
sweating, palpitations
Laboratory finding
Urinary cathecolamines, metanephrine,
creatinine, urinary VMA
Common Symptoms in Patients with Hypertension Due
to Pheochromocytoma.
Symptoms during or following paroxysms
Headache
Sweating
Forceful heartbeat with or without tachycardia
Anxiety or fear of impending death
Tremor
Fatigue or exhaustion
Nausea and vomiting
Abdominal or chest pain
Visual disturbances
Dyspnea
Symptoms between paroxysms
Increased sweating
Cold hands and feet
Weight loss
Constipation
 Pheochromocytoma
Localization
CT scanning
Overall accuracy 90%-95% for adrenal tumours
Less accurate for extra adrenal tumours
Isotope scintigraphy (MIBG scanning)
131I-MIBG stored in chromaffin granule
Sensitivity 99%
False negative 11%
False positive 2%
Blood and Urine analysis
Plasma catecholamine levels > 1000
micrograms
Urinary VMA and Metanephrine levels
 Pheochromocytoma
Treatment
Surgery
Preoperative preparation
To control hypertension & prevent CVS comp/
Alpha adrenergic blockade
Phenoxybenzamine 10 mg qds 1-2 weeks before
surgery
Beta blockade propanolol 10 mg qds 2-3 days
Intraoperatively
Phentolamine
Sodium nitroprusside
 Pheochromocytoma
Treatment
Laparoscopic removal of the tumor
treatment of choice,
open laparatomy
Prognosis
Depends early diagnosis is made
 ADRENAL
INCIDENTALOMA
ADRENAL INCIDENTALOMAS

Masses found incidentally during radiographic

imaging of the abdomen
Incidence : 0.35-4.36% in general population

In evaluation such mass:

Is the mass benign or malignant ?
Does the mass secrete hormones or mass
disfunction
 ADRENAL INCIDENTALOMAS
Etiology
Benign:
Non hormone secreting
lipoma, cyst, ganglioneuroma, adenoma
Hormone secreting
pheochromocytoma, aldosteronism, subclinical Cushings syndrome
Malignant :
Adrenocortical carcinoma
Metastatic neoplasm
Lymphoma
ADRENAL INCIDENTALOMAS
Size is important:
Adrenal masses > 4cm more likely malignant
surgical resection should be consideration

The great majority (+ 89%) are benign, non

functioning masses

A full biochemical workup should be completed

before surgery is done