Adrenal Mass

Adrenal Masses
Differential Diagnoses and Work-up

Dr. Thuraya AlSumai
Breast and Endocrine Surgical Oncology Fellow
Department of Surgery – KFSH&RC
Adrenal Gland
• Anatomy was first described in 1563 (Eustachius).
• Retroperitoneal organ.
• Located superior and medial to the kidney at the level of
the 11th rib.
• Pyramidal in shape and weighs ~ 4 g.
• Consists of the outer cortex and inner medulla
• Activities are regulation of fluid volume and stress
response
Anatomy
Hormones secreted by cortex
CORTICOSTEROIDS
Glucocorticoids Mineralocorticoids
CORTISOL ALDOSTERON
• immune system • blood pressure
• glucose metabolism • water and salt balance
• stress
Glucocorticoids have important
functions in intermediary
metabolism but also affect
connective tissue, bone, immune,
cardiovascular, renal, and central
nervous systems.
Disorders of the Adrenal
Cortex
Cushing's syndrome
Cushing's syndrome
24-hour urinary free cortisol level

Diagnostic approach in CS
1. Establishing the diagnosis of CS
2. Establishing the cause of CS

a. ACTH-dependent vs independent
b. Identifying the source in ACTH-dependent
3. Imaging
1. Establishing the diagnosis of CS
• 24-hour urinary free cortisol

• Most sensitive test
• Low-dose dexamethasone suppression test
• Most specific test
• 1 mg Dexamethasone at 11 pm, measure plasma cortisol at 8 am.
• Midnight plasma cortisol or late-night salivary cortisol
• Newer tests
2. Establishing the cause of CS
Measure plasma ACTH

 Low < 5 pg/mL Adrenal (ACTH-independent)
 High 15 – 500 pg/mL Pituitary
 Very high > 1000 pg/mL Ectopic ACTH
High Dose Dexamethasone suppression test

 To distinguish between the causes of ACTH-dependent CS
(pituitary vs. ectopic)
 8 mg Dexamethasone then next day 24-hr urinary cortisol
collection.
3. Imaging
ACTH-Independent CT Adrenal protocol or MRI
ACTH-Dependent
 Cushing Disease MRI head
 Ectopic ACTH CT Chest

Treatment of CS
• Cushing Disease  Trans-sphenoidal excision
• Ectopic ACTH Tumors Treat the primary tumor
• Adrenal
• Adenoma  Adrenalectomy
• Hyperplasia Ketoconazole
Conn's syndrome
HYPERTENSION
↓ BLOOD POTASSIUM
• TIREDNESS
• MUSCLE WEAKNESS
• POLYURIA
ADRENAL
ADENOMA
Conn's syndrome
Etiology
• Adrenal adenoma (70%)
• Adrenal hyperplasia (30%)

Conn's syndrome
Diagnosis
• ↓ K < 3.2 mmol/dL
• ↑ Plasma aldosterone
• ↓ Renin
• Ald-Renin ratio 1:25
• Salt-loading suppression test
• Adrenal CT
• Adrenal vein sampling
Conn's syndrome
TREATMENT
• ADENOMA SURGICAL REMOVAL

(UNILATERAL ADRENALECTOMY)
• HYPERPLASIA 
•SPIRINOLACTONE
Hypokalemia > 90%

HTN 70%
Addison’s disease
• WEIGHT LOSS (N/V)
• MUSCLE WEAKNESS
• FATIGUE
• LOW BLOOD PRESSURE
• DARKENING OF SKIN
Addison’s disease
• PRIMARY • SECONDARY
• Destruction of adrenal cortex• Lack of ACTH
- Autoimmune disorders
- Drugs
- Tuberculosis
- Chronic infection - Tumors and
- Metastasis infections of
- Hemmorhage pituitary gland
(Waterhouse-Friderichsen syndrome)
CORTISOL
ALDOSTERONE CORTISOL
Addison’s disease
DIAGNOSIS
Characteristic laboratory findings: hyponatremia, hyperkalemia,
eosinophilia, mild azotemia, and fasting or reactive hypoglycemia.
ACTH STIMULATION TEST
Primary: ↑ ACTH ↓ Cortisol
Secondary: ↓ACTH ↓ Cortisol

Addison’s disease
TREATMENT
REPLACEMENT THERAPY
CORTISOL HYDROCORTISONE
ALDOSTERONE FLUDROCORTISONE
Addison’s disease
ADDISONIAN CRISIS
ACUTE ADRENAL INSUFFICIENCY
- SUDDEN PENETRATING PAIN IN LOWER BACK, ABDOMEN OR LEGS
- SEVERE VOMITING AND DIARRHEA, FOLLOWED BY DEHYDRATION
- LOW BLOOD PRESSURE (mimic sepsis)
- LOSS OF CONSCIOUSNESS
- HYDROCORTISONE i.v.
- 0,9 % NaCl i.v.
- DEXTROSE
Disorders of the Adrenal
Medulla
Pheochromocytoma
• Epidemiology:
 Rare
 25% diagnosed incidentally by imaging
 10% tumor: 10% are bilateral, 10% malignant,
10% occur in pediatric patients, 10% are extra-
adrenal, and 10% are familial.
Occur in MEN IIA & IIB (50%); von Hippel Lindau (14%); type 1
neurofibromatosis; Sturge Weber syndrome.
• Signs/Symptoms:
• Headache, palpitation, diaphoresis “classic triad”
• HTN “MC clinical sign”
• Sustained, episodic, or paroxysmal
• Others: tremors, anxiety, flushing, chest pains,
N/V.
Pheochromocytoma (Diagnosis)
• Labwork:
 Plasma fractionated metanephrines
 “Most sensitive 100%”
 24-hour urine metanephrines
 Most specific 95% (screening)
 x2 sets positive diagnostic
 Clonidine suppression test
 In equivocal cases
 Imaging:
 CT/MRI
 MIBG scan
 F-Dopa scan
Pheochromocytoma (Treatment)
• Pre-operative:
 Adequate hydration
 3-4 days to avoid post-op hypotension
 BP control
 Alpha-blocker phenoxybenzamine
 Beta-blocker propranolol
Beta-blockers should NEVER be started before alpha-
blockers.
• Intra-operative:
• Invasive monitoring (CVC, A-line)
• Minimal tumor manipulation
• Avoid anesthetic stress
• Strict BP control
• Post-operative: HYPOTENSION
• ICU
• Volume resuscitation
The Incidental Adrenal Tumor
• Incidentaloma
• 85% of adrenal masses are nonfunctional and BENIGN
• Def. of incidentaloma:
• >1cm
• Discovered on an exam for a non-adrenal cause
• Absence of signs or symptoms of adrenal disorder
• Up to 5% of CT scans performed for various reasons

reveal adrenal lesions.
Questions to be asked:
• Is the mass functional?
• Any physical signs or symptoms?
• Is there biochemical evidence of activity?
• Pheo screen
• Aldosterone & K level
• Glucocorticoid screen
• Is the mass metastatic?
• Any current or previous history of another
malignancy?
• Is imaging suggestive of malignancy?
• Size, HU, washout
So, what to do with adrenal masses?
• Previously, histopathology was the gold standard
• Currently, various imaging techniques are regarded as
ideal, due to ease:
• CT Adrenal Protocol (83% accurate in diagnosing malignancy)
• Chemical shift MRI (94% accurate)
Benign features:
• Round, smooth contour, homogenous
• Attenuation < 10 HU in non-contrast CT
• Contrast washout > 50% after 10 minutes
• Size < 3 cm
Pheochromocytoma
Adrenocortical Cancer
Biochemical tests
Should be performed in all incidentally discovered
adrenal masses > 1 cm.
• Low dose DST, 24-hr urinary cortisol or salivary cortisol.
• Plasma metanephrines, 24-hour urine metanephrines
• Plasma aldosterone/renin activity & Potassium level
•Negative? Serial follow up

• Serial imaging every 6 months for 2 years
• Biochemical testing yearly for 5 years.

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Adrenal Masses

Differential Diagnoses and Work-up

24-hour urinary free cortisol level

1. Establishing the diagnosis of CS

2. Establishing the cause of CS

• 24-hour urinary free cortisol

2. Establishing the cause of CS

Measure plasma ACTH

High Dose Dexamethasone suppression test

ACTH-Independent CT Adrenal protocol or MRI

 Cushing Disease MRI head

 Ectopic ACTH CT Chest

• Cushing Disease  Trans-sphenoidal excision

• Ectopic ACTH Tumors Treat the primary tumor

• Adrenal hyperplasia (30%)

• ADENOMA SURGICAL REMOVAL

Hypokalemia > 90%

ACTH STIMULATION TEST

Primary: ↑ ACTH ↓ Cortisol

Secondary: ↓ACTH ↓ Cortisol

- SUDDEN PENETRATING PAIN IN LOWER BACK, ABDOMEN OR LEGS

- SEVERE VOMITING AND DIARRHEA, FOLLOWED BY DEHYDRATION

- LOW BLOOD PRESSURE (mimic sepsis)

• Up to 5% of CT scans performed for various reasons

•Negative? Serial follow up

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