Tumor adrenal

dr Putra Hendra SpPD

UNIBA
 Approach to the Adrenal Mass

 Functional atau Non-functional?

 Malignant atau Benign?

 Diagnosis
 Dahulu: histopathology adalah gold standard

 Sekarang: various imaging techniques are

regarded as ideal, due to ease:
 CT with or without contrast (83% accurate in
diagnosing malignancy)
 MRI (94% accurate)
 Scintigraphy (92% accurate)
 Unilateral adrenal masses
Functional Non Functional
 Adrenal adenoma  Adrenal adenoma
 Adrenal cortical  Adrenal cortical
carcinoma. carcinoma.
 Pheochromocytoma  Ganglioneuroma
 Myelolipoma
 Hemorrhage in adrenal
gland.
 Metastases to the adrenal
gland.
 Bilateral adrenal masses
Functional Non Functional

 ACTH dependant cushing  Infection(T.B,Fungus).

syndrome  Infiltrative (amyloidosis
 Congenital adrenal ,leukemia,lymphoma)
hyperplasia.  Hemorrhage.
 pheochromocytoma
 No. (%) of
Diagnosis Lesions

Adenoma 788 (75)

Myelolipoma 68 (6)
Hematoma 47 (4)
Cyst 13 (1)
Pheochromocytoma 3 (0.3)
Macronodular Hyperplasia 1 (0.1)

Adrenal Cortical Neoplasm of unknown

malignant potential 1 (0.1)

Presumed benign by imaging/clinical stability 128 (12)

Total 1049 (100)

 Functional tumors:
 Adrenal Medullary Tumors:
Pheochromocytoma
Childhood tumors: ganglioneuromas,
neuroblastomas
 Adrenal Cortical Tumors:
Cortisol-secreting adenoma
Aldosteronoma
Carcinoma
 ADRENAL GLAND DYSFUNCTION
1. Hypercortisolism= Cushing’s Syndrome
2. Hypocortisolism= Addison’s Disease
3. Conn syndrome
4. Pheochromocytoma
 Hypo- & Hyper- Cushing’s Syndrome
Function of the
Adrenal Cortex
Adrenal Anatomy

From: Bramwell, 1892

Atlas of Clinical Medicine
Addison’s Disease http://www-
•Weakness, fatigue medlib.med.utah.edu/Web
•Unintended weight loss Path/jpeg4/ENDO003.jpg
•Hyperpigmentation
•Hypotension
•Salt craving
•Hypoglycemia
•Nausea, emesis, diarrhea
•Irritability www.bmb.leeds.ac.uk/teaching/
•Depression icu3/ lecture/21/Image82.gif
 Pheochromocytoma
 0.01-0.1% of HTN population
 Found in 0.5% of those screened
 M=F
 3rd to 5th decades of life
 Rare, investigate only if clinically suspicion:
 Signs or Symptoms
 Severe HTN, HTN crisis
 Refractory HTN (> 3 drugs)
 HTN present @ age < 20 or > 50 ?
 Adrenal lesion found on imaging (ex.
Incidentaloma)
FREQUENCY OF VARIOUS DIAGNOSES IN
HYPERTENSIVE PATIENTS – 1980s
PRIMARY CARE REFERRAL
Essential 92-95% 89%
Chronic kidney dis 3-6% 5%
Renovascular dis 0.2-1.0% 4%
Pheochromocytoma 0.1-0.2% 0.2%
Aldosteronism 0.1-0.3% 0.5%
Cushing’s syndrome 0.1-0.2% 0.2%
Coarctation 0.1-0.2% 1%
Oral contraceptives 0.2-1.0%
 Catecholamine Producing Tumors
Neural Crest

Sympathoadrenal Progenitor Cell Neuroblastoma

(Neuroblasts)

Chromaffin Cell Sympathetic Ganglion Cell

Intra-adrenal Extra-adrenal Ganglioneuroma

Pheochromocytoma
 Catecholamines Metabolites
TH MAO, COMT
Tyrosine L-Dopa Dopamine Homovanillic acid
(HVA)
DBH

NorepinephrineCOMT Normetanephrine

PNMT MAO

Epinephrine COMT Metaneprine

Tumor Secretion: MAO
• Large Pheo: more metabolites
(metabolized within tumor before Vanillymandelic Acid
release) (VMA)
• Small Pheo: more catecholamines
• Sporadic Pheo: Norepi > Epi
• Familial Pheo: Epi > Norepi
• Paraganglioma: Norepi
• Cheodectoma, glomus jugulare: Norepi
• Gangioneuroma: Norepi
• Malignant Pheo: Dopamine, HVA
• Neuroblastoma: Dopamine, HVA
 Pheo: Signs & Symptoms
 The five P’s: (5P)
 Pressure (HTN) 90%
 Pain (Headache) 80%
 Perspiration 71%
 Palpitation 64%
 Pallor 42%
 Paroxysms (the sixth P!)
 The Classical Triad: (3P)
 Pain (Headache), Perspiration, Palpitations
 Lack of all 3 virtually excluded diagnosis of pheo in a series
of > 21,0000 patients
 Pheo: Paroxysms,
 10-60 min duration
 Frequency: daily to monthly
 Spontaneous
 Precipitated:
 Diagnostic procedures, I.A. Contrast (I.V. is OK)
 Drugs (opiods, unopposed -blockade, anesthesia induction,
histamine, ACTH, glucagon, metoclopramide)
 Strenuous exercise, movement that increases intra-abdo
pressure (lifting, straining)
 Micturition (bladder paraganlgioma)
 Pheo: Signs (metabolic)
 Hypercalcemia
 Mild glucose intolerance
 Lipolysis
 Weight-loss
 Ketosis > VLDL synthesis (TG)
 Diagnosis
 Plasma free metanephrines
 most sensitive test
 seen 99% of patients

 24° urinary catecholamines

 (2x normal is diagnostic)
 VMA
 Clonidine suppression test
 24h Urine Collection
 24h urine collection:
 Creatinine,catecholamines, metanephrines,
vanillymandelic acid (VMA), +/-dopamine
 HPLC with electrochemical detection or mass spect

 Positive results (> 2-3 fold elevation):

 24h Ucatechols > 2-fold elevation
 ULN for total catechols 591-890 nmol/d
 24h Utotal metanephrines > 1.2 ug/d (6.5 umol/d)
 24h UVMA > 3-fold elevation
 ULN 35 umol/d for most assays
 Suppression/Stimulation Testing
 Clonidine suppression
 May precipitate hypotensive shock!
 Unlike normals, pheo patients won’t suppress their
plasma norepi with clonidine
 Glucagon stimulation
 May precipitate hypertensive crisis!
 Pheo patients, but not normals, will have a > 3x
increase in plasma norepi with glucagon
 MIBG Scan
 123I or 131I labelled metaiodobenzylguanidine
 MIBG catecholamine precurosr taken up by the
tumor
 Inject MIBG, scan @ 24h, 48h, 72h
 Lugol’s 1 gtt tid x 9d (from 2d prior until 7d after
MIBG injection to protect thyroid)
 False negative scan:
 Drugs: Labetalol, reserpine, TCAs, phenothiazines
 Must hold these medications for 4-6 wk prior to scan
 Pheo Management
 Prior to 1951, reported mortality for excision of
pheochromoyctoma 24 - 50 %
 HTN crisis, arrhythmia, MI, stroke
 Hypotensive shock
 Currently, mortality: 0 - 2.7 %
 Preoperative preperation, -blockade?
 New anesthetic techniques?
 Anesthetic agents
 Intraoperative monitoring: arterial line, EKG monitor, CVP
line, Swan-Ganz
 Experienced & Coordinated team:
 Endocrinologist, Anesthesiologist and Surgeon
 Hyperadrenalisms
Adrenal cortex
Primary Hyperaldosteronism
Primary hyperaldosteronism – excess aldosterone secretion
which is independent of the renin-angiotensin system
(Conn’s syndrome)
Causes:
Aldosterone secreting adenoma
Bilateral hyperplasia of the cortex
Rarely carcinoma
Clinical features:
Hypertension, hypokalemia, sodium retention, muscle weakness,
paraesthesia, ECG changes, cardiac decompensation
Aldosterone is primarily involved with fluid
and electrolyte balance.

Aldosterone secretion causes sodium

reabsorption in the distal renal tubule in
exchange for potassium and hydrogen ions.

The net effects are, fluid retention, decrease

in plasma potassium and metabolic
alkalosis.
 Therapy
 If tumor with lateralization,
laparascopic adrenalectomy
 If tumor without lateralization
(incidentaloma), or hyperplasia, then
aldosterone blockade
Spironolactone
Eplerinone
 Adrenal Cortical Carcinoma:
the sex hormones

 Epidemiologi:
 Rare: 0.02% of cancers, 0.3 cases/million worldwide
 F > M; two age peaks: <5yo & 40-50s
 Signs/Symptoms:
 Androgen excess is most common  virilization
 Cushing’s syndrome also seen

 Labwork demonstrates:
 Elevated DHEA-sulfate and androstendedione
 Failed dex suppression
 Cortisol-secreting Adenoma:
the sweet part

 Epidemiologi: F > M; ages 30-50s

 Signs/Symptoms:
 May lead to clinical or sub-clinical Cushing’s syndrome
 Most commonly: obesity, HTN, glucose intolerance or
DMII, hypercholesterolemia
 Labwork demonstrates:
 Low DHEA-sulfate (increased cortisol suppresses
pituitary-adrenal axis)
 Elevated 24 hour urinary cortisol