Dr. Samuel L.
1
ADRENALMASS
Presenter: Dr. Samuel L. (GSRIII)
Moderator: Dr. Endale A.
Consultant General & Endocrine Surgeon
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08/17/2025
Dr. Samuel L.
2OUTLINE OF PRESENTATION
Brief embryology, anatomy & physiology
Hyperaldosteronism
Cushing syndrome
Pheochromocytoma
Adrenocortical cancer
Incidentaloma
Miscellaneous adrenal masses
Techniques of adrenalectomy
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Dr. Samuel L.
3OBJECTIVES
After the end of this session, we will be able to know
the differential diagnoses of adrenal masses
different diagnostic & management algorithms to each disease entity
techniques of adrenalectomy
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Dr. Samuel L.
4EMBRYOLOGY
The cortex originates around the 5th
week of gestation from mesodermal
tissue near the gonads
The cortex differentiates further into a thin, definitive cortex and a thicker,
inner fetal cortex
Zona reticularis is formed after birth at around 3 years of age
Medulla is ectodermal in origin and arises from the neural crest cells
During fetal development the migration of adrenocortical and medullary
cells can result in accessory or ectopic tissue
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5
ANATOMY
Paired retroperitoneal organs
Weigh 4-5g each
Arterial supply
Superior adrenal aa- from inf phrenic aa
Middle adrenal aa- from abd aorta
Inferior adrenal aa- from renal aa
Venous return
Rt- short & drains directly to IVC
Lt- into Lt renal vv
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Dr. Samuel L.
8PRIMARY HYPERALDOSTERONISM
Hypertension, hypokalemia, and alkalosis
From a treatment standpoint, the subtypes of primary hyperaldosteronism
thus can be divided into two groups:
Surgically manageable
Medically treated
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9Epidemiology
M:F- 1:2
30-50 yrs
5-13 % of hypertensive pts;
20% in those with resistant HTN
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10Clinical & pathologic Features
Clinical features
Hypertension with or without hypokalemia
HTN is moderate to severe and resistant to medical mx
Headache, fatigue, muscle weakness…
Peripheral edema is rare due to ‘aldosterone escape’
Significant cardiac and metabolic alterations
Pathologic features
Solitary, unilateral , and small (<2cm)
Characteristic golden yellow appearance
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Diagnosis
Elevated aldosterone with suppressed renin levels
Elevated PAC levels in combination with PAC:PRA ratio
Saline suppression test
Failure to suppress PAC below 10 ng/ml and Urinary aldosterone
secretion greater than > 12 ug/24hr with urinary Na excretion more
than 200 mEq /24hr
PAC:PRA > 20-30 in the setting of PAC >15-20 ng/dl
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12Localization
CT - a unilateral 1- to 2-cm hypo dense lesion with a normal contralateral
adrenal gland suggests aldosteronoma
MRI
NP-59 Scan
Adrenal venous sampling
First confirm proper cannulation
Then compare aldosterone levels
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Dr. Samuel L.
14TREATMENT
SURGICAL- laparoscopic Vs Open
Pre-op rx with spironolactone reduces surgical risks
Pre-op ARBs also reduce risk of postop hypoaldosteronism
Medical
Also in poor surgical candidates
Spironolactone, eplerenone
Dexamethasone- for glucocorticoid suppressible
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15POST-OP OUTCOMES
K levels normalizes in 95% pts immediately
HTN improves in 75% within a month
1/3 will no longer require medication
2/3 require reduced dose
There is association between increased age and longer duration of HTN
with persistent post-op HTN
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17
CUSHINGSYNDROME
Cushing’s syndrome refers to a complex
of symptoms and signs resulting from
hypersecretion of cortisol regardless of
etiology.
Cushing’s disease refers to a pituitary
tumor, usually an adenoma, which
leads to bilateral adrenal hyperplasia
and hypercortisolism
• Rare disease (10/1mil)
• M:F ratio is 1:8
• Most are sporadic
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19LAB STUDIES
SCREENING
Overnight dexamethasone suppression test
48 hr./low-dose dexamethasone suppression test
Late-night salivary cortisol measurement
Urinary cortisol
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20EVALUATION OF THE UNDERLYING
CAUSES
Plasma ACTH
ACTH less than 2 pmol/l (10 pg/ml) indicate ACTH-independent Cushing’s
syndrome
ACTH greater than 4 pmol/l (20 pg/ml)
High dose dexamethasone suppression
Pituitary MRI
Bilateral petrosal vein sampling
Chest and abdominal CT
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Dr. Samuel L.
22Management
MEDICAL
For perioperative control of hypercortisolism or
When surgery is not feasible
Metyrapone, ketoconazole, and mitotane
SURGERY
Pituitary adenoma- TSS, *bilateral laparoscopic adrenalectomy
Ectopic TSH secreting tumors-resection, *medical therapy or *bilateral
adrenalectomy
Adrenal causes- adrenalectomy (unilateral or bilateral; open or laparoscopic)
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23PHEOCHROMOCYTOMA
Arise from the cells of the adrenal medulla
MEN2A, MEN2B, VHL disease, NF1
10 percent tumor
peak incidence in the fourth and fifth decades
no gender predilection
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24Clinical Features
Classic triad of headaches, palpitations, and extreme hypertension
Paroxysmal spells
In 0.1-0.2 % of hypertensive pts
Myocardial ischemia
Raynaud’s type phenomenon
Other sxs : nausea, lassitude, heat intolerance, anxiety, abdominal pain,
pallor, fever, or glucose intolerance
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25Diagnosis
Identification of the classic signs
24 hr. urine metanephrines and fractionated catecholamines
Plasma free metanephrines
Localization
CT of abdomen & Pelvis-heterogeneous, central necrosis
MRI- hyper intense ‘bright white’ signal on T2
MIBG scan-
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26Pre-op management
Alpha blockage- phenoxybenzamine
For a minimum of 1-3 wks
Orthostatic hypotension & ‘stuffy nose’
Ca2+ channel blockers- nicardipine
Volume expansion- fluids, high salt diet
Beta blocker- as needed
Metyrosin - in refractory HTN/ Tachycardia
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27Intra-op mx
The most volatile BP swings occur with induction of anesthesia
With occlusion of the adrenal vein, the pt may rapidly vasodilate
Excellent communication should be maintained b/n the surgeon &
anesthesiologist
Physical manipulation of the tumor should be minimized
RX- Adrenalectomy
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28Special populations
Pregnant
Supine HTN
MRI is the best imaging choice
Follow with meds until safe GA for
delivery
CS with tumor excision
Children
HTN is more often sustained
Tendency to be bilateral & extra-
adrenal
Malignancy is less frequent
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29Malignant Pheochromocytoma
There are no definitive histologic criteria defining malignant
pheochromocytomas
Malignancy is usually diagnosed with evidence of invasion into surrounding
structures or distant metastases
Mets: to bone, liver, regional lymph nodes, lung, and peritoneum
Treatment
Resection if feasible
External-beam radiation for un resectable lesions
Therapeutic 131 I-MIBG irradiation
Chemotherapy
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30Adrenocortical cancer
Rare neoplasms (2 per 1 million in the world)
Bimodal age distribution
The majority are sporadic
~ 50% of adrenocortical cancers are nonfunctioning
Functional tumors secrete cortisol (30%), androgens (20%), estrogens (10%),
aldosterone (2%); or multiple hormones (35%)
CF: rapid onset of Cushing’s syndrome accompanied by virilizing features
Enlarging abdominal mass, pain
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31Adrenocortical cancer
DIAGNOSTIC TESTS
Lab tests
CT/MRI:
tumor heterogeneity, irregular margins, and the presence of hemorrhage and
adjacent LAP or liver metastases
moderately bright signal intensity on T2-weighted images
significant lesion enhancement, and slow contrast washout
evidence of local invasion into adjacent structures
CT of chest & pelvis: staging
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Adrenocorticalcancer
Pathology : criteria to
predict malignancy
nuclear grade III or IV;
mitotic rate greater than 5 per 50 high-
power fields;
atypical mitoses;
clear cells comprising 25% or less of the
tumor;
a diffuse architecture;
microscopic necrosis;
invasion of venous, sinusoidal, and capsular
structure
Tumors with four or more of these criteria were
likely to metastasize and/or recur
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34Treatment
SURGERY
Open adrenalectomy is the procedure of choice
R0 resection must be the goal
Enbloc resection with contiguous structures
Surgical debulking
CHEMOTHERAPY
Etoposide, cisplatin, doxorubicin, paclitaxel +_ mitotane
RADIOTHERAPY –incomplete resection, palliation of bone mets
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35INCIDENTALOMA
These are adrenal lesions discovered during imaging performed for
unrelated reasons
Frequency: 5.9% of autopsies & 4.3 % from images
Although non-functional by definition , most of them may be sub clinically
functioning
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37Screening for subclinical disease
Subclinical Cushing’s Syndrome
Ranges from slightly attenuated diurnal cortisol rhythm to atrophy of the
contralateral gland
Do dexamethasone suppression test
Subclinical Pheochromocytoma
Refers to totally asymptomatic adrenal incidentaloma that histologically proves
to be pheochromocytoma
24 hr urine metanephrine & VMA, or fractionated urinary catecholamine
Subclinical Primary Aldosteronism
Pt with adrenaloma who is normotensive or hypertensive with normokalemia
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38Screening for subclinical disease
Screening for adrenal Ca
4-25 % harbor Ca
Imaging findings
CT-ragged tumor with stippled calcifications and with areas of necrosis; enlarged LN or
local invasion
MRI-heterogeneously increased, early T2-weighted signal, weak and late enhancement
after gadolinium
FNAB has limited role
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39Management: Surgery Vs Follow-up
• Surgery
Lab evidence for sub
clinically functioning
tumor
Associated metabolic
features
Evidence of primary
or solitary metastatic ca
• Follow-up: yearly for 5-10
yrs.
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40
Miscellaneousadrenal masses
ADRENAL METASTASES
From the lung , melanoma, breast, kidney, and GI neoplasms
~50% are functional
Appropriate biochemical evaluation prior to further imaging, bx or rx
Imaging: CT with contrast, MRI
The definitive dx of malignancy requires percutaneous bx or surgical
pathology
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41Management of adrenal metastases
Appropriate mx
depends on:
Type & extent of
primary malignancy
DFI
Patient comorbidity
Resection of isolated
adrenal metastases
may offer a survival
benefit.
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42Miscellaneous adrenal masses
ADRENAL HEMORRHAGE
Causes
trauma, surgery, anticoagulant rx,
septicemia or tumor
Increased ACTH
Adrenal medullar venous
thrombosis
ADRENAL CYSTS
Benign or malignant(7%)
Endothelial cysts, epithelial cysts
Pseudocysts can arise from
hemorrhage
Small, asymptomatic or non-
functioning- can be followed
Size >6cm, symptomatic,
functioning or suspected
malignancy- surgery
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43Miscellaneous adrenal masses
Lymphangioma
Pseudocysts
Paracytic infections
Myolipoma
Surgery
Symptomatic
Suspicion of malignancy
Hydatid cyst
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44Techniques of adrenalectomy
OPEN Vs LAPAROSCOPIC
OPEN ADRENALECTOMY
Anterior approach
Posterior approach
Lateral approach
Thoraco -abdominal approach
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Dr. Samuel L.
46ANTERIOR APPROACH
• Midline incision or bilateral subcostal Incision
• Access to Rt adrenal
• Mobilize the liver medially; reflect hepatic
flexure of the colon
• Kocher maneuver & exposure of RP
• Gerota’s fascia is incised
• Mobilize the sup & lat surfaces first
• Divide the adrenal vein
• Access to Lt adrenal
• Medial visceral rotation
• Via lesser sac by division of the gastrocolic
ligament
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47Complications of adrenalectomy
Infection
Bleeding
Hemodynamic instability
Adrenal insufficiency
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48Postoperative care
Depends on the operative approach, extent of resection, and functional or nonfunctional nature of the
tumor
Pheochromocytoma
ICU for a minimum of 4 hrs
a-blockers: discontinue
B-blockers- if started pre-op, it should be continued & weaned post-op
Aldosteronoma
DC all antihypertensive meds except b-blockers
DC spironolactone
Check serum K the next morning
Follow BP as an outpatient
Cushing’s syndrome
Continue glucocorticoids with subsequent dose tapering
Mineralocorticoid supplementation after bilateral adrenalectomy
#7 Catecholamines are cleared by several mechanisms :
reuptake by sympathetic nerve endings,
peripheral inactivation by COMT & MAO
direct excretion by the kidneys
#8 Primary hyperaldosteronism -autonomous adrenal hypersecretion of aldosterone
Secondary hyperaldosteronism-the adrenal glands function normally, and increased plasma renin levels stimulate the hypersecretion of aldosterone
#10 Aldosterone-secreting adrenocortical carcinoma should be suspected in a unilateral tumor larger than 4 cm
#11 Certain medications (spironolactone, diuretics, ACE, Estrogen …) should be discontinued 4-6 wks prior to the test. And control of HTN can be achieved by a-blockers.
#12 Unilateral aldosteronoma : younger age, more severe HTN, more profound hypokalemia, higher urine & plasma aldosterone.
Unaffected by Renin- ang feedback, sensitive to ACTH
Vs idiopathic hyperaldosteronism
Enhanced sensitivity to small changes in Renin- ang system & unaffected by ACTH
#18 Signs of protein wasting including the presence of thin skin in the young, easy bruising, and proximal muscle weakness could more reliably distinguish Cushing’s from metabolic syndrome
#19 A normal value of urine cortisol (<135 nmol /24 h) excludes the diagnosis of Cushing’s syndrome
#20 A suppression of cortisol of more than 50% of basal level implies a positive test result
#21 A suppression of cortisol of more than 50% of basal level implies a positive test result.
#22 Nelson’s syndrome – a locally aggressive pituitary tumor that secretes high concentrations of ACTH (usually>300 pg/ml) and results in skin pigmentation
after bilateral adrenalectomy in patients with Cushing’s disease
Steroid supplementation- lifelong or for 6-12 mo after bilateral and unilateral adrenalectomy respectively
#23 10% are bilateral, 10% are malignant, 10% occur in pediatric patients, 10% are extra-adrenal, and 10% are familial.
#24 The timing of the paroxysmal spells varies greatly from cycling as frequently as every 7–15 min to daily or weekly episodes
These episodes may be initiated by nearly any mechanism that applies pressure to the tumor
Invasive procedures such as diagnostic needle biopsy, angiography, general anesthesia, and unrelated surgical procedures
#25 The diagnosis of malignancy in the absence of distant disease is determined intraoperatively by assessment of tumor invasion
7.6 cm Vs 5.3 cm
#26 Metyrosin-inhibitor of tyrosine hydroxylase, which prevents the conversion of l-tyrosine into l- dopa
Selective a blockers- better tolerated; for long term therapy like in metastatic pheochromocytoma
#29 Malignant pheochromocytomas are more likely to express p53 and bcl-2 and have activated telomerase
Expression of Ki-67, and COX-2
#31 The sensitivity, specificity, and likelihood ratio of tumor size in predicting malignancy were reported as 96%, 51%, and 2 for tumors ≥4 cm, and 90%, 78%, and 4.1 for tumors ≥6 cm
#34 Patients who undergo complete resection have 5-year actual survival rates ranging from 32% to 48%, whereas median survival is <1 year in those undergoing incomplete excision
#35 Adrenaloma: adrenal lesion which is detected accidentally or not; without biochemical evidence or sxs suggestive of cortical or medullary secretion or sxs of malignant disease
#38 FNAB- It is useful in cases of coexistent extra-adrenal malignancy like lung Ca
-can’t differentiate cortical adenoma from Ca
#40 CT- look for size, shape, contour, invasion of adjacent structures
- attenuation
- rate of contrast washout
#46 In addition to the adrenal vein, large and/or invasive tumors may have multiple large parasitic blood vessels that also need to be ligated