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ADRENALS
https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

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ANATOMY

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PHYSIOLOGY

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PHYSIOLOGY
• Cholesterol – common precursor of all steroid hormones derived from
the adrenal cortex
• Mineralocorticoids
1. Aldosterone – regulated by renin-angiotensin system
2. 11 – deoxycorticosterone (DOC)
3. Cortisol – major adrenal glucocorticoid

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PHYSIOLOGY
• Adrenal androgen (sex steroids)
1. Dehydroepiandrosterone (DHEA)
Dehydroepiandrosterone (DHEAS)
2. Androstenedione
3. testosterone and estrogen – in small amount
• Catecholamines
1. Epinephrine
2. Norepinephrine
3. Dopamine

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DISORDER OF THE ADRENAL CORTEX
• Primary Hyperaldosteronism
autonomous aldosterone secretion
suppression of renin secretion

- solitary functioning adrenal adenoma (~70%)
idiopathic bilateral hyperplasia (~30%)

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Signs and symptoms

- typically presents with hypertension: long standing, moderate to
severe, may be difficult to control despite multiple-drug therapy
- headaches, polydipsia, polyuria, nocturia, muscle weaknesand
fatigue (hypokalemia)

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Laboratory studies – spontaneous hypokalemia (K <3.2mmol/L)
hypokalemia on diuretic therapy (K<3mmol/L)
Radiologic studies – CT scans with 0.5cm cuts

MRI scans
Scintigraphy with 131I-6β-iodomethyl
noriodocholesterol (NP-59)

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Treatment
- Preoperative control of hypertension and adequate potassium
supplementation (keep K>3.5mmol/L)
- unilateral tumors producing aldosterone are best managed by
ADRENALECTOMY
- >90% successful in improving hypokalemia and 70% succesful in
correcting hypertension

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• Cushing’s Syndrome
- complex of symptoms and signs resulting from ypersecretion of
cortisol regardless of etiology
Cushing’s Disease
- refers to a pituitary tumor, usually an adenoma, which leads to
bilateral adrenal hyperplasia and hypercortisolism

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Signs and Symptoms

- Progressive truncal obesity is the most common (95%)

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Diagnostic Test:
Laboratory studies
- characterized by elevated glucocorticoid levels that are not
suppressible by exogenous hormone administration and loss of diurnal
variation
- Overnight low-dose dexamethasone suppression test
normal adults suppress cortisol levels to <3µg/dl
Radiologic studies – Ctscan and MRI can identify adrenal tumor 95%
sensitivity

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Treatment:
Laparoscopic adrenalectomy – treatment of choice for patients with
adrenal adenomas
Open adrenalectomy – for patients with large tumors (>6cm) or those
suspected to be adrenocortical cancers
Transphenoidal excision of pituitary adenoma – treatment of choice
in Cushing’s Disease; successful in 80% of patients
- Pituitary irradiation has been used for patients with persistent or
reccurent disease after surgery

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• Sex Steroids Excess
- Adrenal adenomas or carcinomas that secrete adrenal androgens
leads to virilizing syndromes
- women develop hirsutism, amenorrhea, infertility, increased muscle

mass, deepend voice, temporal baldness

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• Sex Steroids Excess
Diagnostic Test
- virilizing tumors produce excessive amounts of androgen precurser,
DHEA, which can be measure in plasma and urine 17-ketosteroids
Treatment
- Adrenalectomy

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• Congenital adrenal hyperplasia (CAH)
- group of disorders that result from deficiencies or complete absence of
enzymes involved in adrenal steroidogenesis
- 21-hydoroxylase (CYP21A2) deficiency is the most common (90%)

lead to virilization
- 11β-hydroxylase deficiency is the second most common
lead to hypertension, virilization and hyperpigmentation
- Congenital adrenal lipoid hyperplasia is the most severe form,
caused by cholesterol desmolase deficiency
result in fatal salt-wasting syndrome in female patients

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• Congenital adrenal hyperplasia (CAH)
Diagnostic Test
- karyotype analysis and measurement of plasma and urinary steroids
Treatment
- traditionally managed medically with Cortisol and mineralocorticoid
replacement
- recently, bilateral laparoscopic adrenalectomy has been proposed as
an alternative treatment

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DISORDERS OF THE ADRENAL
MEDULLA
• Pheochromocytomas
- rare tumors with prevalence rates from 0.3% to 0.95%
- often called the “10 percent tumor”

10% bilateral, 10% malignant, 10% occur in pediatric patients
10% are extra-adrenal and 10% are familial
-occur in families with MEN2A and MEN2B in 50% of patients

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MEDULLA
Signs and Symptoms

- headache, palpitations and diaphoresis (classic triad)
- nonspecific symptoms: anxiety, tremulousness, paresthesia,
flushing, chest pain, shortness of breath, abdominal pain, nausea and
vomiting
- most common clinical sign is hypertension

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MEDULLA
Diagnostic test
Biochemical studies
- diagnosed by testing 24-hour urine samples for catecholamines and
their metabolites
- determining plasma meatanerphrine levels
- urinary metanerphines are 98% sensitive and 98% specific

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MEDULLA
Diagnostic test
Radiologic studies
- Ctscans are 85% to 95% sensitive and 70% to 100% specific;
should be performed without contrast
- MRI scans are 95% sensitive and almost 100% specific; study of
choice in pregnant women
-131I-radiolabeled MIBG is useful for localizing
pheochromocytomas especially in ectopic positions

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MEDULLA
Treatment
- medical management is aimed chiefly at blood pressure control
and volume repletion preoperatively
- Adrenalectomy is the treatment of choice; the chief goal of
surgery is to resect the tumor completely with minimal tumor
manipulation or rupture of the tumor capsule
- usually performed via an open anterior approach to facilitae
detection of bilater tumors, extra-adrenal lesions or metastatic lesions

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MEDULLA
• Hereditary Pheochromocytomas
- tumor tend to be mutiple and bilateral
- unilateral adrenalectomy is recommended in the absence of

obvious lesions in the contralateral adrenal gland
- patients with tumors in both adrenal glands, cortical-sparing

subtotal adrenalectomy may preserve adrenocortical function and
avoid the morbidity of bilateral total adrenalectomy

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MEDULLA
• Malignant Pheochromocytomas
- approximately 12% to 29% of pheochromocytomas are malignant
and are associated with decreased survival
- no definitive histologic criteria defining malignant

pheochromocytomas
- malignancy is diagnosed when there is evidence of invasion into

surrounding structures or distant metastasis

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MEDULLA
• Malignant Pheochromocytomas
- in general, soft tissue lesions are treated with resection if feasible
- external-beam radiation can be used for unresectable lesions or

symptomatic skeletal metastases
- therapeutic 131I-MIBG irradiation may be useful in patient with

diffuse disease showing uptake on a diagnostic scan

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ADRENAL INSUFFICIENCY
• PRIMARY Adrenal Insufficiency - resulting from adrenal disease
• SECONDARY Adrenal Insufficiency - due to deficiency of ACTH

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• Signs and Symptoms
Acute Adrenal Insufficiency
- suspected in stressed patients with any of relevant risk
- may mimic sepsis, M.I., or pulmonary embulos and presents with
fever, weakness, confusion, N&V, lethargy abdominal pain or severe
hypotension
Chronic Adrenal Insufficiency
- subtle symptoms: fatigue, salt craving, weight loss, nausea,
vomiting and abdominal pain

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Diagnostic studies
- characteristic lab findings include hyponatremia, hyperkalemia,
eosinophilia, mild azotemia and fasting or reactive hypoglycemia
- diagnosed by ACTH stimulation test: ACTH (250µg) is infused IV

and cortisol levels are measured at 0, 30 and 60 mins.
- peak cortisol levels <20µg/dL suggest adrenal insufficiency

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Treatment
- treatment must be inititiated based on clinical suspicion alone,
even before test results are obtained or the patient is unlikely to
survive
- volume resuscitation with at least 2 to 3L of a 0.9% saline solution
or 5% dextrose in saline solution
- dexamethasone (4mg) should be administered IV
- once patient has stabilized, infection should be sought, identified
and treated

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Thank you

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ADRENALS

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suppression of renin secretion

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Signs and symptoms

https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

Radiologic studies – CT scans with 0.5cm cuts

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https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

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Signs and Symptoms

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https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

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- women develop hirsutism, amenorrhea, infertility, increased muscle

https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

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- 21-hydoroxylase (CYP21A2) deficiency is the most common (90%)

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https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

- often called the “10 percent tumor”

-occur in families with MEN2A and MEN2B in 50% of patients

https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

Signs and Symptoms

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- unilateral adrenalectomy is recommended in the absence of

- patients with tumors in both adrenal glands, cortical-sparing

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- no definitive histologic criteria defining malignant

- malignancy is diagnosed when there is evidence of invasion into

https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

- external-beam radiation can be used for unresectable lesions or

- therapeutic 131I-MIBG irradiation may be useful in patient with

https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

• PRIMARY Adrenal Insufficiency - resulting from adrenal disease

• SECONDARY Adrenal Insufficiency - due to deficiency of ACTH

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- diagnosed by ACTH stimulation test: ACTH (250µg) is infused IV

https://lookaside.fbsbx.com/file/ADRENALS.pptx?token=AWwrqc0EVEhvRmzDFPxQRbOXpO9ok…RSecp-s6ct71zSWObbqPi0zjd5bZiAUWeQTIvC03wowlW5H54vpb35NC7fhuopGHIniufnPc2cTkQ 04/01/19, 3`42 PM

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