5. Anatomy
• T11
• 4 – 5 g
• 5 x 3 x 1 cm
• Yellow
• 3 zones: glomerulosa, fasciculata, reticularis
• GFR = ACS
6. Physiology
• Cholesterol = common precursor of all steroid
hormones derived from adrenal cortex
• Mineralocorticoids: aldosterone
• Glucocorticoids: cortisol
• Sex hormones: adrenal androgen
• Catecholamines: epinephrine, norepinephrine,
dopamine
7. Physiology
• Aldosterone
– Regulated primarily by renin-angiotensin system
and hyperkalemia
– Circulates in plasma chiefly as a complex with
albumin
8. Physiology
• Cortisol
– Regulated by ACTH (secreted by anterior pituitary
gland which under to control of corticotrophin
releasing hormone (CRH) secreted by
hypothalamus)
– Peak secretion in early morning and lowest level in
the evening
9.
10. Physiology
• Androgen
– Peripheral conversion to testosterone and
dihydrotestosterone
– Promote the formation of male genitalia in fetal
development
– Responsible for secondary male characteristics
– Excess lead to precocious puberty, virilization,
acne, hirsutism
16. Disorders of Adrenal Cortex
• Hyperaldosteronism
• Cushing’s syndrome
• Sex steroid excess
• Congenital adrenal hyperplasia
• Adrenocortical cancer
17. Hyperaldosteronism
• primary hyperaldosteronism = Conn’s
syndrome
• Between age 30-50 years old
• Hypertension and hypokalemia
• Most results from
– Solitary functioning adrenal adenoma
– Idiopathic bilateral hyperplasia
18. Hyperaldosteronism
• Symptoms and signs
– Hypertension difficult to control despite multiple-drug
therapy
– Hypokalemia
– Muscle weakness
– Polydipsia
– Polyuria
– Nocturia
– Headache
– fatigue
19. Hyperaldosteronism
• Laboratory test
– K < 3.2, or < 3 while on diuretic therapy
– Elevated plasma aldosterone level
– Suppressed plasma renin activity
– Fail to suppress aldosterone level with sodium
loading
20. Hyperaldosteronism
• Radiologic test
– CT
– MRI
– Selective venous catheterization and adrenal vein
sampling for aldosterone
– Scintigraphy with I131-6β-idomethyl
noriodocholesterol (NP-59)
21.
22. Hyperaldosteronism
• Treatment
– Adrenalectomy for unilateral adrenal tumor
– Preop control of blood pressure and K
– Medical: spironolactone, amiloride, triamterene,
captopril
– Dexamethasone for glucocorticoid-suppressible
hyperaldosteronism
30. Adrenocortical Cancer
• Associated with mutation of p53 (Li-Fraumeni
syndrome, MEN1, 11p (Beckwith-Wiedemann
syndrome, 2p (Carney complex), 9q
• 50% are nonfunctioning present with
abdominal mass and abdominal or back pain
• Commonly metastasize to liver, lung, bone
31. Adrenocortical Cancer
• Diagnostic tests
– Serum electrolyte to rule out hypokalemia
– Urinary catecholamines to rule out
pheochromocytoma
– DST and urine cortisol to rule out Cushing’s
syndrome
– CT
– MRI
32. Adrenocortical Cancer
• CT characteristics
– Tumor heterogeneity
– Irregular margins
– Presence of hemorrhage
– Adjacent lymphadenopathy
– Lever metastasis
33. Adrenocortical Cancer
• Pathology
– Gross exam: large, hemorrhage, necrosis
– Capsular or vascular invasion: most reliable sign of
cancer
– Hyperchromatic and large and prominent nuclei
34. Adrenocortical Cancer
• Weiss’s criteria to distinguish malignant from
benign 4 or more likely to recur/metastasize
– Nuclear grade III, IV
– Mitotic > 5/50 HPF
– Atypical mitoses
– Clear cells comprising 25% or less of the tumor
– A diffuse architecture
– Microscopic necrosis
– Invasion of venous, sinusoidal, and capsular structure
35. Adrenocortical Cancer
• Treatment
– Most important predictor of survival: adequacy of
resection
– Isolated recurrent disease: surgical debulking
– Chemotherapy: etoposide, cisplatin, doxorubicin,
paclitaxel
36.
37. Sex Steroid Excess
• Adrenal androgen virilizing syndrome
• Feminizing adrenal tumor: less common
• Diagnostic test: elevated DHEA, elevated 17-
ketosteroids
• Treatment: adrenalectomy
38. Congenital Adrenal Hyperplasia
• Deficiencies of enzyme involved in adrenal
steroidogenesis especially 21-hydroxylase
(most common)
• Deficiency of glucocorticoids and aldosterone
lead to overproduction of adrenal androgens
• Presents at birth with: virilization, diarrhea,
hypovolemia, hyponatremia, hyperkalemia,
hyperpigmentation
40. Congenital Adrenal Hyperplasia
• Treatment
– Cortisol and mineralocorticoid replacement:
steroid required are often supraphysiologic and
lead to iatrogenic hypercortisolism
– Alternatively, bilateral laparoscopic adrenalectomy
42. Pheochromocytoma
• 40-50 years old
• Extra-adrenal tumors (functional
paraganglioma) may be found at sites of
sympathetic ganglia in the organ of
Zuckerkandl, neck, mediatinum, abdomen,
and pelvis
43. Pheochromocytoma
• Associated with MEN2A and MEN2B, VHL
disease, NF1 gene,
• mutations of SDHB, SDHC, SDHD gene
• 10 percent tumor
– 10% bilateral
– 10% malignant
– 10% occur in children
– 10% extra-adrenal
– 10% familial
44. Pheochromocytoma
• Symptoms and signs
– Classic triad: headache, palpitation, diaphoresis
– Most common classical sign: hypertension
– Anxiety, tremulousness, paresthesia, flushing,
chest pain, shortness of breath, abdominal pain,
nausea, vomiting
– MI, CVA
45. Pheochromocytoma
• Diagnostic Tests
– 24-hour urine samples for catecholamines
– Plasma metanephrine level
– Urinary metanephrine level
– VMA are slightly less sensitive and specific
– CT and MRI: include diaphragm to aortic
bifurcation
– MIBG scan: localize pheochromocytoma especially
ectopic position
47. Pheochromocytoma
• Hereditary pheochromocytoma
– Multiple and bilateral
– Treatment:
• Cortical-sparing subtotal adrenalectomy: risk to
recurrence
• Autotransplantation after total adrenalectomy
48. • Malignant pheochromocytoma
– Usually diagnosed when invasion surrounding
structures or distant metastasis
– Most common sites of metastasis: bone, liver,
regional lymph nodes, lung, and peritoneum
– Decreased survival
Pheochromocytoma
49. Adrenal Incidentaloma
• Adrenal lesions discovered during imaging
performed for unrelated reasons
• Incidence from CT scan: 0.4-4.4%
• The adrenal in common site of metastasis of
lung, breast, melanoma, RCC, lymphoma
50.
51.
52. Adrenal Insufficiency
• Most common Primary AI are autoimmune
disease, infection, and metastatic deposits
• Most common secondary AI is exogenous
steroid therapy
53.
54. Adrenal Insufficiency
• Acute
– In stressed patients
– Severe hypotension, fever, weakness, confusion,
nausea, vomiting, lethargy, abdominal pain
• Chronic
– In patients with metastatic tumor
– Subtle: fatigue, weight loss, hyperpigmented
55. Adrenal Insufficiency
• Diagnostic studies:
– hypoNa, hyperK, eosinophilia, hypoglycemia
– ACTH stimulation test cortisol level < 20
mcg/dL suggest AI
– ACTH level to exclude secondary cause
– High ACTH + low plasma cortisol = primary AI
56. Adrenal Insufficiency
• Treatment
– Volume resuscitation
– Blood sampling for electrolyte, glucose, and
cortisol
– Dexamethasone 4 mg IV, hydrocortisone 100 mg
IV q8hr
– Treat underlying cause
58. Choices
• Laparoscopic or open
• Gland may be approached anteriorly, laterally,
or posteriorly via retroperitoneum
• Laparoscopic when benign and < 6cm in
diameter
• Open is the safe option for adrenocortical
cancer and malignant pheochromocytoma
59.
60.
61. Open Approach
• Anterior: suspected regional LN metastasis
• Posterior: small and prior abdominal surgery
• Thoracoabdominal: major vascular invasion
62. Anterior Approach
• Bilateral subcostal incision with extension
midline and cephalad (Mercedes-Benz
incision)
• Inspect liver and peritoneum for metastasis
• For large tumor, mobilize liver and hepatic
flexure
• Mobilize duodenum to expose IVC
63.
64.
65.
66. • Develop plane between IVC and the tumor to
evaluate resectability
• Assess periaortic LN
• Ligate Rt adrenal vein
• Divide small vessels
Editor's Notes
Synthesis of catecholamines
metabolism
organ of Zuckerkandl = mass of chromaffin cell derived from neural crest located along aorta, SMA, renal a