Adrenal tumor, from Schwartz Principle of Surgery

1. Adrenal Tumor
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2. • Embryology
• Anatomy
• Physiology
• Disorders of adrenal cortex
• Disorders of adrenal medulla
• Adrenal incidentaloma
• Adrenal insufficiency
• Adrenal surgery

3. Embryology
• Cortex: from mesoderm
• Medulla: from ectoderm

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

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

12. Physiology
• Catecholamines
– Epinephrine
– Norepinephrine
– Dopamine
– Produce in CNS, SNS, and adrenal medulla

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)

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

23. Cushing’s Syndrome
• หน้าจันทร์แรม (moonface) แก้มอ้อยเชื่อม (hyperglycemia)
เอื้อมไม่ไหว (proximal muscle weakness) ยายมีหนวด
(hirsutism) ปวดกระดูก (osteoporosis) ลูกทะเล (Na
retention) เท่เหมือนควาย (buffalo hump) ลายหน้าท้อง
(striae) หมอง skin (hyperpigmentation) กินจนอ้วน
(truncal obesity) ด่วน mismens (abnormal
menstruation) เด่น pressure (hypertension) เจอ RC
(polycythemia) ขี่ immune (immune suppression)
wound healing (delayed wound healing) addicting
steroid (steroid addiction) คอยให้โต (growth
retardation) โผล่ PU (PU) ดู coag (coagulopathy) แฝง
จิตเพ้อ (mental disorder) เฮ้อ pancreatitis

24. Cushing’s Syndrome
• Truncal obesity = most common symptom
• Early diagnosis: recognize these manifestation
• Cushing’s disease: pituitary tumor  bilateral
adrenal hyperplasia and hypercortisolism

28. Cushing’s Syndrome
• Radiologic studies:
– CT
– MRI

29. Cushing’s Syndrome
• Treatment
– Adrenal adenoma: laparoscopic adrenalectomy
– Cushing’s disease: transphenoidal excision of
pituitary adenoma
– Ectopic ACTH production: treating the primary
tumor

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

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

39. Congenital Adrenal Hyperplasia
• Diagnostic tests
– Karyotype analysis
– Measurement of plasma and urinary steroids
– Dexamethasone suppression test: distinguish
hyperplasia from neoplasia
– CT
– MRI
– Iodocholesterol scan

40. Congenital Adrenal Hyperplasia
• Treatment
– Cortisol and mineralocorticoid replacement:
steroid required are often supraphysiologic and
lead to iatrogenic hypercortisolism
– Alternatively, bilateral laparoscopic adrenalectomy

41. Disorders of Adrenal Medulla
• pheochromocytoma

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

46. Pheochromocytoma
• Treatment
– adrenalectomy
– Blood pressure control and volume repletion:
• alpha blockade: phenoxybenzamine
• Beta blocker
• CCB: nicardipine

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

52. Adrenal Insufficiency
• Most common Primary AI are autoimmune
disease, infection, and metastatic deposits
• Most common secondary AI is exogenous
steroid therapy

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

57. SURGERY

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

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

66. • Develop plane between IVC and the tumor to
evaluate resectability
• Assess periaortic LN
• Ligate Rt adrenal vein
• Divide small vessels