Surgery of the Adrenals

Mete DUREN, M.D.

Department of Endocrine Surgery
 Hyperaldosteronism
• Aldosterone: Principle action in distal
renal tubule
– Increased sodium resorption, decreased
sodium excretion
– Decreased potassium resorption
– Increased hydrogen ion secretion
Renin-angiotensin system
 Hyperaldosteronism-
Diagnosis
• Primary Hyperaldosteronism:
– Hypernatremia: Positive sodium balance,
hypervolemia and HTN
– Hyporeninemia
– Hypokalemia: Hyperglycemia (from B-cell
insulin reduction), muscle weakness,
polyuria
– Metabolic alkalosis
Hyperaldosteronism-Differential
• Primary aldosteronism secondary to an adrenal
adenoma (Conn’s syndrome)
• Primary aldosteronism from adrenal hyperplasia
• Adrenal cortical carcinoma
• Secondary hyperaldosteronism: increased renin
secretion from renal artery stenosis, congestive
heart failure, renal salt-wasting, juxtoglomerular
hyperplasia (Bartter syndrome)
 Hyperaldosteronism
• Diagnosis: HTN, hypokalemia
– urinary or plasma aldosterone
– plasma renin
– saline load: 2000L NS over 4-hour period
and failure of aldosterone level to suppress
• Localization: CT/MRI, Selective venous
sampling
• Treatment: Adrenalectomy for adenoma,
carcinoma and some types of hyperplasia
 Cushing’s Syndrome
• Obesity • Striae
• Facial plethora • Acne
• Hirsutism • Psychological
• Menstrual disorders • Bruising
• Hypertension • Abnormal oral glucose
• Muscular weakness tolerance
• Back pain • Diabetes
Hypercortisolism (Cushing Syndrome)

• Adrenal tumors
• Ectopic ACTH
• Pituitary ACTH secretion
 Cushing’s Syndrome
• 70% of patients have HTN
• 0.2% of ALL causes of HTN
• 80% Corticotropin-dependent
(CORTICOTROPIN = ACTH)
– Pituitary-dependent (70% of Cushing’s)
– Ectopic (10% of Cushing’s)
• ACTH-independent
– Adrenal adenoma, hyperplasia or carcinoma
 Cushing’s Syndrome
Treatment Options
• Ectopic
– Remove primary tumor
– Bilateral adrenalectomy
• Pituitary dependent:
– *Transphenoidal tumor removal*
– Pituitary irradiation
– Pharmacologic
– Bilateral adrenalectomy
• Corticotropin-Independent
– Bilateral/unilateral adrenalectomy
stria
stria
POSTOP ADRENAL
INSUFFICIENCY
 Pheochromocytoma
• Primarily in adrenal medulla
• Produce catecholamines
• Clinically: HTN, tachycardia,
nervousness and sweating
• Secretion patterns:
– Sustained
– Episodic
– Combination
 Pheochromocytoma
• 1-2/100,000 adults
• 0.1-1% of patients with HTN
• 1/3 sudden deaths after minor surgery
• “10% tumor”- 10% malignant, 10%
multifocal, 10% bilateral, 10% extra-
adrenal,10% familial and 10% in children
• MEN 2a/2b, neurofibromatosis, von Hippel-
Lindau syndrome
 Pheochromocytoma
• Diagnosis: 24hr urine metanephrines,
vanillylmandelic acid and
catecholamines (Detect > 98%)
• BIOCHEMICAL DIAGNOSIS BEFORE
LOCALIZATION STUDIES
• Localization: CT, MRI,
Metaiodobenzylguanidine (MIBG)
Pheochromocytoma
 Pheochromocytoma
• Treatment surgery…
• BUT FIRST PREPARATION
• Begin with alpha-blockade and volume
replacement
• Followed +/- beta-blockade
 Incidentaloma
• Functional tumor?
*Na K Aldosteron, Cortisol
*VMA, Metanephrine, Normetanephrine
• Age <50?
• MR-CT?
*<2 cm *2-5 cm *>5 cm
Parathyroid
Glands
 Parathyroid Glands
• Primary – Indications for
• Secondary surgery
• Tertiary – Diagnosis
– Localization
– Treatment
Parathyroidectomy
Parathyroidectomy
Caution, Trouble & Tales of Woe
• Familial Hypocalciuric Hypercalcemic
Syndrome (FHH)
• Familial hyperparathyroidism, MEN 1,
MEN2A
• Missing Parathyroids…when to stop
ENDOCRINE PANCREAS
• Insulin
• Glucagon
• Somatostatin
(which
prevents the
release of the
other two
hormones)

Islets of Langerhans
 ENDOCRINE PANCREAS
• A or alpha cells: Secrete glucagon,
increases blood sugar levels. Respond to
gastrointestinal hormones, neural inputs and
paracrine agents such as somatostatin.
• B or beta cells. Secrete insulin, which
decreases blood sugar levels, and amylin
which modulates the action of insulin.
Respond to gastrointestinal hormones,
blood sugar levels and neural inputs.
 ENDOCRINE PANCREAS
• D or delta cells: Release somatostatin, a
paracrine inhibitor of insulin secretion.
• Neurosecretory PP cells: Release pancreatic
polypeptide, a paracrine inhibitor of acinar
cell secretion.
• D1 cells: Secrete vasoactive intestinal
peptide (VIP) which causes localized
vasodilation and participates in the
regulation of insulin secretion.
 Endocrine Pancreas
• Insulinoma • DIAGNOSIS
• Gastrinoma (ZES) • LOCALIZATION
• Glucagonoma • TREATMENT
• Somatostatinoma
• VIPoma
• PPoma
 Multiple endocrine neoplasias
(MEN SYNDROMES)
• MEN 1: Characteristics and Management
– Autosomal dominant, MENIN gene,
Chromosome 11
– Parathyroid hyperplasia
– Pituitary: #1 Prolactinoma, #2 Growth hormone
secreting
– Pancreas: #1 Gastrinoma, #2 insulinoma, non-
functional
 Multiple endocrine neoplasias
(MEN SYNDROMES)
• MEN 2a
– Autosomal dominant, Ret proto-oncogene
mutation on chromosome 10
– Medullary thyroid carcinoms (virtually 100%)
– Parathyroid hyperplasia
– Pheochromocytoma
 Multiple endocrine neoplasias
(MEN SYNDROMES)
• Men 2b
– Autosomal dominant, Ret proto-oncogene
mutation on chromosome 10
– Phenotypical appearance: Marfanoid habitus,
mucosal neuromas
– Medullary thyroid carcinoma (virtually 100%)
– Pheochromocytoma
 Question 1.
1. A biochemically euthyroid woman presents with a
3 cm. right thyroid nodule. The first step in
management is:
a. Thyroid sonogram
b. Fine-needle aspiration
c. I-123 radionucliotide scanning
d. Thyroidectomy
e. CT-scan
 Question 2.
2. A patient is suspected to have a
medullary thyroid carcinoma (MTC).
Which serologic test is elevated in
patients with MTC?
a. Pentagastrin
b. Calcitonin
c. Calcium
d. Gastrin
e. Thyroglobulin
 Question 3.
3. Which gene analysis should be completed in
patients with MTC?
a. RET proto-oncogene on chromosome 10
b. RET proto-oncogene on chromosome 11
c. MENIN gene on chromosome 10
d. MENIN gene on chromosome 11
e. None of the above
 Question 4.
4. A patient with known
hyperparathyroidism presents to the ER
with a calcium of 16mg/dl. The next
most appropriate step in management is:
a. Emergent parathyroidectomy
b. Intravenous pamidronate
c. Intravenous furosemide
d. Intravenous saline hydration
e. Corticosteroids and mithramycin
 Question 5.
5. A patient presents with hypercalcemia,
severe recurrent PUD and acromegaly.
Of the following, the most likely
diagnosis is:
a. MEN 1
b. MEN 2A
c. MEN 2A or 3
d. MEN IV
e. Pituitary adenocarcinoma
 Question 6.
6. A 30 yo woman presents with hypertension.
You suspect a pheochromocytoma. The first
investigation ordered is:
a. CT of the abdomen/pelvis
b. MRI of the abdomen/pelvis
c. Retroperitoneal sonogram
d. MIBG scintigraphy
e. 24hr urine catecholamines, VMA,
metanephrines
 Question 7.
7. A 60yo man undergoes a AAA repair.
Postoperatively he is responding poorly to fluids
and pressors. His SVR is reduced and he is
tachycardic. You suspect that he may be adrenally
insufficient.
 Question 7
7. Your first step in management is:
a. Obtain a serum cortisol level
b. Dexamethasone suppression test
c. Cosytropin (cotrosyn) stimulation test
d. Administer dexamethasone
e. None of the above
 Question 8.
8. A 45 year old man presents with
hypergastrinemia. Which of the following is NOT
in the differential?
a. Hyperparathyroidism
b. Chronic atrophic gastritis
c. Renal failure
d. Gastric outlet obstruction
e. MEN 2b
 Question 9.
9. A patient is explored for a gastrinoma.
There are no tumors identified in the
pancreas on palpation or with intraoperative
ultrasound.
 Question 9.
10. The next most appropriate step in management
is:
a. Subtotal pancreatectomy
b. Total pancreatectomy
c. Duodenotomy
d. Pancreaticoduodenectomy
e. Abdominal closure