Adrenal Diseases

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

HyperaldosteronismDiagnosis • 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 Facial plethora Hirsutism Menstrual disorders Hypertension Muscular weakness Back pain

• • • • •

Striae Acne Psychological Bruising Abnormal oral glucose tolerance • 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% extraadrenal,10% familial and 10% in children • MEN 2a/2b, neurofibromatosis, von HippelLindau 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 • Secondary • Tertiary

– Indications for surgery – 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 Gastrinoma (ZES) Glucagonoma Somatostatinoma VIPoma PPoma

• DIAGNOSIS • LOCALIZATION • TREATMENT

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, nonfunctional

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