30: Pathophysiology, evaluation, and management of adrenal disorders

Question 1

The adrenal gland is made of two embryologically and functionally distinct units, which are:
A) Medulla and cortex
B) Hypothalamus and pituitary gland
C) Thyroid and parathyroid gland
D) Pancreas and spleen

Answer 1

A) Medulla and cortex

Explanation: The adrenal gland is made of two embryologically and functionally distinct units, the cortex (outer layer and endocrine) and medulla (inner layer and neurocrine).

Question 2

The cortex of the adrenal gland is derived from which embryonic structure?
A) Ectoderm
B) Mesoderm
C) Endoderm
D) Epiblast

Answer 2

B) Mesoderm

Explanation: The cortex of the adrenal gland is derived from the intermediate mesoderm of the urogenital ridge in the fifth week of gestation as mesenchymal cells proliferate to form the outer layer of the fetal adrenal.

Question 3

The medulla of the adrenal gland is derived from which embryonic structure?
A) Ectoderm
B) Mesoderm
C) Endoderm
D) Neural crest cells

Answer 3

D) Neural crest cells

Explanation: The medulla of the adrenal gland is derived from neural crest cells located in the sympathetic ganglia, which become enveloped by the cortex by the ninth week and ends by week 18.

Question 4

At what age does the adrenal gland reach its full development?
A) At birth
B) At 6 months of age
C) At 12 months of age
D) At 18 months of age

Answer 4

C) At 12 months of age

Explanation: At birth, the gland is twice the weight of the adult gland and continues to develop until 12 months of age.

Question 5

What is the approximate weight of each adrenal gland?
A) 1-2 g
B) 2-3 g
C) 3-4 g
D) 4-5 g

Answer 5

D) 4-5 g

Explanation: The adrenal glands weigh 4–5 g each.

Question 6

What is the blood supply of the adrenal gland?
A) Superior adrenal artery
B) Middle adrenal artery
C) Inferior adrenal artery
D) All of the above

Answer 6

D) All of the above

Explanation: The blood supply of the adrenal gland arises from three arteries: superior adrenal (inferior phrenic artery), middle adrenal (aorta), and inferior (ipsilateral renal artery).

Question 7

How is the right adrenal gland bordered medially?
A) Inferior vena cava
B) Aorta
C) Liver
D) Spleen

Answer 7

A) Inferior vena cava

Explanation: The right adrenal gland is triangular and is bordered medially by the inferior vena cava (IVC).

Question 8

How is the left adrenal gland bordered medially?
A) Inferior vena cava
B) Aorta
C) Liver
D) Spleen

Answer 8

B) Aorta

Explanation: The left adrenal gland is crescent shaped and is bordered medially by the aorta.

Question 9

How is the left adrenal vein drained?
A) Directly into the IVC
B) Into the renal vein
C) Into the superior mesenteric vein
D) Into the hepatic portal vein

Answer 9

B) Into the renal vein

Explanation: The short right adrenal vein drains directly into the IVC; the left vein is longer and drains into the renal vein.

Question 10

Fig. 30.1 Adrenal vascular supply demonstrating inflow from the superior, middle, and inferior adrenal arteries bilaterally. Whereas the right (R.) adrenal vein drains directly into the posterior inferior vena cava, the left (L.) adrenal vein often communicates with the inferior phrenic vein before draining into the left renal vein. Source: (Courtesy of the University of Kentucky.)

Answer 10

Question 11

Fig. 30.2 Vascular supply of adrenal glands. a, Artery; L, left; R, right; v, vein.

Answer 11

Question 12

What is the primary source of cholesterol for the adrenal glands?
A) High-density lipoprotein (HDL)
B) Low-density lipoprotein (LDL)
C) Triglycerides
D) Free fatty acids

Answer 12

B) Low-density lipoprotein (LDL)

Explanation: Low-density lipoprotein (LDL) serves as the primary source of cholesterol for the adrenals.

Question 13

Which layer of the adrenal cortex is responsible for the production of aldosterone?
A) Zona glomerulosa
B) Zona fasciculata
C) Zona reticularis
D) All of the above

Answer 13

A) Zona glomerulosa

Explanation: The zona glomerulosa is the outermost region of the adrenal gland and the only source of aldosterone synthase (CYP11B2), making it the sole source of aldosterone (the primary mineralocorticoid) in the body.

Question 14

What is the primary product of the zona fasciculata?
A) Aldosterone
B) Dehydroepiandrosterone (DHEA)
C) Cortisol
D) Androstenedione

Answer 14

C) Cortisol

Explanation: The site of production of glucocorticoids is the zona fasciculata, with cortisol being the primary product.

Question 15

What is the main inhibitor of aldosterone secretion?
A) Angiotensin II
B) Renin
C) Atrial natriuretic peptide (ANP)
D) Adrenocorticotropic hormone (ACTH)

Answer 15

C) Atrial natriuretic peptide (ANP)

Explanation: The main inhibitor of aldosterone secretion is atrial natriuretic peptide (ANP).

Question 16

What is the primary product of the zona reticularis?
A) Aldosterone
B) Dehydroepiandrosterone (DHEA)
C) Cortisol
D) Androstenedione

Answer 16

B) Dehydroepiandrosterone (DHEA)

Explanation: The innermost zone of the adrenal cortex, the zona reticularis, contains large amounts of 17α-hydroxylase and 17,20-lyase, resulting in the production of dehydroepiandrosterone (DHEA), sulfated DHEA (DHEA-S), and androstenedione.

Question 17

Fig. 30.3 Steroid hormone synthesis beginning with cholesterol and resulting in mineralocorticoid, glucocorticoid, and androgen production in the adrenal cortex. Enzymes are listed in boxes and genes in parentheses.

Answer 17

Question 18

What is the primary amino acid precursor for the production of catecholamines in the adrenal medulla?
A) Tryptophan
B) Tyrosine
C) Phenylalanine
D) Methionine

Answer 18

B) Tyrosine

Explanation: Catecholamines, including epinephrine, norepinephrine, and dopamine, are produced from the amino acid tyrosine.

Question 19

What proportion of the adrenal gland is composed of the medulla?
A) 5%
B) 10%
C) 15%
D) 20%

Answer 19

B) 10%

Explanation: The medulla composes 10% of adrenal mass.

Question 20

What is the primary innervation of chromaffin cells in the adrenal medulla?
A) Postganglionic sympathetic fibers
B) Preganglionic sympathetic fibers
C) Parasympathetic fibers
D) Sensory fibers

Answer 20

B) Preganglionic sympathetic fibers

Explanation: Chromaffin cells in the medulla are innervated by preganglionic sympathetic fibers of T11 to L2 similar to the sympathetic ganglia.

Question 21

4: What are the three catecholamines produced in the adrenal medulla?
A) Epinephrine, acetylcholine, and dopamine
B) Norepinephrine, serotonin, and dopamine
C) Epinephrine, norepinephrine, and dopamine
D) Serotonin, acetylcholine, and epinephrine

Answer 21

C) Epinephrine, norepinephrine, and dopamine

Explanation: The systemic stress response is modulated by catecholamines that are produced from the amino acid tyrosine and consist of epinephrine (E) (80%), norepinephrine (NE) (19%), and dopamine (1%).

Question 22

What are the metabolites and enzymes involved in the majority of catecholamine metabolism?
A) Metanephrine, normetanephrine, and VMA; COMT and MAO
B) Epinephrine, norepinephrine, and dopamine; ACE and renin
C) Aldosterone, cortisol, and DHEA; CYP11B2 and 11β-hydroxylase
D) ACTH, CRH, and TRH; POMC and GH

Answer 22

A) Metanephrine, normetanephrine, and VMA; COMT and MAO

Explanation: The majority of catecholamine metabolism occurs in the adrenal medulla, and the metabolites metanephrine, normetanephrine, and VMA and the enzymes COMT and MAO are the most important.

Question 23

What is Cushing syndrome?
A. Hyperthyroidism secondary to excessive production of glucocorticoids by the adrenal cortex
B. Hypothyroidism secondary to excessive production of glucocorticoids by the adrenal cortex
C. Hypercortisolism secondary to excessive production of glucocorticoids by the adrenal cortex
D. Hypocortisolism secondary to excessive production of glucocorticoids by the adrenal cortex

Answer 23

C. Hypercortisolism secondary to excessive production of glucocorticoids by the adrenal cortex.

Explanation: Cushing syndrome is defined as hypercortisolism secondary to excessive production of glucocorticoids by the adrenal cortex.

Question 24

What is the most common cause of hypercortisolism in patients of the Western world?
A. ACTH-dependent cushing syndrome
B. ACTH-independent cushing syndrome
C. Exogenous cushing syndrome
D. Adrenal tumors

Answer 24

C. Exogenous cushing syndrome.

Explanation: Exogenous cushing syndrome is the most common cause of hypercortisolism in patients of the Western world. It results from administration of even low doses of synthetic glucocorticoids taken orally, topically, or inhaled.

Question 25

What is the most common cause of ACTH-dependent hypercortisolism?
A. Adrenal tumors
B. Ectopic ACTH syndrome
C. ACTH-independent macronodular adrenal hyperplasia
D. Cushing disease

Answer 25

D. Cushing disease.

Explanation: Approximately 80% of ACTH-dependent hypercortisolism results from primary pituitary pathology, known as Cushing disease. Microadenomas and small tumors of the pituitary are the most common causes of Cushing disease.

Question 26

Fig. 30.4 Clinically relevant causes of excess cortisol production. ACTH, Adrenocorticotropic hormone; CRH, corticotropin-releasing hormone.

Answer 26

Question 27

What are the classic symptoms of hypercortisolism seen in patients with Cushing syndrome?
a. Peripheral muscle weakness, easy bruisability, and abdominal striae
b. Central obesity, moon facies, and buffalo hump
c. Hypotension, weight loss, and hair loss
d. Nausea, vomiting, and diarrhea

Answer 27

b. Central obesity, moon facies, and buffalo hump are classic symptoms of hypercortisolism seen in patients with Cushing syndrome. However, these symptoms are nonspecific and can be seen in other conditions as well.

Explanation: Cushing syndrome can present with a variety of clinical symptoms that vary considerably, and not all patients exhibit all classic symptoms. Central obesity, moon facies, and buffalo hump are frequently seen in patients with Cushing syndrome, but they are not specific and can be seen in other conditions. Peripheral muscle weakness, easy bruisability, and abdominal striae are other nonspecific symptoms that can be seen in patients with Cushing syndrome.

Question 28

What percentage of patients with Cushing syndrome may have urolithiasis?
a. 10%
b. 25%
c. 50%
d. 75%

Answer 28

c. 50% of patients with Cushing syndrome may have urolithiasis.

Explanation: Urolithiasis, or the formation of urinary stones, can be a common complication in patients with Cushing syndrome, with up to 50% of patients affected. This is likely due to increased levels of urinary calcium excretion, which can promote stone formation.

Question 29

What is the most common cause of endogenous Cushing syndrome?
a. Adrenal tumors
b. Ectopic ACTH production
c. Primary pigmented nodular adrenocortical disease
d. Cushing disease (CD)

Answer 29

d. Cushing disease (CD) accounts for 80%–85% of cases of endogenous Cushing syndrome, with microadenomas and small tumors of the pituitary being the most common cause of CD.

Question 30

Which diagnostic test is a 24-hour direct measurement of bioavailable cortisol?
a. Overnight low-dose dexamethasone suppression test (LD-DST)
b. 24-hour urinary free cortisol (UFC) test
c. Late-night salivary cortisol test
d. Midnight plasma cortisol measurement

Answer 30

b. The 24-hour urinary free cortisol (UFC) test is a direct measurement of bioavailable cortisol that is frequently used to diagnose Cushing syndrome.

Question 31

What is the gold standard approach for distinguishing ectopic ACTH production from CD?
a. Measuring serum ACTH levels
b. High-dose dexamethasone suppression test
c. Direct measurement of ACTH in the inferior petrosal sinus (IPS) after CRH stimulation
d. Imaging techniques

Answer 31

c. Direct measurement of ACTH in the inferior petrosal sinus (IPS) after CRH stimulation has become the gold standard approach for distinguishing ectopic ACTH production from CD. High levels of ACTH in the IPS, when compared with those in peripheral blood, indicate CD, whereas levels similar to peripheral plasma suggest an ectopic ACTH source.

Question 32

What are the two most frequently used tests to diagnose Cushing’s Syndrome?
A. ACTH stimulation test and serum cortisol level test
B. 24-hour urinary free cortisol test and overnight low-dose dexamethasone suppression test
C. Adrenaline test and aldosterone test
D. Thyroid-stimulating hormone (TSH) test and free thyroxine (FT4) test

Answer 32

B. The two most frequently used tests to diagnose Cushing’s Syndrome are the 24-hour urinary free cortisol (UFC) test and the overnight low-dose dexamethasone suppression test (LD-DST).

Question 33

When evaluating incidentalomas, why may the UFC test be inadequate?
A. It is expensive
B. It is not reliable
C. It has low sensitivity
D. It is not specific

Answer 33

C. When evaluating incidentalomas, the UFC test may be inadequate because of its low sensitivity.

Question 34

How does the overnight low-dose dexamethasone suppression test (LD-DST) diagnose Cushing’s Syndrome?
A. It measures cortisol levels directly
B. It measures ACTH levels
C. It stimulates the corticotropic cells of the anterior pituitary and suppresses ACTH production
D. It measures cortisol levels in saliva

Answer 34

C. The overnight low-dose dexamethasone suppression test (LD-DST) stimulates the corticotropic cells of the anterior pituitary, which in turn suppresses ACTH production and results in lower serum cortisol levels. A patient’s failure to suppress cortisol after dexamethasone administration is indicative of CS.

Question 35

What is the first step in identifying the cause of Cushing’s Syndrome?
A. Perform abdominal imaging
B. Measure serum ACTH
C. Perform an adrenalectomy
D. Check cortisol levels

Answer 35

B. The first step in identifying the cause of Cushing’s Syndrome is to measure serum ACTH. Low levels indicate an ACTH-independent cause, and high levels indicate an ACTH-dependent cause.

Question 36

What should be considered if serum ACTH levels are low?
A. Cushing’s Disease
B. Ectopic ACTH Syndrome
C. Adrenal tumor
D. Exogenous source of steroids

Answer 36

C. If serum ACTH levels are low, an ACTH-independent cause should be considered, and abdominal imaging should be performed to look for an adrenal tumor.

Question 37

How can one distinguish between Cushing’s Disease and ectopic ACTH Syndrome?
A. By measuring cortisol levels
B. By performing an adrenalectomy
C. By measuring ACTH levels in the inferior petrosal sinus
D. By performing an MRI of the pituitary gland

Answer 37

C. Direct measurements of ACTH in the inferior petrosal sinus after CRH stimulation have become the gold standard approach for distinguishing ectopic ACTH production from Cushing’s Disease. High levels of ACTH in the inferior petrosal sinus, when compared with those in peripheral blood, indicate Cushing’s Disease, whereas levels similar to peripheral plasma suggest an ectopic ACTH source.

Question 38

How should the cessation of glucocorticoid administration be done in patients with exogenous Cushing’s Syndrome?
A. It should be abrupt
B. It should be rapid
C. It should be gradual
D. It should be done in one step

Answer 38

C. The cessation of glucocorticoid administration in patients with exogenous Cushing’s Syndrome should be gradual so that the HPA axis has ample time to recover. The process can take weeks to months and varies greatly among patients.

Question 39

What is steroid withdrawal syndrome?
A. It is the inability to tolerate glucocorticoid therapy
B. It is the normalization of HPA axis testing
C. It is the onset of Cushing’s Syndrome after cessation of glucocorticoid administration
D. It is the inability to tolerate steroid dose reduction despite apparent normalization in HPA axis testing

Answer 39

D. Steroid withdrawal syndrome is the inability to tolerate steroid dose reduction despite apparent normalization in HPA axis testing.

Question 40

How long can the recovery of the HPA axis take in patients with exogenous Cushing’s Syndrome?
A. A few hours
B. A few days
C. A few weeks to months
D. A few years

Answer 40

C. The recovery of the HPA axis in patients with exogenous Cushing’s Syndrome can take a few weeks to months, and it varies greatly among patients.

Question 41

What is the importance of being aware of steroid withdrawal syndrome?
A. It can cause hypertension
B. It can cause hypotension
C. It can cause adrenal insufficiency
D. It can make it difficult to reduce the steroid dose

Answer 41

D. Being aware of steroid withdrawal syndrome is important because it can make it difficult to reduce the steroid dose, even if the HPA axis testing appears to be normalized.

Question 42

What is the current standard of care for ACTH-secreting pituitary adenomas?
A. Chemotherapy
B. Radiation therapy
C. Trans-sphenoidal surgical resection
D. Medication therapy

Answer 42

C. Trans-sphenoidal surgical resection is the current standard of care for ACTH-secreting pituitary adenomas.

Question 43

What is the success rate of curing macroadenomas through neurosurgical treatment?
A. Over 80%
B. Between 60% and 80%
C. Less than 15%
D. None of the above

Answer 43

C. Less than 15% of patients with macroadenomas are cured after excision of tumors 1 cm or larger.

Question 44

What is a common complication after resection of a pituitary adenoma?
A. Nelson syndrome
B. Adrenal insufficiency
C. Hypopituitarism
D. Hypercortisolism

Answer 44

C. Hypopituitarism after resection of a pituitary adenoma is a known complication, with rates varying from 5% to 50%.

Question 45

When is bilateral adrenalectomy recommended for treating ACTH-secreting pituitary adenomas?
A. When the tumor is less than 1 cm in size
B. When the tumor is resistant to neurosurgical treatment
C. When there is a mild case of hypercortisolism
D. When the patient has mild hypopituitarism

Answer 45

B. Bilateral adrenalectomy is recommended when at least one attempt to treat the primary tumor has failed, and when hypercortisolism is life-threatening and swift definitive treatment is mandatory.

Question 46

What is the disadvantage of bilateral adrenalectomy?
A. A risk of progressive growth of the pituitary adenoma
B. A high risk of postoperative hypopituitarism
C. A low success rate in resolving hypercortisolism
D. A requirement for lifelong mineralocorticoid and glucocorticoid replacement

Answer 46

A. The disadvantage of bilateral adrenalectomy is that patients are at risk for progressive growth of their pituitary adenoma, which can result in ocular chiasm compression, oculomotor deficiencies, and, rarely, a rise in intracranial pressure. This is known as the Nelson-Salassa syndrome, or just Nelson syndrome, which is found in 8%–29% of patients who have undergone bilateral adrenalectomy.

Question 47

What is the ideal treatment for an ACTH-producing tumor in ectopic ACTH syndrome?
A. Chemotherapy
B. Radiation therapy
C. Excision of the tumor
D. Bilateral adrenalectomy

Answer 47

C. Excision of the ACTH-producing tumor is ideal but possible in only 10% of patients.

Question 48

What is an excellent option for patients with unresectable or unidentifiable ACTH-producing tumors?
A. Chemotherapy
B. Radiation therapy
C. Excision of the tumor
D. Bilateral adrenalectomy

Answer 48

D. Bilateral adrenalectomy is an excellent option for patients with unresectable or unidentifiable ACTH-producing tumors.

Question 49

How should cortisol-producing adrenal masses be treated in ACTH-independent disease?
A. With chemotherapy
B. With radiation therapy
C. With partial or total adrenalectomy
D. With medical treatment

Answer 49

C. Cortisol-producing adrenal masses should be treated with either partial or total adrenalectomy.

Question 50

What medications can be used to treat hypercortisolism when surgical intervention is not possible?
A. Chemotherapy drugs
B. Antibiotics
C. Medications that block enzymes of steroid synthesis
D. Blood pressure medications

Answer 50

C. Medications that block enzymes of steroid synthesis such as metyrapone, aminoglutethimide, ketoconazole and etomidate can be used to bridge the patient waiting for surgery or when surgical intervention is not possible.

Question 51

Why might medical treatment for hypercortisolism be necessary?
A. To cure the underlying disease
B. To reduce the risk of recurrence after surgery
C. To manage symptoms while waiting for surgery
D. To prevent the need for surgery

Answer 51

C. Medical treatment for hypercortisolism may be necessary to manage symptoms while waiting for surgery, or when surgical intervention is not possible.

Question 52

What is the rate-limiting step in the RAAS cascade?
A. Release of angiotensin II
B. Release of aldosterone
C. Release of renin
D. Release of ACTH

Answer 52

C. The release of renin from the JG cells is the rate-limiting step in the RAAS cascade.

Question 53

What triggers the release of aldosterone from the zona glomerulosa?
A. Angiotensin II
B. ACTH
C. Renin
D. Potassium

Answer 53

A. Angiotensin II triggers the release of aldosterone from the zona glomerulosa.

Question 54

In Conn syndrome, what is the cause of elevated aldosterone secretion?
A. Elevated renin levels
B. Low renal perfusion pressure
C. Increased renal sympathetic nervous activity
D. An aldosterone-producing tumor

Answer 54

D. In Conn syndrome, aldosterone secretion is independent of the RAAS, and plasma renin levels will be suppressed. This finding is in contrast with patients who have secondary hyperaldosteronism, in whom elevated renin levels are the cause of elevations in aldosterone secretion.

Question 55

What is the difference in clinical presentation between patients with idiopathic hyperplasia and those with aldosterone-producing adenomas?
A. Patients with idiopathic hyperplasia have less severe HTN and are less likely to be hypokalemic compared with patients with aldosterone-producing adenomas.
B. Patients with idiopathic hyperplasia have more severe HTN and are more likely to be hypokalemic compared with patients with aldosterone-producing adenomas.
C. Both conditions have similar clinical presentations.
D. Patients with idiopathic hyperplasia have more severe HTN but are less likely to be hypokalemic compared with patients with aldosterone-producing adenomas.

Answer 55

A. Patients with idiopathic hyperplasia have less severe HTN and are less likely to be hypokalemic compared with patients with aldosterone-producing adenomas.

Question 56

What is the recommended treatment for unilateral adrenal hyperplasia in primary aldosteronism?
A. Medical management
B. Unilateral adrenalectomy
C. Bilateral adrenalectomy
D. Chemotherapy

Answer 56

B. Unilateral adrenal hyperplasia is distinctly uncommon but, when appropriately diagnosed, is potentially curable with adrenalectomy. In comparison with idiopathic hyperplasia, aldosterone-producing adenomas are associated with more profound HTN and hypokalemia.

Question 57

Fig. 30.5 Subtypes of primary aldosteronism.

Partin, Alan W.; Kavoussi, Louis R.; Peters, Craig A.; Dmochowski, Roger R.. Campbell Walsh Wein Handbook of Urology - E-Book (p. 778). Elsevier Health Sciences. Kindle Edition.

Answer 57

Question 58

Box 30.1 Subtypes of Primary Aldosteronism

Partin, Alan W.; Kavoussi, Louis R.; Peters, Craig A.; Dmochowski, Roger R.. Campbell Walsh Wein Handbook of Urology - E-Book (p. 778). Elsevier Health Sciences. Kindle Edition.

Answer 58

Question 59

What is the hallmark clinical presentation of hyperaldosteronism?
A. Refractory HTN and proteinuria
B. Hypokalemia and renal failure
C. Cardiac events and stroke
D. Atrial fibrillation and HTN

Answer 59

A. Virtually all patients present with refractory HTN.

Question 60

How often is hypokalemia present in hyperaldosteronism?
A. 10% of the time
B. 25% of the time
C. 50% of the time
D. 75% of the time

Answer 60

C. Hypokalemia is classically a hallmark of the disease but may only be present 10%–50% of the time.

Question 61

What are some potential consequences of the HTN associated with hyperaldosteronism?
A. Joint pain and stiffness
B. Vision problems
C. Renal failure and proteinuria
D. Digestive issues

Answer 61

C. Cardiac and renal disease may be present because of the HTN. Stroke, atrial fibrillation, cardiac events, proteinuria, and renal failure are all increased in hyperaldosteronism.

Question 62

What is the clinical significance of hypokalemia in hyperaldosteronism?
A. It is the hallmark of the disease
B. It can cause joint pain and stiffness
C. It is associated with cardiac and renal disease
D. It is not clinically significant

Answer 62

C. Hypokalemia is associated with cardiac and renal disease in hyperaldosteronism.

Question 63

What is the relationship between hyperaldosteronism and atrial fibrillation?
A. There is no relationship between the two.
B. Hyperaldosteronism increases the risk of atrial fibrillation.
C. Atrial fibrillation can cause hyperaldosteronism.
D. The two conditions have similar clinical presentations.

Answer 63

B. Hyperaldosteronism increases the risk of atrial fibrillation.

Question 64

What should be done prior to screening a patient for primary hyperaldosteronism?
A. Obtain a morning (between 8–10 am) plasma aldosterone concentration (PAC) and pra.
B. Encourage the patient to consume more sodium and licorice.
C. Begin treatment with beta-blockers as first-line therapy for HTN.
D. Discontinue all medications prior to screening.

Answer 64

D. Hypokalemia needs correction and significant medications discontinued prior to screening the patient.

Question 65

What medications should be used as first line to treat HTN in patients with primary hyperaldosteronism?
A. ACE inhibitors
B. Beta-blockers
C. Calcium channel blockers or alpha blockers
D. Diuretics

Answer 65

C. Alpha or calcium channel blockers should be employed as first line to treat HTN.

Question 66

What is the normal range for plasma aldosterone concentration (PAC) in primary hyperaldosteronism screening?
A. <5 ng/dL
B. <10 ng/dL
C. <20 ng/dL
D. <30 ng/dL

Answer 66

C. PAC >20 ng/dL is considered abnormal.

Question 67

What imaging modality is recommended for patients suspected of primary hyperaldosteronism?
A. CT scan
B. MRI
C. PET scan
D. X-ray

Answer 67

A. All patients suspected of primary hyperaldosteronism should get cross-sectional imaging.

Question 68

What is the typical appearance of hyperaldosteronomas on imaging?
A. Unilateral, high-density, enhancing lesion
B. Bilateral, low-density, enhancing lesion
C. Unilateral, low-density, nonenhancing lesion
D. Bilateral, high-density, nonenhancing lesion

Answer 68

C. Hyperaldosteronomas are typically unilateral, low-density, nonenhancing lesion <10 Hounsfield units (HU), with an average size of 1.6–1.8 cm and a normal-appearing contralateral adrenal gland.

Question 69

Fig. 30.6 Primary aldosteronism diagnosis and treatment algorithm.

Partin, Alan W.; Kavoussi, Louis R.; Peters, Craig A.; Dmochowski, Roger R.. Campbell Walsh Wein Handbook of Urology - E-Book (p. 779). Elsevier Health Sciences. Kindle Edition.

Answer 69

Question 70

In primary aldosteronism screening, what is the significance of a PAC level >20 ng/dL and PRA below the detection level?
A. They indicate a normal result and no further testing is needed.
B. They suggest the need for a flurocortisone suppression test.
C. They are diagnostic of primary aldosteronism and no confirmatory testing is needed.
D. They are suggestive of primary aldosteronism and require further confirmatory testing.

Answer 70

C. If there is HTN, hypokalemia, PRA below detection, and PAC >20 ng/dL, no confirmatory testing is needed.

Question 71

What is the flurocortisone suppression test used for in the diagnosis of primary aldosteronism?
A. To measure PAC in the upright position
B. To measure aldosterone, sodium, and creatinine levels in 24-hour urine
C. To suppress PAC and diagnose primary aldosteronism
D. To determine adrenal vein–to–IVC ratios

Answer 71

C. The flurocortisone suppression test involves 0.1 mg every 6 hours in addition to NaCl 2 g every 8 hours, both for 4 days. PAC is measured in the upright position. Failure to suppress PAC to less than 6 ng/dL is diagnostic of primary aldosteronism.

Question 72

What is the diagnosis of primary aldosteronism made based on in the oral sodium loading test?
A. Serum aldosterone levels
B. Serum sodium levels
C. 24-hour urine measurements of aldosterone, sodium, and creatinine
D. Serum creatinine levels

Answer 72

C. The diagnosis of primary aldosteronism is made when the 24-hour aldosterone is >12 μg/day.

Question 73

What is the diagnostic cutoff for PAC levels in the IV saline infusion test?
A. <5 ng/dL
B. >5 ng/dL
C. <10 ng/dL
D. >10 ng/dL

Answer 73

B. The infusion is performed in the morning after an overnight fast while the patient is in a recumbent position. After the IV infusion of saline, PAC is measured; a level >5 ng/dL is diagnostic of primary aldosteronism, and levels >10 ng/dL are suggestive of aldosterone-producing adenomas.

Question 74

What is adrenal vein sampling (AVS) used for in the diagnosis of primary aldosteronism?
A. To measure PAC in the upright position
B. To measure aldosterone, sodium, and creatinine levels in 24-hour urine
C. To suppress PAC and diagnose primary aldosteronism
D. To determine lateralization of aldosterone production

Answer 74

D. AVS can be useful to determine lateralization of aldosterone production.

Question 75

What is the preferred treatment for patients with adrenal tumors who are eligible for surgery?
A) Medical therapy with aldosterone receptor agonists
B) Radiotherapy
C) Chemotherapy
D) Adrenalectomy

Answer 75

D) Adrenalectomy is the preferred treatment for patients with adrenal tumors who are eligible for surgery. According to the text, “When total or partial adrenalectomy is feasible, this is the procedure of choice.”

Question 76

What proportion of patients undergoing adrenalectomy can expect improvement in blood pressure?
A) All patients
B) Most patients
C) Half of the patients
D) None of the patients

Answer 76

B) Most patients undergoing adrenalectomy can expect improvement in blood pressure. According to the text, “The majority undergoing adrenalectomy will have improvement in HTN, and most will discontinue some or all medications, a significant portion will have no change in blood pressure.”

Question 77

What is the first-line medical therapy for patients with adrenal tumors who are not eligible for surgery?
A) Spironolactone
B) Eplerenone
C) Radiotherapy
D) Chemotherapy

Answer 77

A) Spironolactone is a first-line medical therapy for patients with adrenal tumors who are not eligible for surgery. According to the text, “Medical therapy is successful at normalizing both HTN and potassium and consists of aldosterone receptor agonists spironolactone and eplerenone. Spironolactone is initiated at doses of 25–50 mg/day and can be titrated up to 400 mg/day, depending on blood pressure, serum potassium levels, and side effects.”

Question 78

What is a potential side effect of spironolactone therapy?
A) Increased appetite
B) Weight gain
C) Gynecomastia
D) Increased libido

Answer 78

C) Gynecomastia is a potential side effect of spironolactone therapy. According to the text, “Side effects include gynecomastia, impotence, and menstrual disturbances.”

Question 79

What is the maximum dose of eplerenone that can be used to treat patients with adrenal tumors?
A) 25 mg/day
B) 50 mg/day
C) 100 mg/day
D) 200 mg/day

Answer 79

C) The maximum dose of eplerenone that can be used to treat patients with adrenal tumors is 100 mg/day. According to the text, “Eplerenone is better tolerated because of increased selectivity for the aldosterone receptor. Treatment should be initiated with 25 mg/day and titrated up to 100 mg/day (Table 30.1).”

Question 80

Table 30.1 Perioperative Glucocorticoid Administration in Patients on Chronic Steroid Therapy

Partin, Alan W.; Kavoussi, Louis R.; Peters, Craig A.; Dmochowski, Roger R.. Campbell Walsh Wein Handbook of Urology - E-Book (p. 782). Elsevier Health Sciences. Kindle Edition.

Answer 80

Question 81

What is a pheochromocytoma?
A) A tumor of medullary hormone-producing cells
B) A tumor of adrenal glandular cells
C) A tumor of the parathyroid gland
D) A tumor of the thyroid gland

Answer 81

A) A pheochromocytoma is a tumor of medullary catecholamine-producing cells.

Question 82

What proportion of hypertension cases are caused by pheochromocytoma?
A) 1%
B) 5%
C) 10%
D) 25%

Answer 82

A) Pheochromocytoma is responsible for 0.5% of hypertension cases, according to the text.

Question 83

What percentage of incidental adrenal masses are pheochromocytomas?
A) 0.5%
B) 5%
C) 10%
D) 25%

Answer 83

B) Pheochromocytomas account for 5% of incidental adrenal masses, according to the text.

Question 84

Where can extra-adrenal pheochromocytomas (paragangliomas) occur?
A) Only in the adrenal glands
B) Only in the head and neck
C) In various locations throughout the body
D) In the lungs

Answer 84

C) Extra-adrenal pheochromocytomas (paragangliomas) can occur in various locations throughout the body, including the head, neck, thorax, abdomen, pelvis, and bladder, according to the text.

Question 85

What are the common locations of paragangliomas?
A) The brain and spinal cord
B) The liver and spleen
C) The kidneys and bladder
D) Between the aortic bifurcation and the root of the inferior mesenteric artery

Answer 85

D) The organ of Zuckerkandl, which is a cluster of chromaffin cells, lies between the aortic bifurcation and the root of the inferior mesenteric artery and is a common location for paragangliomas, according to the text.

Question 86

What causes the variability of clinical manifestations of pheochromocytomas?
a) The variability of the amount of NE produced
b) The variability of the amount of dopamine produced
c) The variability of the amount of E produced
d) The variability of the enzyme PNMT

Answer 86

d) The variability of the enzyme PNMT

Explanation: The variability of the enzyme phenylethanolamine-N-methyltransferase (PNMT) causes the variability of clinical manifestations of pheochromocytomas depending upon the amounts of E, NE, and dopamine that are produced.

Question 87

What is the cause of the variability in clinical manifestations of pheochromocytoma?
A) The location of the tumor
B) The size of the tumor
C) The amounts of E, NE, and dopamine produced by the tumor
D) The patient’s age

Answer 87

C) The variability of clinical manifestations of pheochromocytoma is caused by the variability of the amounts of E, NE, and dopamine that are produced by the tumor, according to the text.

Question 88

What percentage of pheochromocytomas are familial?
A) 10%
B) 33%
C) 50%
D) 75%

Answer 88

B) Familial cases account for 33% of pheochromocytomas, according to the text.

Question 89

What are two examples of genetic conditions that can cause hereditary pheochromocytomas?
A) Down syndrome and Turner syndrome
B) Von Hippel-Lindau (VHL) and neurofibromatosis
C) Marfan syndrome and Ehlers-Danlos syndrome
D) Huntington’s disease and muscular dystrophy

Answer 89

B) Von Hippel-Lindau (VHL) and neurofibromatosis are examples of genetic conditions that can cause hereditary pheochromocytomas, according to the text.

Question 90

How is malignant pheochromocytoma defined?
A) By the size of the tumor
B) By the location of the tumor
C) By the presence of clinical metastases
D) By the age of the patient

Answer 90

C) Malignant pheochromocytoma is defined by the presence of clinical metastases, according to the text.

Question 91

Pheochromocytoma is often referred to as the 10% tumor because it:
a) is familial in nature
b) occurs in children
c) is extra-adrenal in location
d) is malignant in nature

Answer 91

c) is extra-adrenal in location. extra-adrenal, familial, pediatric, bilateral, and malignant.

Partin, Alan W.; Kavoussi, Louis R.; Peters, Craig A.; Dmochowski, Roger R.. Campbell Walsh Wein Handbook of Urology - E-Book (p. 783). Elsevier Health Sciences. Kindle Edition.

Question 92

What is the most common location for pheochromocytomas?
a) left adrenal gland
b) right adrenal gland
c) extra-adrenal tissue
d) both adrenal glands

Answer 92

B

Question 93

What is the classic hallmark of pheochromocytoma?
A) Severe hypertension
B) Episodic sudden perspiration
C) Tachycardia
D) Headache

Answer 93

B) Episodic sudden perspiration. The triad of headache, episodic sudden perspiration, and tachycardia is a classic hallmark of pheochromocytoma.

Explanation: The triad of headache, episodic sudden perspiration, and tachycardia is a classic hallmark of pheochromocytoma. However, more than 20% of patients can be asymptomatic and depending on the catecholamine milieu of each tumor, symptomatology can vary greatly.

Question 94

What percentage of patients with pheochromocytoma demonstrate persistently elevated blood pressure?
A) 10% - 20%
B) 30% - 40%
C) 50% - 60%
D) 70% - 80%

Answer 94

C) 50% - 60%. The remainder of patients demonstrate persistently elevated blood pressure, and a minority are normotensive.

Explanation: Paroxysmal hypertension is classic in pheochromocytoma; however, such episodic spikes in blood pressure are documented in only approximately 30%–50% of patients and can occur in essential hypertension. The remainder of patients demonstrate persistently elevated blood pressure, and a minority are normotensive.

Question 95

What is more common in extra-adrenal lesions and in the SDHB mutation?
A) Metastatic disease
B) Malignancy
C) Bilateral tumors
D) Pediatric tumors

Answer 95

A) Metastatic disease. Metastatic disease is more common in extra-adrenal lesions and in the SDHB mutation.

Explanation: Metastatic disease is more common in extra-adrenal lesions and in the SDHB mutation. Common sites of metastasis include bone, lung, liver, and lymph nodes. Most metastatic pheochromocytomas are discovered within 5 years of the original diagnosis. Hereditary pheochromocytomas occur at a younger age and are more often multifocal and bilateral.

Question 96

What imaging technique is the gold standard for imaging pheochromocytoma?
A) Computed tomography (CT)
B) Magnetic resonance imaging (MRI)
C) Positron emission tomography (PET)
D) Metaiodobenzylguanidine scintigraphy (MIBG)

Answer 96

C) Positron emission tomography (PET). Fluorine-18 fluorodeoxyglucose positron emission tomography is the gold standard for imaging pheochromocytoma.

Explanation: Positron emission tomography (PET) is the gold standard for imaging pheochromocytoma. It uses fluorine-18 fluorodeoxyglucose to visualize the glucose metabolism of the tumor. This test can be especially useful for identifying malignant pheochromocytomas and detecting metastasis.

Question 97

What is the most sensitive and specific agent for diagnosing pheochromocytoma?
A) CT
B) MRI
C) PET
D) MIBG

Answer 97

D) MIBG. Metaiodobenzylguanidine scintigraphy (MIBG) is the most sensitive and specific agent for diagnosing pheochromocytoma. MIBG is a radiopharmaceutical that is selectively taken up by chromaffin tissue, making it the most sensitive and specific agent for diagnosing pheochromocytoma. It is used for recurrences or extra-adrenal disease.

Question 98

What is the typical attenuation measurement on unenhanced CT for pheochromocytomas?
A) < 5 HU
B) 5-10 HU
C) > 10 HU
D) 15-20 HU

Answer 98

C) > 10 HU. Pheochromocytomas typically measure an attenuation of >10 HU on unenhanced CT.

Explanation: Pheochromocytomas typically measure an attenuation of >10 HU on unenhanced CT due to their rich vascularity and low lipid content. This can help differentiate them from other adrenal masses, such as adenomas, which typically have a lower attenuation value.

Question 99

When is functional imaging typically required in the diagnosis of pheochromocytoma?
A) When CT or MRI reveals an adrenal mass
B) When there are symptoms of paroxysmal hypertension
C) When there are no other diagnostic tests available
D) In the vast majority of cases, functional imaging is not required

Answer 99

D) In the vast majority of cases, functional imaging is not required. When CT or MRI reveals an adrenal mass and biochemical testing suggests a pheochromocytoma, surgery is indicated. In the vast majority of cases, functional imaging is not required.

Explanation: When CT or MRI reveals an adrenal mass and biochemical testing suggests a pheochromocytoma, surgery is indicated. In the vast majority of cases, functional imaging is not required. However, in certain cases, such as when there is suspicion of metastatic disease, functional imaging may be used to help guide treatment.

Question 100

Table 30.2 Test Characteristics for Diagnosis of Pheochromocytoma From a Large Multicenter Cohort Study

Answer 100

Question 101

Table 30.3 Relative Merits for and Against Use of Plasma-Free Metanephrines and Urinary Fractionated Metanephrines in the Diagnosis of Pheochromocytoma

Answer 101

Question 102

What genetic mutations should be tested for in all patients younger than 50 years of age?

Answer 102

: A) RET, VHL, SDHB, and SDHD. All patients younger than 50 years of age should receive genetic testing for the RET, VHL, SDHB, and SDHD gene mutations.

Explanation: Pheochromocytomas can be associated with certain genetic mutations, such as RET, VHL, SDHB, and SDHD. Patients younger than 50 years of age should be tested for these mutations, as they are more likely to have a hereditary form of the disease.

Question 103

What is the gold standard treatment for pheochromocytoma?
A) Chemotherapy
B) Radiation therapy
C) Surgical excision via minimally invasive surgery
D) Hormone replacement therapy

Answer 103

C) Surgical excision via minimally invasive surgery. Surgical excision via minimally invasive surgery is the gold standard for the treatment of pheochromocytoma.

Explanation: Surgical excision via minimally invasive surgery is the gold standard for the treatment of pheochromocytoma. The preoperative evaluation by a cardiologist is recommended to optimize blood pressure control prior to surgery.

Question 104

What is the drug of choice for preoperative treatment of pheochromocytoma?
A) Atenolol
B) Phenoxybenzamine
C) Terazosin
D) Prazosin

Answer 104

B) Phenoxybenzamine. Phenoxybenzamine is the drug of choice for preoperative treatment of pheochromocytoma.

Explanation: Alpha blockers, such as phenoxybenzamine, are the mainstay of preoperative treatment for pheochromocytoma. Phenoxybenzamine is an irreversible alpha blocker that is started 14 days prior to surgery and titrated to blood pressure (BP) 120–130/80 mm Hg in a seated position.

Question 105

Should beta blockers be used before alpha blockers in the preoperative management of pheochromocytoma?
A) Yes, beta blockers are the first-line treatment.
B) No, beta blockers should never be used before alpha blockers.
C) Beta blockers may be used if alpha blockers are not effective.
D) It depends on the specific symptoms and presentation of the patient.

Answer 105

B) No, beta blockers should never be used before alpha blockers. Beta blockers should never be used before alpha blockers in the preoperative management of pheochromocytoma.

Explanation: Beta blockers should never be used before alpha blockers in the preoperative management of pheochromocytoma because they can cause unopposed alpha-mediated vasoconstriction, which can lead to hypertensive crisis.

Question 106

What is the role of intravascular volume in the preoperative management of pheochromocytoma?
A) It is not important.
B) It should be decreased to prevent hypertension.
C) It should be increased to improve outcome.
D) It should be maintained to avoid hypotension.

Answer 106

C) It should be increased to improve outcome. Intravascular volume is crucial to successful outcome in the preoperative management of pheochromocytoma.

Explanation: Intravascular volume is crucial to successful outcome in the preoperative management of pheochromocytoma. Once alpha blockade is initiated, salt and fluid intake should be encouraged prior to surgery, and intravenous fluid resuscitation can be utilized the day prior to surgery to improve intravascular volume.

Question 107

Fig. 30.7 Preoperative medical management in patients with pheochromocytoma.

Partin, Alan W.; Kavoussi, Louis R.; Peters, Craig A.; Dmochowski, Roger R.. Campbell Walsh Wein Handbook of Urology - E-Book (p. 786). Elsevier Health Sciences. Kindle Edition.

Answer 107

Question 108

What are the potential postoperative complications that need to be monitored after surgical excision of pheochromocytoma?
A) Hypertension and hyperglycemia
B) Hypotension and hypoglycemia
C) Hypertension and hypoglycemia
D) Hypotension and hyperglycemia

Answer 108

B) Hypotension and hypoglycemia. Hypotension from the lasting effects of alpha blockade or hypoglycemia from the increase in insulin after tumor excision are potential postoperative complications that need to be monitored after surgical excision of pheochromocytoma.

Explanation: Hypotension can occur after surgical excision of pheochromocytoma due to the lasting effects of alpha blockade, which can lead to decreased vascular resistance. Hypoglycemia can also occur due to the increase in insulin after tumor excision. Both of these potential complications need to be monitored postoperatively.

Question 109

How can hypotension be managed in the postoperative period after surgical excision of pheochromocytoma?
A) Fluid restriction
B) Administration of beta blockers
C) Administration of vasopressors
D) Administration of insulin

Answer 109

C) Administration of vasopressors. Hypotension in the postoperative period after surgical excision of pheochromocytoma can be managed by the administration of vasopressors.

Explanation: If hypotension occurs in the postoperative period after surgical excision of pheochromocytoma, it can be managed by the administration of vasopressors, such as phenylephrine or norepinephrine. Fluids should also be administered to maintain intravascular volume.

Question 110

What are some of the causes of Addison’s disease?
A) High blood pressure and heart disease
B) Autoimmune adrenalitis, infections, and surgical excision
C) Lung cancer and emphysema
D) Kidney stones and urinary tract infections

Answer 110

B) Autoimmune adrenalitis, infections, and surgical excision. Causes of Addison’s disease include autoimmune adrenalitis, infections, and surgical excision.

Explanation: Addison’s disease is a disorder of decreased adrenal function, which can be caused by a number of factors, including autoimmune adrenalitis, infections (such as tuberculosis or HIV), and surgical excision of the adrenal glands.

Question 111

What are some of the clinical characteristics of Addison’s disease?
A) Hypertension and tachycardia
B) Fatigue, anorexia, and hyperpigmentation
C) Fever and respiratory distress
D) Abdominal pain and diarrhea

Answer 111

B) Fatigue, anorexia, and hyperpigmentation. Patients with Addison’s disease may have fatigue, anorexia, and hyperpigmentation, which is the hallmark of Addison’s crisis.

Explanation: Addison’s disease can cause a variety of symptoms, including fatigue, anorexia, and hyperpigmentation, which is the result of excess production of melanin. These symptoms can be nonspecific and may be mistaken for other conditions. In more severe cases, an Addison’s crisis may occur, which can cause life-threatening symptoms.

Question 112

What are some of the symptoms of an Addison’s crisis?
A) Headache and dizziness
B) Acute abdomen and nausea
C) Muscle weakness and joint pain
D) Blurred vision and hearing loss

Answer 112

B) Acute abdomen and nausea. An Addison’s crisis is life-threatening and may include acute abdomen, nausea, vomiting, fever, and hypovolemia.

Explanation: An Addison’s crisis is a life-threatening complication of Addison’s disease, which can be triggered by stress or other factors. Symptoms of an Addison’s crisis can include acute abdomen, nausea, vomiting, fever, and hypovolemia. Other symptoms may include confusion, lethargy, and even coma. Prompt medical attention is necessary to manage an Addison’s crisis.

Question 113

What is Adrenocortical carcinoma (ACC)?
A) A benign adrenal tumor
B) A rare and universally poor prognosis malignant adrenal tumor
C) A common and treatable form of adrenal tumor
D) A type of adrenal tumor that is associated with hyperaldosteronism

Answer 113

B) A rare and universally poor prognosis malignant adrenal tumor. Adrenocortical carcinoma (ACC) is a rare and universally poor prognosis malignant adrenal tumor.

Explanation: ACC is a rare and aggressive form of adrenal tumor that has a poor prognosis. It is considered a type of cancer that arises from the outer layer of the adrenal gland, called the adrenal cortex. It may be associated with a number of syndromes but is mostly sporadic, and >50% of patients have symptoms at the time of diagnosis. The most common symptoms are abdominal pain or fullness and symptoms related to increased adrenal hormone production.

Question 114

What are the most common symptoms of ACC?
A) Nausea and vomiting
B) Abdominal pain and fullness
C) Headaches and dizziness
D) Chest pain and shortness of breath

Answer 114

B) Abdominal pain and fullness. The most common symptoms of ACC are abdominal pain or fullness and symptoms related to increased adrenal hormone production.

Explanation: The most common symptoms of ACC are related to increased adrenal hormone production, which can cause a variety of symptoms depending on the specific hormone involved. The most common hormone involved is cortisol, which can lead to Cushing’s syndrome (CS), characterized by weight gain, high blood pressure, and other symptoms. The second most common hormone involved is increased androgens mainly 17-ketosteroids, which can lead to virilization, characterized by male-pattern baldness, hirsutism, and oligomenorrhea. Other symptoms of ACC may include nausea and vomiting, headaches, dizziness, chest pain, and shortness of breath, depending on the location and size of the tumor.

Question 115

What is the best test to identify an adrenal tumor?
A) Blood tests
B) Urine tests
C) Cross-sectional imaging
D) Endoscopy

Answer 115

C) Cross-sectional imaging. Cross-sectional imaging is the best test to identify an adrenal tumor.

Explanation: Cross-sectional imaging, such as computed tomography (CT) or magnetic resonance imaging (MRI), is the most accurate way to identify an adrenal tumor. These tests can provide detailed images of the adrenal gland and surrounding structures, allowing doctors to assess the size, location, and characteristics of the tumor. Other tests, such as blood and urine tests, can also be helpful in evaluating adrenal function and detecting certain types of hormone-producing tumors, but imaging is the most important test for identifying an adrenal tumor.

Question 116

What is the typical size of an ACC tumor?
A) Less than 1 cm
B) 1-3 cm
C) 3-5 cm
D) Greater than 5 cm

Answer 116

D) Greater than 5 cm. ACC tumors typically present large, with 90% greater than 5 cm, and the majority greater than 10-12 cm.

Explanation: ACC tumors are typically large, with most tumors greater than 10-12 cm in size. In fact, 90% of ACC tumors are greater than 5 cm in size. This is in contrast to most adrenal tumors, which are small and often discovered incidentally on imaging tests. The large size of ACC tumors can make them more challenging to treat, and many patients may require surgery to remove the tumor and surrounding tissue.

Question 117

What is the recommended management approach for ACCs larger than 8 cm?
A) Open surgery
B) Laparoscopic surgery
C) Radiation therapy
D) Chemotherapy

Answer 117

A) Open surgery. ACCs larger than 8 cm should be considered for open surgery.

Explanation: ACCs are often large and aggressive, and surgical removal is the mainstay of treatment. For tumors larger than 8 cm, open surgery is generally recommended, as this allows for more complete removal of the tumor and surrounding tissue. In some cases, adjacent organs or tissues may also need to be removed. For smaller tumors, laparoscopic or robotically assisted procedures may be considered, as these are less invasive and can result in faster recovery times.

Question 118

What is neuroblastoma?

Answer 118

Neuroblastoma is a malignancy that arises from the neural crest cells, which give rise to the adrenal medulla and sympathetic ganglia. It is the most common solid extracranial tumor of childhood.

Explanation: Neuroblastoma is a cancer that arises from immature nerve cells, usually in the adrenal glands but also in other areas of the body, such as the neck, chest, or pelvis. It most commonly affects young children, with a median age at diagnosis of 17 months. The symptoms of neuroblastoma depend on the location and size of the tumor and may include abdominal distension, pain, weight loss, fever, and bone pain. Treatment for neuroblastoma usually involves a combination of surgery, chemotherapy, and radiation therapy, depending on the stage of the disease and other factors. The prognosis for neuroblastoma can vary widely depending on these factors, with some cases having a good prognosis and others being more difficult to treat.

Question 119

Fig. 30.8 Summary of evaluation of adrenal mass using modern cross-sectional imaging.

Partin, Alan W.; Kavoussi, Louis R.; Peters, Craig A.; Dmochowski, Roger R.. Campbell Walsh Wein Handbook of Urology - E-Book (p. 789). Elsevier Health Sciences. Kindle Edition.

Answer 119

Question 120

What is the most common type of adrenal tumor?
A) Adenoma
B) Neuroblastoma
C) Adrenocortical carcinoma
D) Pheochromocytoma

Answer 120

A) Adenoma. Adenoma is the most common type of adrenal tumor.

Explanation: An adenoma is a common mass found in the adrenal gland, which is usually nonfunctional, meaning that it does not produce hormones. Adenomas are the most common type of adrenal tumor and are often discovered incidentally during cross-sectional imaging. They are usually well-circumscribed, homogeneous, and have a high lipid content, which leads to an attenuation of <10 HU on unenhanced CT. In contrast, functional tumors, such as pheochromocytomas and adrenocortical carcinomas, often produce hormones and can cause symptoms.

Question 121

What is the most valuable diagnostic tool for detecting an adrenal adenoma?
A) MRI
B) PET scan
C) Noncontrast CT
D) Ultrasound

Answer 121

C) Noncontrast CT. Noncontrast CT is the most valuable diagnostic tool for detecting an adrenal adenoma.

Explanation: Noncontrast CT is the most commonly used imaging modality for detecting adrenal adenomas. Adenomas are usually <4 cm, well-rounded, and homogeneous, and have a high lipid content, which leads to an attenuation of <10 HU on unenhanced CT. When IV contrast is used, lesions that wash out >50% of gained enhancement on delayed films are usually benign adenomas. MRI, PET scan, and ultrasound may also be used to detect adrenal tumors, but noncontrast CT is the most valuable diagnostic tool for detecting an adrenal adenoma.

Question 122

What is a myelolipoma?

Answer 122

A myelolipoma is a nonfunctional fat-containing tumor of the adrenal gland that usually does not require intervention unless it is larger than 4-5 cm.

Explanation: A myelolipoma is a rare, benign tumor of the adrenal gland that is composed of mature fat cells and hematopoietic elements, such as red blood cells and bone marrow cells. It is a nonfunctional tumor, meaning that it does not produce hormones, and it is usually discovered incidentally during cross-sectional imaging. Myelolipomas are usually small and asymptomatic, and no treatment is required unless they are larger than 4-5 cm or cause symptoms. In these cases, surgical excision may be considered. Myelolipomas are generally considered to have a good prognosis, as they are benign and do not typically recur after surgical removal.

Question 123

At what size should adrenal tumors be considered for excision in urologic practice?

A. Less than 2 cm
B. Less than 4 cm
C. Greater than 4 cm
D. Greater than 6 cm

Answer 123

C. Greater than 4 cm.

Explanation: According to the material, adrenal tumors larger than 4 cm should be strongly considered for excision whether functional or not. Tumors smaller than 4 cm generally do not require intervention unless they are functional. Therefore, options A and B are incorrect, and option D is outside of the range provided in the material.

Question 124

Fig. 30.9 Testing algorithm for ruling out hypercortisolemia secondary to an adrenal mass. In case of a positive result during late-night salivary cortisol or 24-hour urinary free cortisol evaluations, repeat testing is often prudent.

Partin, Alan W.; Kavoussi, Louis R.; Peters, Craig A.; Dmochowski, Roger R.. Campbell Walsh Wein Handbook of Urology - E-Book (p. 791). Elsevier Health Sciences. Kindle Edition.

Answer 124

Question 125

Table 30.4 Characteristics of Incidental Adrenal Masses as Described in a Systematic Review of Published Series of Adrenal Incidentalomas That Include 20 or More Patients

Partin, Alan W.; Kavoussi, Louis R.; Peters, Craig A.; Dmochowski, Roger R.. Campbell Walsh Wein Handbook of Urology - E-Book (p. 791). Elsevier Health Sciences. Kindle Edition.

Answer 125

Question 126

What are the hormones that should be tested to assess the functionality of an adrenal mass?
a. Cortisol, insulin, and aldosterone
b. Cortisol, catecholamine, and aldosterone
c. Estrogen, progesterone, and testosterone
d. Melatonin, dopamine, and epinephrine

Answer 126

b. Cortisol, catecholamine, and aldosterone. These are the three hormones that should be tested to assess the functionality of an adrenal mass.

Question 127

What is the test used to diagnose hypercortisolism?
a. Serum cortisol test
b. 24-hour urine cortisol test
c. Late-night salivary cortisol test
d. All of the above

Answer 127

d. All of the above. The serum cortisol test, 24-hour urine cortisol test, and late-night salivary cortisol test are all used to diagnose hypercortisolism.

Question 128

What is the diagnostic criteria for Conn syndrome?
a. An aldosterone-to-renin ratio (ARR) of ≥20 along with serum aldosterone >15 ng/mL
b. A cortisol-to-renin ratio (CRR) of ≥20 along with serum cortisol >15 ng/mL
c. A catecholamine-to-renin ratio (CAR) of ≥20 along with serum catecholamines >15 ng/mL
d. None of the above

Answer 128

a. An aldosterone-to-renin ratio (ARR) of ≥20 along with serum aldosterone >15 ng/mL is the diagnostic criteria for Conn syndrome.

Question 129

What is a contraindication for adrenal surgery?

A) Functional tumors
B) Tumors >6 cm
C) Severe cardiopulmonary morbidity precluding anesthesia
D) Equivocal tumors

Answer 129

C) Severe cardiopulmonary morbidity precluding anesthesia

Explanation: Adrenal surgery is contraindicated in patients with severe cardiopulmonary morbidity that precludes anesthesia, as well as in those with uncorrected coagulopathy and widespread metastatic disease. The indications for adrenal surgery include functional tumors that fail medical therapy, suspicion of malignancy, tumors larger than 6 cm, and tumors between 4-6 cm that are equivocal or symptomatic.

Question 130

Fig. 30.10 Recommended surgical approach for nonmetastatic primary adrenocortical carcinoma.

Answer 130

Question 131

What is the optimal approach for adrenocortical carcinoma (ACC) surgery?
A. Open surgery
B. Laparoscopic or robotic surgery
C. Both A and B
D. None of the above

Answer 131

C. Both A and B.

Explanation: The optimal approach for ACC surgery is a subject of ongoing debate. Tumors >6 cm that are most certainly ACC might want to be addressed with open surgery, whereas smaller tumors or tumors that are equivocal can be removed with minimally invasive surgery, ideally transperitoneal. Both laparoscopic or robotic surgery and open surgery have their advantages and disadvantages, and the decision on which approach to use should be made on a case-by-case basis.

Question 132

Which tumors should be addressed with open surgery for ACC?
A. Tumors >6 cm
B. Tumors <6 cm
C. Tumors of any size
D. None of the above

Answer 132

A. Tumors >6 cm.

Explanation: Tumors >6 cm that are most certainly ACC might want to be addressed with open surgery. This is because open surgery allows for better visualization and tactile feedback, which can be important in cases where the tumor is large or the diagnosis is uncertain.

Question 133

Which type of surgery is preferred for smaller or equivocal ACC tumors?
A. Open surgery
B. Laparoscopic or robotic surgery
C. Both A and B
D. None of the above

Answer 133

B. Laparoscopic or robotic surgery.

Explanation: Smaller tumors or tumors that are equivocal can be removed with minimally invasive surgery, ideally transperitoneal. Laparoscopic or robotic surgery is preferred for these cases because it offers several advantages, including less postoperative pain, shorter hospital stays, and faster recovery times.

Question 134

What should be included in the ACC surgery, regardless of the approach used?
A. Lymph node dissection
B. Tumor spillage
C. Both A and B
D. None of the above

Answer 134

A. Lymph node dissection.

Explanation: Regardless of the approach used, ACC surgery should include a lymph node dissection to determine the extent of disease and to help guide adjuvant therapy. Tumor spillage should be avoided during surgery to prevent the dissemination of cancer cells and the potential for recurrence.

Question 135

What are the advantages of the open anterior transabdominal or thoracoabdominal approach for adrenalectomy?
A. Less ileus and shorter hospitalizations
B. Smaller operative fields
C. Excellent surgical exposure and better vascular access and control
D. Less major organ injury

Answer 135

C. Excellent surgical exposure and better vascular access and control.

Explanation: The open anterior transabdominal or thoracoabdominal approach provides excellent surgical exposure and better vascular access and control compared to other approaches. However, it comes at the expense of ileus and major organ injury.

Question 136

What is a disadvantage of the open anterior transabdominal or thoracoabdominal approach for adrenalectomy?
A. Smaller operative fields
B. Less ileus and shorter hospitalizations
C. Major organ injury
D. None of the above

Answer 136

C. Major organ injury.

Explanation: One of the disadvantages of the open anterior transabdominal or thoracoabdominal approach is major organ injury. This is because the approach requires extensive dissection through the abdominal cavity, which can result in injury to other organs.

Question 137

What is an advantage of the retroperitoneal approach for adrenalectomy?
A. Smaller operative fields
B. Less ileus and shorter hospitalizations
C. Excellent surgical exposure and better vascular access and control
D. Better for obese patients

Answer 137

D. Better for obese patients.

Explanation: The retroperitoneal approach, which includes the flank and lumbodorsal approaches, has smaller operative fields but has less ileus and shorter hospitalizations compared to the anterior transabdominal or thoracoabdominal approach. This approach may be better for the obese patient.

Question 138

What is a disadvantage of the retroperitoneal approach for adrenalectomy?
A. Major organ injury
B. Less ileus and shorter hospitalizations
C. Poor surgical exposure
D. None of the above

Answer 138

C. Poor surgical exposure.

Explanation: One of the disadvantages of the retroperitoneal approach for adrenalectomy is poor surgical exposure. This approach provides limited visualization of the surrounding structures, making it more difficult to identify and avoid injury to other organs.

Question 139

What position is the patient placed in for a flank retroperitoneal approach for adrenalectomy?
A. Supine position
B. Prone position
C. Lateral decubitus position
D. Trendelenburg position

Answer 139

C. Lateral decubitus position.

Explanation: The patient is placed in a lateral decubitus position with the table flexed and the kidney rest elevated for a flank retroperitoneal approach for adrenalectomy.

Question 140

What is an axillary roll used for in a flank retroperitoneal approach for adrenalectomy?
A. To support the patient’s head and neck
B. To elevate the patient’s leg
C. To prevent the patient from rolling over
D. To prevent brachial plexus injury

Answer 140

D. To prevent brachial plexus injury.

Explanation: An axillary roll is placed for a flank retroperitoneal approach to prevent brachial plexus injury. The ipsilateral arm is placed over the opposite arm with the elbow slightly flexed.

Question 141

What is the purpose of early ligation of the adrenal vein during adrenalectomy for functional tumors?
A. To prevent traumatic handling of the gland
B. To identify the adrenal gland and the renal vein
C. To prevent injury to the great vessels
D. To prevent the release of catecholamines into the circulation

Answer 141

D. To prevent the release of catecholamines into the circulation.

Explanation: Early ligation of the adrenal vein is optimal for functional tumors to prevent the release of catecholamines into the circulation, which can cause a hypertensive crisis. This is because the adrenal vein is responsible for carrying hormones produced by the adrenal gland, including catecholamines.

Question 142

ow is the incision closed after a flank retroperitoneal adrenalectomy?
A. In one layer
B. In two layers
C. With surgical staples
D. With sutures and surgical glue

Answer 142

B. In two layers.

Explanation: The incision for a flank retroperitoneal adrenalectomy is closed in two layers, and the skin is closed. This is to ensure proper wound healing and to minimize the risk of complications such as infection or dehiscence.

Question 143

Fig. 30.11 Surgical incision over 11th rib for flank adrenalectomy. The patient is in flexion, with the kidney rest deployed to maximally expose the right retroperitoneum.

Answer 143

Question 144

Fig. 30.12 Flank approach. Incision of muscle (m.) overlying 11th rib.

Answer 144

Question 145

Fig. 30.13 Flank approach. Excision of the 11th rib.

Answer 145

Question 146

Fig. 30.14 Flank approach. Mobilization of the intercostal neurovascular bundle from the 11th rib. This is performed with a combination of blunt dissection with a Kittner dissector and sharp dissection with Metzenbaum scissors.

Answer 146

Question 147

What is the posterior lumbodorsal approach used for in adrenalectomy?
A. To provide direct access to the adrenal glands
B. To minimize surgical incisions
C. To avoid injury to major blood vessels
D. To address large tumors or ACC

Answer 147

A. To provide direct access to the adrenal glands.

Explanation: The posterior lumbodorsal approach is used to provide direct access to the adrenal glands during adrenalectomy. However, exposure is severely limited, and this approach is not suitable for large tumors or ACC.

Question 148

What type of tumors is the posterior lumbodorsal approach not suitable for in adrenalectomy?
A. Functional tumors
B. Small tumors
C. Large tumors or ACC
D. Tumors with no symptoms

Answer 148

C. Large tumors or ACC.

Explanation: The posterior lumbodorsal approach is not suitable for large tumors or ACC in adrenalectomy due to the limited exposure provided by this approach.

Question 149

What is an advantage of the posterior lumbodorsal approach in adrenalectomy?
A. Minimizes surgical incisions
B. Avoids injury to major blood vessels
C. Provides direct access to the adrenal glands
D. Shortens hospital stays
Answer: C. Provides direct access to the adrenal glands.

Answer 149

C. Provides direct access to the adrenal glands.

Explanation: The main advantage of the posterior lumbodorsal approach is that it provides direct access to the adrenal glands during adrenalectomy. This can be particularly useful for cases of bilateral hyperplasia.

Question 150

When might the posterior lumbodorsal approach be used in adrenalectomy?
A. For small, benign tumors
B. For large, malignant tumors
C. For bilateral hyperplasia
D. For tumors located in the peritoneal cavity

Answer 150

C. For bilateral hyperplasia.

Explanation: The posterior lumbodorsal approach might be used in adrenalectomy for cases of bilateral hyperplasia, where both adrenal glands need to be accessed. However, this approach is not suitable for large tumors or ACC due to the limited exposure it provides.

Question 151

Fig. 30.15 Posterior approach—possible locations for lumbodorsal incisions.

Answer 151

Question 152

Fig. 30.16 Bilateral posterior approach—anatomic relations to the adrenal gland as seen from behind. v, Vein.

Answer 152

Question 153

When is the anterior transabdominal approach used in adrenalectomy?
A. For small tumors
B. For large tumors requiring adequate exposure, caval involvement, or extensive nodal dissection
C. For tumors in the posterior lumbodorsal area
D. For tumors that have not spread to nearby organs

Answer 153

B. For large tumors requiring adequate exposure, caval involvement, or extensive nodal dissection.

Explanation: The anterior transabdominal approach is used in adrenalectomy for large tumors that require adequate exposure, caval involvement, or extensive nodal dissection.

Question 154

What are the different approaches for the anterior transabdominal approach in adrenalectomy?
A. Subcostal, chevron, or midline
B. Flank and lumbodorsal
C. Retroperitoneal and transperitoneal
D. Anterior and posterior

Answer 154

A. Subcostal, chevron, or midline.

Explanation: The different approaches for the anterior transabdominal approach in adrenalectomy are subcostal, chevron, or midline.

Question 155

What organs need to be approached with caution during adrenalectomy with the anterior transabdominal approach on the left side?
A. Liver, gallbladder, and pancreas
B. Spleen, pancreas, and kidney
C. Spleen, liver, and stomach
D. Pancreas, stomach, and colon

Answer 155

B. Spleen, pancreas, and kidney.

Explanation: During adrenalectomy with the anterior transabdominal approach on the left side, the spleen is reflected medially, and the adrenal can be seen just posterior and inferior to the splenic artery and lateral to the pancreas. The gland needs to be dissected out with caution of the spleen, pancreas, and kidney.

Question 156

How is the right adrenal approached with the anterior transabdominal approach in adrenalectomy?
A. By retracting the spleen
B. By releasing the hepatic flexure of the colon
C. By retracting the pancreas
D. By releasing the duodenum

Answer 156

B. By releasing the hepatic flexure of the colon.

Explanation: The right adrenal is approached with the anterior transabdominal approach in adrenalectomy by retracting the liver, releasing the hepatic flexure of the colon, Kocherizing the duodenum, and identifying the gland just lateral to the IVC and just superior to the right renal vein.

Question 157

Fig. 30.17 Anterior approach. The transperitoneal approach may be attempted through a midline incision or subcostal incision. The subcostal incision can be extended into a full chevron for bilateral adrenalectomy or if a large unilateral tumor is encountered.

Answer 157

Question 158

Fig. 30.19 Anterior approach. The left adrenal vein is dissected out and ligated.

Answer 158

Question 159

Fig. 30.20 Kocher maneuver. The peritoneum is incised, and sharp dissection and blunt dissection are used to mobilize the second stage of the duodenum away from the renal hilum.

Answer 159

Question 160

What are the different minimally invasive surgical approaches used for adrenal surgery?
A. Laparoscopic, open, and robotic
B. Laparoscopic, robotic, single-site, and natural orifice
C. Open, retroperitoneal, and transperitoneal
D. Flank and lumbodorsal

Answer 160

B. Laparoscopic, robotic, single-site, and natural orifice.

Explanation: The different minimally invasive surgical approaches used for adrenal surgery are laparoscopic (trans- and retroperitoneal approaches), robotic, single-site (LESS), and natural orifice (NOTES) techniques.

Question 161

What is the emerging gold standard for adrenal surgery?
A. Open surgery
B. Flank retroperitoneal approach
C. Minimally invasive surgery
D. Posterior lumbodorsal approach

Answer 161

C. Minimally invasive surgery.

Explanation: Minimally invasive surgery, including laparoscopic, robotic, single-site, and natural orifice techniques, is emerging as the gold standard for adrenal surgery.

Question 162

What is the advantage of minimally invasive surgical approaches for adrenal surgery?
A. Better exposure and vascular control
B. Lower risk of complications and shorter hospital stays
C. Greater access to surrounding structures
D. Ability to perform extensive nodal dissection

Answer 162

B. Lower risk of complications and shorter hospital stays.

Explanation: The main advantage of minimally invasive surgical approaches for adrenal surgery is the lower risk of complications and shorter hospital stays compared to open surgery. These approaches also generally result in less postoperative pain and a faster recovery time for the patient.

Question 163

Fig. 30.21 Four-trocar configuration for left transperitoneal laparoscopic adrenalectomy.

Answer 163

Question 164

Fig. 30.22 Transperitoneal laparoscopic adrenalectomy. Incision of the line of Toldt and medial dissection of the left colon with cautery endoscopic scissors.

Answer 164

Question 165

Fig. 30.23 Transperitoneal laparoscopic adrenalectomy. Exposure and dissection of the renal vein and left adrenal vein.

Answer 165

Question 166

Fig. 30.24 Transperitoneal laparoscopic adrenalectomy. Ligation and division of left adrenal vein.

Answer 166

Question 167

Fig. 30.25 Transperitoneal laparoscopic adrenalectomy. Division of adrenal arterial supply and superomedial dissection with downward traction on the kidney.

Answer 167

Question 168

Fig. 30.26 Transperitoneal laparoscopic adrenalectomy. The adrenal gland is mobilized off the medial aspect of the kidney.

Answer 168

Question 169

Fig. 30.28 Left robotic adrenalectomy trocar placement. A total of four ports are placed: one 12-mm camera port (●), one 12-mm assistant port ( ), and two 8-mm robotic arm ports ( ). The distance between each port should be at least 8 cm.

Answer 169

Question 170

Fig. 30.29 Right robotic adrenalectomy trocar placement. A total of five ports are used: one 12-mm camera port (●), one 12-mm assistant port ( ), two 8-mm robotic arm ports ( ) are established, and to retract the liver, a 5-mm trocar (○) is placed with a retraction device.

Answer 170

Question 171

Fig. 30.30 Surgical room setup for left robotic adrenalectomy. The slave unit of the robot is brought in over the patient’s left shoulder as indicated in the diagram.

Answer 171

Question 172

Fig. 30.31 Robot-assisted posterior retroperitoneal adrenalectomy. Camera port; 8-mm robotic ports; assistant port.

Answer 172

Question 173

In what types of patients should partial adrenalectomy be considered?
A. Patients with functional tumors
B. Patients with large tumors
C. Patients with bilateral adrenal tumors, solitary adrenal gland, or familial syndromes
D. Patients with tumors that have metastasized

Answer 173

C. Patients with bilateral adrenal tumors, solitary adrenal gland, or familial syndromes such as von Hippel-Lindau disease, familial pheochromocytoma, and multiple endocrine neoplasia type IIA.

Explanation: Partial adrenalectomy should be considered in patients with bilateral adrenal tumors, solitary adrenal gland, or familial syndromes such as von Hippel-Lindau disease, familial pheochromocytoma, and multiple endocrine neoplasia type IIA.

Question 174

Why is intraoperative ultrasound important in partial adrenalectomy?
A. To assess the extent of tumor involvement
B. To monitor blood flow to the adrenal gland
C. To identify the optimal incision site
D. To localize the tumor for accurate incision

Answer 174

D. To localize the tumor for accurate incision.

Explanation: Intraoperative ultrasound is important in partial adrenalectomy to localize the tumor for accurate incision. This helps to ensure that only the affected portion of the adrenal gland is removed, while leaving the healthy tissue intact.

Question 175

Box 30.2 Indications for Partial Adrenalectomy

Answer 175

Question 176

Which of the following techniques is used in ablative therapy for adrenal disease?
a. Laser ablation
b. Chemotherapy
c. Cryoablation
d. Radiation therapy

Answer 176

c. Cryoablation is one of the techniques used in ablative therapy for adrenal disease, along with microwave and radiofrequency ablation.

Explanation: Ablative therapy is a minimally invasive procedure that uses extreme cold or heat to destroy abnormal tissue in the body, such as tumors in the adrenal glands. Cryoablation uses extremely low temperatures to freeze and destroy the tumor. Microwave and radiofrequency ablation use heat to destroy the tumor.

Question 177

What is the recommended size limit for tumors that can be treated with microwave, radiofrequency, and cryoablation techniques?
a. 2 cm
b. 4 cm
c. 6 cm
d. 8 cm

Answer 177

b. Tumors larger than 4 cm are not recommended for treatment with microwave, radiofrequency, and cryoablation techniques.

Explanation: While ablative therapy can be effective for treating adrenal tumors, the size of the tumor is an important consideration. Larger tumors may be more difficult to treat with these techniques and may require surgery instead. The recommended size limit for treatment with microwave, radiofrequency, and cryoablation techniques is 4 cm.

Question 178

Which type of adrenal tumor is not recommended for treatment with microwave, radiofrequency, and cryoablation techniques?
a. Adenoma
b. Pheochromocytoma
c. Neuroblastoma
d. Metastatic tumor

Answer 178

b. Pheochromocytoma is not recommended for treatment with microwave, radiofrequency, and cryoablation techniques.

Explanation: Pheochromocytoma is a rare type of adrenal tumor that can produce high levels of hormones that can cause high blood pressure, rapid heartbeat, and other symptoms. This type of tumor is not recommended for treatment with microwave, radiofrequency, and cryoablation techniques because the release of hormones during the destruction of the tumor can lead to dangerous complications. Surgery is the preferred treatment for pheochromocytoma.

Question 179

What type of electrolyte disturbance can occur after adrenal surgery?
a. Hyperkalemia
b. Hypocalcemia
c. Hypomagnesemia
d. Hypernatremia

Answer 179

a. Hyperkalemia is a potential electrolyte disturbance that can occur after adrenal surgery.

Explanation: Adrenal glands produce hormones that regulate electrolyte levels in the body, and their removal during surgery can disrupt this balance. Hyperkalemia, which is an abnormally high level of potassium in the blood, is one of the potential electrolyte disturbances that can occur after adrenal surgery. Other potential electrolyte imbalances include hypocalcemia, hypomagnesemia, and hyponatremia.

Question 180

Box 30.3 Intraoperative Complications of Adrenal Surgery

Answer 180

Question 181

Box 30.4 Postoperative Complications of Adrenal Surgery

Answer 181

Question 182

30% of adenomas are lipid-poor and don’t exhibit signal drop out on MRI or <10 HU on CT. In this case, what should be obtained?

Answer 182

CT Washout imaging (15 minutes post-contrast). > 60% absolute washout and >40% relative washout highly specific for lipid-poor adenoma (Cancer is greedy and will hang onto the contrast)

Question 183

What masses of the adrenal should undergo metabolic evaluation?

Answer 183

All adrenal masses > 1cm

Question 184

What are the steps of a low dose dexamethasone suppression test?

Answer 184

• 1mg dexamethasone at 11pm (stop oral contraceptives due to false positives) to suppress ACTH secretion from the anterior pituitary
• check morning cortisol; <5 mcg/dL rules out ACTH-independent glucocorticoid secretion by an adrenal nodule, which wouldn’t respond to ACTH suppression

Question 185

What are alternatives to low dose DST?

Answer 185

late night (11pm) salivary cotrsiol testing and 24-hour urinary cortisol

Question 186

Once hypercortisolism has been diagnosed, what is the next step?

Answer 186

Late-afternoon serum ACTH to determine if the process is ACTH-dependent (>10 pg/ML) or ACTH-independent (< 5pg/mL)

Question 187

How do you screen for pheochromocytoma?

Answer 187

Plasma-free metanephrines can be drawn at the same time as morning cortisol after DST. No caffeine for 24 hours. No Tylenol for 5 days. Draw in supine position after 20 minutes rest. 4x normal is definitive. The other option is 24-hour total urinary fractionated metanephrines.

Question 188

If adrenal mass >1cm and hypertension or hypokalemia, then what should be tested?

Answer 188

Screen for Conn’s Syndrome
-obtain morning/sitting plasma aldosterone (PAC) > 15 ng/ML = Conn’s syndrome
-Renin levels (PRA or ARR) > 20-30 = Conn’s Syndrome

If Positive then confirmatory test
-24-hour urine stuy with salt loading (urine aldosterone > 14 mcg and urine potassium > 30 meq/24h are confirmatory)

Adrenal vein sampling prior to adrenalectomy

Question 189

If strong suspicion for ACC (stigmata of feminization or virilization) then what should be tested for?

Answer 189

Sex steroids (dehydroepiandrosterone DHEA-S, androstenedione)

Question 190

What surveillance is necessary for adrenal masses?

Answer 190

2 years, CT 6, 12, 24 months

Question 191

What is mitotane?

Answer 191

c. Adrenocortical carcinoma

Explanation: Mitotane is a medication used in the treatment of adrenocortical carcinoma, a rare form of cancer that affects the adrenal glands. It is a steroidogenesis inhibitor and is cytotoxic for the adrenal cortex. It has a palliative benefit in metastatic ACC.

Question 192

What condition is metyrosine used to treat?
a. Type 1 diabetes
b. High blood pressure
c. Asthma
d. Pheochromocytoma

Answer 192

d. Pheochromocytoma

Explanation: Metyrosine is used in the treatment of pheochromocytoma, a type of tumor that occurs in the adrenal glands and can cause overproduction of certain hormones, leading to high blood pressure and other symptoms. It is used if alpha-blockade is insufficient because tyrosine is a precursor molecule in catecholemine synthesis.

Question 193

How does metyrosine work?
a. By increasing the production of neurotransmitters
b. By inhibiting the production of neurotransmitters
c. By blocking the receptors for neurotransmitters
d. By stimulating the growth of nerve cells

Answer 193

b. By inhibiting the production of neurotransmitters

Explanation: Metyrosine works by inhibiting the production of certain neurotransmitters, including dopamine, norepinephrine, and epinephrine, which are produced in excessive amounts in people with pheochromocytoma. It is a tyrosine hydroxylase inhibitor and anti-HTN.

Question 194

What are the side effects of mitotane?
a. Drowsiness, dizziness, headache, and diarrhea
b. Nausea, vomiting, loss of appetite, and fatigue
c. Liver damage and allergic reactions
d. None of the above

Answer 194

b. Nausea, vomiting, loss of appetite, and fatigue

Explanation: Mitotane can cause side effects such as nausea, vomiting, loss of appetite, and fatigue. However, it can also cause more serious side effects such as liver and kidney damage.

Question 195

What is metyrapone used for?
a. High blood pressure
b. Type 2 diabetes
c. Cushing’s syndrome
d. Asthma

Answer 195

c. Cushing’s syndrome

Explanation: Metyrapone is used in the diagnosis and treatment of Cushing’s syndrome, a condition that occurs when the body produces too much cortisol. It is used if hypercortisolemia persists after adrenalectomy to decrease cortisol production by the contralateral kidney.

Question 196

How does metyrapone work?
a. By increasing the production of cortisol
b. By inhibiting the production of cortisol
c. By blocking the receptors for cortisol
d. By stimulating the growth of adrenal cells

Answer 196

b. By inhibiting the production of cortisol

Explanation: Metyrapone works by blocking an enzyme called 11-beta-hydroxylase, which is involved in the production of cortisol in the adrenal glands.

Question 197

What are some side effects of metyrapone?
a. Dizziness, headache, and nausea
b. Skin rash and vomiting
c. Changes in blood pressure, glucose levels, and electrolyte levels
d. All of the above

Answer 197

d. All of the above

Explanation: Metyrapone can cause side effects such as dizziness, headache, nausea, skin rash, and vomiting. It can also cause changes in blood pressure, glucose levels, and electrolyte levels, so it is important for patients taking metyrapone to have regular blood tests and monitoring of their vital signs.

Question 198

What type of diuretic should be used for peri-operative management for adrenalectomy in Conn’s syndrome?
a. Thiazide diuretics
b. Loop diuretics
c. Potassium-sparing diuretics
d. Osmotic diuretics

Answer 198

c. Potassium-sparing diuretics

Explanation: Patients with Conn’s syndrome require potassium-sparing diuretics such as amiloride, triamterene, spironolactone, or eplerenone for peri-operative management for adrenalectomy.

Question 199

What are potential medical therapies for Nelson’s syndrome?

Answer 199

Octreotide, temozolomide, pasireotide

Question 200

What is used to find ectopic steroid producing tumors?

Answer 200

Iodocholesterol

Question 201

What is one test to differentiate between pheochromocytoma and essential HTN?

Answer 201

Oral clonidine test which should drop norepinephrine significantly in essential HTN only.

Question 202

What is the classic triad of adrenal hemorrhage? What imaging? What treatment?

Answer 202

Mass, anemia, jaundice, males can have scrotal hemorrhage. U/S to exclude neuroblastoma.

Question 203

What are important history questions to ask in a patient with adrenal mass?

Answer 203

Weight gain or loss
Sweating episodes
Headaches
Palpitations
Bruisability
Muscular weakness
HTN
Flushing