30: Pathophysiology, evaluation, and management of adrenal disorders Flashcards

Question

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 1

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.

Answer 2

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.

Answer 3

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.

Answer 4

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.

Answer 5

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

Answer 6

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.

Answer 7

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).

Answer 8

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

Answer 9

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.

Answer 10

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.

Answer 11

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.

Answer 12

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.

Answer 13

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.

Answer 14

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

Answer 15

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.

Answer 16

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.

Answer 17

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

Answer 18

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

Answer 19

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

Answer 20

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.

Answer 21

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.

Answer 22

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

Answer 23

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

Answer 24

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

Answer 25

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.

Answer 26

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

Answer 27

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

Answer 28

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

Answer 29

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.

Answer 30

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

Answer 31

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.

Answer 32

A. Virtually all patients present with refractory HTN.

Answer 33

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

Answer 34

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.

Answer 35

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

Answer 36

B. Hyperaldosteronism increases the risk of atrial fibrillation.

Answer 37

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

Answer 38

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

Answer 39

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

Answer 40

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

Answer 41

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.

Answer 42

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

Answer 43

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.

Answer 44

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

Answer 45

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.

Answer 46

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

Answer 47

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."

Answer 48

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."

Answer 49

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."

Answer 50

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

Answer 51

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)."

Answer 52

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

Answer 53

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

Answer 54

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

Answer 55

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.

Answer 56

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.

Answer 57

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.

Answer 58

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.

Answer 59

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

Answer 60

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

Answer 61

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

Answer 62

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.

Answer 63

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.

Answer 64

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.

Answer 65

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.

Answer 66

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.

Answer 67

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.

Answer 68

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.

Answer 69

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.

Answer 70

: 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.

Answer 71

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.

Answer 72

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.

Answer 73

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.

Answer 74

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.

Answer 75

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.

Answer 76

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.

Answer 77

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.

Answer 78

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.

Answer 79

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.

Answer 80

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.

Answer 81

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.

Answer 82

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.

Answer 83

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.

Answer 84

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.

Answer 85

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.

Answer 86

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.

Answer 87

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.

Answer 88

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.

Answer 89

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.

Answer 90

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

Answer 91

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.

Answer 92

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

Answer 93

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.

Answer 94

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.

Answer 95

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.

Answer 96

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.

Answer 97

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.

Answer 98

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.

Answer 99

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.

Answer 100

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.

Answer 101

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.

Answer 102

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.

Answer 103

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.

Answer 104

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.

Answer 105

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.

Answer 106

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.

Answer 107

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.

Answer 108

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.

Answer 109

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.

Answer 110

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.

Answer 111

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

Answer 112

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.

Answer 113

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.

Answer 114

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.

Answer 115

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.

Answer 116

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.

Answer 117

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.

Answer 118

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.

Answer 119

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.

Answer 120

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.

Answer 121

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.

Answer 122

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.

Answer 123

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)

Answer 124

All adrenal masses > 1cm

Answer 125

- 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

Answer 126

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

Answer 127

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

Answer 128

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.

Answer 129

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

Answer 130

Sex steroids (dehydroepiandrosterone DHEA-S, androstenedione)

Answer 131

2 years, CT 6, 12, 24 months

Answer 132

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.

Answer 133

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.

Answer 134

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.

Answer 135

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.

Answer 136

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.

Answer 137

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.

Answer 138

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.

Answer 139

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.

Answer 140

Octreotide, temozolomide, pasireotide

Answer 141

Iodocholesterol

Answer 142

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

Answer 143

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

Answer 144

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

30: Pathophysiology, evaluation, and management of adrenal disorders Flashcards

(203 cards)