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Section II General Surgery P203Surgical Recall Sixth > Endocrine, C57 P418-437 > Flashcards

Endocrine, C57 P418-437 Flashcards

1
Q 
ADRENAL GLAND
ANATOMY
Where is the drainage of the
left adrenal vein?
P418
A

Left renal vein

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2
Q 
ADRENAL GLAND
ANATOMY
Where is the drainage of the
right adrenal vein?
P418
A

Inferior vena cava (IVC)

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3
Q

ADRENAL GLAND
NORMAL ADRENAL PHYSIOLOGY
What is CRH?
P419

A

Corticotropin-Releasing Hormone:
released from anterior hypothalamus and
causes release of ACTH from anterior
pituitary

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4
Q

ADRENAL GLAND
NORMAL ADRENAL PHYSIOLOGY
What is ACTH?
P419

A

AdrenoCorticoTropic Hormone: released
normally by anterior pituitary, which in
turn causes adrenal gland to release
cortisol

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5
Q 
ADRENAL GLAND
NORMAL ADRENAL PHYSIOLOGY
What feeds back to inhibit
ACTH secretion?
P419
A

Cortisol

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6
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
CUSHING’S SYNDROME
What is Cushing’s syndrome?
P419
A

Excessive cortisol production (Think:

Cushing’s = Cortisol

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7
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What is the most common
cause? 
P419
A

Iatrogenic (i.e., prescribed prednisone)

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8
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What is the second most
common cause?
P419
A

Cushing’s disease (most common

noniatrogenic cause)

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9
Q

ADRENAL GLAND
CUSHING’S SYNDROME
What is Cushing’s disease?
P419

A

Cushing’s syndrome caused by excess

production of ACTH by anterior pituitary

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10
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What is an ectopic ACTH
source?
P419
A

Tumor not found in the pituitary that
secretes ACTH, which in turn causes
adrenal gland to release cortisol without
the normal negative feedback loop

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11
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What are the signs/
symptoms of Cushing’s
syndrome?
P419
A

Truncal obesity, hirsutism, “moon”
facies, acne, “buffalo hump,” purple
striae, hypertension, diabetes, weakness,
depression, easy bruising, myopathy

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12
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
How can cortisol levels be
indirectly measured over a
short duration?
P419
A

By measuring urine cortisol or the
breakdown product of cortisol,
17 hydroxycorticosteroid (17-OHCS),
in the urine

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13
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What is a direct test of
serum cortisol?
P419
A

Serum cortisol level (highest in the morning

and lowest at night in healthy patients)

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14
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What initial tests should be
performed in Cushing’s
syndrome?
P420
A

Electrolytes
Serum cortisol
Urine-free cortisol, urine 17-OHCS
Low-dose dexamethasone suppression test

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15
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What is the low-dose
dexamethasone suppression
test?
P420
A 
Dexamethasone is a synthetic cortisol
that results in negative feedback on
ACTH secretion and subsequent cortisol
secretion in healthy patients; patients
with Cushing’s syndrome do not
suppress their cortisol secretion
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16
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
After the dexamethasone
test, what is next?
P420
A

Check ACTH levels

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17
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
Can plasma ACTH levels be
checked directly?
P420
A

Yes

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18
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What is the workup in a
patient suspected of having
Cushing’s syndrome?
P420 (picture)
A

(see picture)

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19
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
In ACTH-dependent
Cushing’s syndrome, how do
you differentiate between a
pituitary vs. an ectopic
ACTH source?
P421
A 
High-dose dexamethasone test:
    Pituitary source—cortisol is
       suppressed
Ectopic ACTH source—no cortisol
       suppression
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20
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
Summarize the “Cushing’s syndrome” lab values found in the majority of patients
with the following conditions:
Healthy patients
P421
A

Normal cortisol and ACTH,
suppression with low-dose or
high-dose dexamethasone (<1/2)

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21
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
Summarize the “Cushing’s syndrome” lab values found in the majority of patients
with the following conditions:
Cushing’s disease
(pituitary ACTH hypersecretion)
P421
A

High cortisol and ACTH, no suppression
with low-dose dexamethasone, suppression
with high-dose dexamethasone

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22
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
Summarize the “Cushing’s syndrome” lab values found in the majority of patients
with the following conditions:
Adrenal tumor
P421
A

High cortisol, low ACTH, no
suppression with low-dose or high-dose
dexamethasone

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23
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
Summarize the “Cushing’s syndrome” lab values found in the majority of patients
with the following conditions:
Ectopic ACTH-producing tumor
P421
A

High cortisol and ACTH, no suppression

with low-dose or high-dose dexamethasone

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24
Q

ADRENAL GLAND
CUSHING’S SYNDROME
What is the test for equivocal results for
differentiating pituitary vs. ectopic ACTH tumor?
P421

A

Bilateral petrosal vein sampling,

especially with CRH infusion

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25
``` ADRENAL GLAND CUSHING’S SYNDROME What is the most common site of ectopic ACTH-producing tumor? P421 ```
>66% are oat cell tumors of the lung | #2 is carcinoid
26
``` ADRENAL GLAND CUSHING’S SYNDROME How are the following tumors treated: Adrenal adenoma? P421 ```
Adrenalectomy (almost always unilateral)
27
``` ADRENAL GLAND CUSHING’S SYNDROME How are the following tumors treated: Adrenal carcinoma? P421 ```
``` Surgical excision (only 33% of cases are operable) ```
28
``` ADRENAL GLAND CUSHING’S SYNDROME How are the following tumors treated: Ectopic ACTH-producing tumor? P421 ```
Surgical excision, if feasible
29
``` ADRENAL GLAND CUSHING’S SYNDROME How are the following tumors treated: Cushing’s disease? P421 ```
Transphenoidal adenomectomy
30
ADRENAL GLAND CUSHING’S SYNDROME What medication must be given to a patient who is undergoing surgical correction of Cushing’s syndrome? P422
Cortisol (usually hydrocortisone until PO | is resumed)
31
``` ADRENAL GLAND CUSHING’S SYNDROME What medications inhibit cortisol production? P422 ```
1. Ketoconazole 2. Metyrapone 3. Aminoglutethimide 4. Mitotane
32
``` ADRENAL GLAND CUSHING’S SYNDROME Give the mechanism of action: Ketoconazole (an antifungal) P422 ```
Inhibits 11 ℬ--hydroxylase, c17-20 lyase, | and cholesterol side-chain cleavage
33
``` ADRENAL GLAND CUSHING’S SYNDROME Give the mechanism of action: Aminoglutethimide (an anticonvulsant) P422 ```
Inhibits cleavage of cholesterol side | chains
34
``` ADRENAL GLAND CUSHING’S SYNDROME Give the mechanism of action: Mitotane P422 ```
Inhibits 11 ℬ-hydroxylase and cholesterol side-chain cleavage; causes irreversible adrenocortical cells (and thus can be used for “medical adrenalectomy”)
35
``` ADRENAL GLAND CUSHING’S SYNDROME Give the mechanism of action: Metyrapone P422 ```
Inhibits 11 ℬ-hydroxylase
36
``` ADRENAL GLAND CUSHING’S SYNDROME What is a complication of BILATERAL adrenalectomy? P422 ```
Nelson’s syndrome—occurs in 10% of | patients after bilateral adrenalectomy
37
ADRENAL GLAND CUSHING’S SYNDROME What is Nelson’s syndrome? P422
``` Functional pituitary adenoma producing excessive ACTH and mass effect producing visual disturbances, hyperpigmentation, amenorrhea, with elevated ACTH levels Think: Nelson = Nuclear reaction in the pituitary ```
38
ADRENAL GLAND ADRENAL INCIDENTALOMA What is an incidentaloma? P422
Tumor found in the adrenal gland incidentally on a CT scan performed for an unrelated reason
39
``` ADRENAL GLAND ADRENAL INCIDENTALOMA What is the incidence of incidentalomas? P422 ```
4% of all CT scans (9% of autopsies)
40
``` ADRENAL GLAND ADRENAL INCIDENTALOMA What is the most common cause of incidentaloma? P423 ```
``` Nonfunctioning adenoma ( >75% of cases) ```
41
``` ADRENAL GLAND ADRENAL INCIDENTALOMA What is the differential diagnosis of incidentaloma? P423 ```
``` Nonfunctioning adenoma Pheochromocytoma Adrenocortical carcinoma Aldosteronoma Metastatic disease Nodular hyperplasia ```
42
``` ADRENAL GLAND ADRENAL INCIDENTALOMA What is the risk factor for carcinoma? P423 ```
Solid tumor >6 cm in diameter
43
ADRENAL GLAND ADRENAL INCIDENTALOMA What is the treatment? P423
``` Controversial for smaller/medium-sized tumors, but almost all surgeons would agree that resection is indicated for solid incidentalomas >6 cm in diameter because of risk of cancer ```
44
``` ADRENAL GLAND ADRENAL INCIDENTALOMA What are the indications for removal of adrenal incidentaloma less than 6 cm? P423 ```
``` MRI T2 signal >2 Hormonally active = hyperfunctioning tumor Enlarging cystic lesion Does not look like an adenoma ```
45
``` ADRENAL GLAND ADRENAL INCIDENTALOMA What tumor must be ruled out prior to biopsy or surgery for any adrenal mass? P423 ```
Pheochromocytoma (24-hour urine for | catecholamine, VMA, metanephrines)
46
ADRENAL GLAND PHEOCHROMOCYTOMA What is it? P423
Tumor of the adrenal MEDULLA and sympathetic ganglion (from chromaffin cell lines) that produces catecholamines (norepinephrine > epinephrine)
47
ADRENAL GLAND PHEOCHROMOCYTOMA What is the incidence? P423
Cause of hypertension in ≈1/500 hypertensive patients (≈10% of U.S. population has hypertension)
48
``` ADRENAL GLAND PHEOCHROMOCYTOMA Which age group is most likely to be affected? P423 ```
``` Any age (children and adults); average age is 40 to 60 years ```
49
``` ADRENAL GLAND PHEOCHROMOCYTOMA What are the associated risk factors? P423 ```
MEN-II, family history, von Recklinghausen | disease, von Hippel-Lindau disease
50
``` ADRENAL GLAND PHEOCHROMOCYTOMA What are the signs/ symptoms? P424 ```
``` “Classic” triad: 1. Palpitations 2. Headache 3. Episodic diaphoresis Also, hypertension (50%), pallor → flushing, anxiety, weight loss, tachycardia, hyperglycemia ```
51
``` ADRENAL GLAND PHEOCHROMOCYTOMA How can the pheochromocytoma SYMPTOMS triad be remembered? P424 ```
``` Think of the first three letters in the word PHEochromocytoma: Palpitations Headache Episodic diaphoresis ```
52
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the most common sign of pheochromocytoma? P424 ```
Hypertension
53
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the differential diagnosis? P424 ```
``` Renovascular hypertension, menopause, migraine headache, carcinoid syndrome, preeclampsia, neuroblastoma, anxiety disorder with panic attacks, hyperthyroidism, insulinoma ```
54
``` ADRENAL GLAND PHEOCHROMOCYTOMA What diagnostic tests should be performed? P424 ```
``` Urine screen: VanillylMandelic Acid (VMA), metanephrine, and normetanephrine (all breakdown products of the catechols) Urine/serum epinephrine/ norepinephrine levels ```
55
``` ADRENAL GLAND PHEOCHROMOCYTOMA What are the other common lab findings? P424 ```
``` Hyperglycemia (epinephrine increases glucose, norepinephrine decreases insulin) Polycythemia (resulting from intravascular volume depletion) ```
56
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the most common site of a pheochromocytoma? P424 ```
Adrenal >90%
57
``` ADRENAL GLAND PHEOCHROMOCYTOMA What are the other sites for pheochromocytoma? P424 ```
Organ of Zuckerkandl, thorax | (mediastinum), bladder, scrotum
58
``` ADRENAL GLAND PHEOCHROMOCYTOMA What are the tumor localization tests? P424 ```
CT scan, MRI, I-MIBG, PET scan, | OctreoScan (In-pentetreotide scan)
59
``` ADRENAL GLAND PHEOCHROMOCYTOMA What does I-MIBG stand for? P425 ```
IodineMetaIodoBenzylGuanidine
60
``` ADRENAL GLAND PHEOCHROMOCYTOMA How to remember MIBG and pheochromocytoma? P425 ```
Think: MIBG = My Big = and thus | “My Big Pheo” = MIBG Pheo
61
``` ADRENAL GLAND PHEOCHROMOCYTOMA How does the I-MIBG scan work? P425 ```
I-MIBG is a norepinephrine analog that collects in adrenergic vesicles and, thus, in pheochromocytomas
62
ADRENAL GLAND PHEOCHROMOCYTOMA What is the role of PET scan? P425
Positron Emission Tomography is helpful in localizing pheochromocytomas that do not accumulate MIBG
63
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the scan for imaging adrenal cortical pheochromocytoma? P425 ```
NP-59 (a cholesterol analog)
64
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the localizing option if a tumor is not seen on CT, MRI, or I-MIBG? P425 ```
IVC venous sampling for catecholamines | gradient will help localize the tumor
65
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the tumor site if epinephrine is elevated? P425 ```
Must be adrenal or near the adrenal gland (e.g., organ of Zuckerkandl), because nonadrenal tumors lack the capability to methylate norepinephrine to epinephrine
66
``` ADRENAL GLAND PHEOCHROMOCYTOMA What percentage of patients have malignant tumors? P425 ```
≈10%
67
``` ADRENAL GLAND PHEOCHROMOCYTOMA Can histology be used to determine malignancy? P425 ```
No; only distant metastasis or invasion | can determine malignancy
68
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the classic pheochromocytoma “rule of 10’s”? P425 ```
``` 10% malignant 10% bilateral 10% in children 10% multiple tumors 10% extra-adrenal ```
69
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the preoperative/ medical treatment? P425 ```
Increase intravascular volume with a -blockade (e.g., phenoxybenzamine or prazosin) to allow reduction in catecholamine-induced vasoconstriction and resulting volume depletion; treatment should start as soon as diagnosis is made +/-- ℬ-blockers
70
``` ADRENAL GLAND PHEOCHROMOCYTOMA How can you remember phenoxybenzamine as a medical treatment of pheochromocytoma? P426 ```
PHEochromocytoma = | PHEnoxybenzamine
71
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the surgical treatment? P426 ```
Tumor resection with early ligation of venous drainage (lower possibility of catecholamine release/crisis by tying off drainage) and minimal manipulation
72
``` ADRENAL GLAND PHEOCHROMOCYTOMA What are the possible perioperative complications? P426 ```
Anesthetic challenge: hypertensive crisis with manipulation (treat with nitroprusside), hypotension with total removal of the tumor, cardiac dysrhythmias
73
``` ADRENAL GLAND PHEOCHROMOCYTOMA In the patient with pheochromocytoma, what must be ruled out? P426 ```
MEN type II (almost all cases are | bilateral)
74
``` ADRENAL GLAND PHEOCHROMOCYTOMA What is the organ of Zuckerkandl? P426 ```
``` Body of embryonic chromaffin cells around the abdominal aorta (near the inferior mesenteric artery); normally atrophies during childhood, but is the most common site of extra-adrenal pheochromocytoma ```
75
ADRENAL GLAND CONN’S SYNDROME What is it? P426
Primary hyperaldosteronism due to | high aldosterone production
76
``` ADRENAL GLAND CONN’S SYNDROME How do you remember what Conn’s syndrome is? P426 (picture) ```
CONn’s disease = HYPERALdosterone |  = “CON HYPER AL”
77
``` ADRENAL GLAND CONN’S SYNDROME What are the common sources? P427 ```
Adrenal adenoma or adrenal hyperplasia; aldosterone is abnormally secreted by an adrenal adenoma (66%) > hyperplasia > carcinoma
78
``` ADRENAL GLAND CONN’S SYNDROME What is the normal physiology for aldosterone secretion? P427 ```
``` BP in the renal afferent arteriole is low Low sodium and hyperkalemia cause renin secretion from juxtaglomerular cells Renin then converts angiotensinogen to angiotensin I Angiotensin converting enzyme in the lung then converts angiotensin I to angiotensin II Angiotensin II then causes the adrenal glomerulosa cells to secrete aldosterone ```
79
``` ADRENAL GLAND CONN’S SYNDROME What is the normal physiologic effect of aldosterone? P427 ```
Aldosterone causes sodium retention for exchange of potassium in the kidney, resulting in fluid retention and increased BP
80
ADRENAL GLAND CONN’S SYNDROME What are the signs/symptoms? P427
Hypertension, headache, polyuria, | weakness
81
``` ADRENAL GLAND CONN’S SYNDROME What are the two classic clues of Conn’s syndrome? P427 ```
1. Hypertension | 2. Hypokalemia
82
``` ADRENAL GLAND CONN’S SYNDROME Classically, what kind of hypertension? P427 ```
Diastolic hypertension
83
``` ADRENAL GLAND CONN’S SYNDROME What are the renin levels with Conn’s syndrome? P427 ```
Normal or decreased!
84
``` ADRENAL GLAND CONN’S SYNDROME What percentage of all patients with hypertension have Conn’s syndrome? P427 ```
1%
85
``` ADRENAL GLAND CONN’S SYNDROME What diagnostic tests should be ordered? P427 ```
1. Plasma aldosterone concentration | 2. Plasma renin activity
86
``` ADRENAL GLAND CONN’S SYNDROME What ratio of these diagnostic tests is associated with primary hyperaldosteronism? P427 ```
Aldosterone to renin ratio of >30
87
``` ADRENAL GLAND CONN’S SYNDROME What is secondary hyperaldosteronism? P428 ```
Hyperaldosteronism resulting from abnormally high renin levels (renin increases angiotensin/aldosterone)
88
``` ADRENAL GLAND CONN’S SYNDROME What diagnostic tests should be performed? P428 ```
CT scan, adrenal venous sampling for | aldosterone levels, saline infusion
89
``` ADRENAL GLAND CONN’S SYNDROME What is the saline infusion test? P428 ```
Saline infusion will decrease aldosterone levels in normal patients but not in Conn’s syndrome
90
``` ADRENAL GLAND CONN’S SYNDROME What is the preoperative treatment? P428 ```
Spironolactone, K⁺ supplementation
91
ADRENAL GLAND CONN’S SYNDROME What is spironolactone? P428
``` Antialdosterone medication (works at the kidney tubule) ```
92
``` ADRENAL GLAND CONN’S SYNDROME What are the causes of Conn’s syndrome? P428 ```
Adrenal adenoma (66%) Bilateral idiopathic adrenal hyperplasia (30%) Adrenal cancer ( < 1%)
93
``` ADRENAL GLAND CONN’S SYNDROME What is the treatment of the following conditions: Adenoma? P428 ```
Unilateral adrenalectomy (laparoscopic)
94
``` ADRENAL GLAND CONN’S SYNDROME What is the treatment of the following conditions: Unilateral hyperplasia? P428 ```
Unilateral adrenalectomy (laparoscopic)
95
``` ADRENAL GLAND CONN’S SYNDROME What is the treatment of the following conditions: Bilateral hyperplasia? P428 ```
Spironolactone (usually no surgery)
96
``` ADRENAL GLAND CONN’S SYNDROME What are the renin levels in patients with PRIMARY hyperaldosteronism? P428 ```
Normal or low (key point!)
97
ADRENAL GLAND ADDISON’S DISEASE What is it? P428
Acute adrenal insufficiency
98
``` ADRENAL GLAND ADDISON’S DISEASE What are the electrolyte findings? P428 ```
HYPERkalemia, hyponatremia
99
``` ADRENAL GLAND ADDISON’S DISEASE How do you remember what ADDISON’s disease is? P428 ```
Think: ADDison’s disease = ADrenal | Down
100
ADRENAL GLAND INSULINOMA What is it? P428
Insulin-producing tumor arising from ℬ cells
101
ADRENAL GLAND INSULINOMA What is the incidence? P429
``` #1 Islet cell neoplasm; half of ℬ cell tumors of the pancreas produce insulin ```
102
``` ADRENAL GLAND INSULINOMA What are the associated risks? P429 ```
Associated with MEN-I syndrome (PPP = Pituitary, Pancreas, Parathyroid tumors)
103
``` ADRENAL GLAND INSULINOMA What are the signs/ symptoms? P429 ```
Sympathetic nervous system symptoms resulting from hypoglycemia: palpitations, diaphoresis, tremulousness, irritability, weakness
104
``` ADRENAL GLAND INSULINOMA What are the neurologic symptoms? P429 ```
Personality changes, confusion, | obtundation, seizures, coma
105
ADRENAL GLAND INSULINOMA What is Whipple’s triad? P429
``` 1. Hypoglycemic symptoms produced by fasting 2. Blood glucose 50 mg/dL during symptomatic attack 3. Relief of symptoms by administration of glucose ```
106
``` ADRENAL GLAND INSULINOMA What is the differential diagnosis? P429 ```
``` Reactive hypoglycemia Functional hypoglycemia with gastrectomy Adrenal insufficiency Hypopituitarism Hepatic insufficiency Munchausen syndrome (insulin self-injections) Nonislet cell tumor causing hypoglycemia Surreptitious administration of insulin or OHAs ```
107
``` ADRENAL GLAND INSULINOMA What lab tests should be performed? P429 ```
Glucose and insulin levels during fast; C-peptide and proinsulin levels (if selfinjection of insulin is a concern, as insulin injections have no proinsulin or C-peptides)
108
``` ADRENAL GLAND INSULINOMA What diagnostic tests should be performed? P429 ```
Fasting hypoglycemia with inappropriately high levels of insulin 72-hour fast, then check glucose and insulin levels every 6 hours (monitor very closely because patient can develop hypoglycemic crisis)
109
``` ADRENAL GLAND INSULINOMA What is the diagnostic fasting insulin to glucose ratio? P430 ```
>0.4
110
``` ADRENAL GLAND INSULINOMA What localizing tests should be performed? P430 ```
CT scan, A-gram, endoscopic U/S, venous catheterization (to sample blood along portal and splenic veins to measure insulin and localize tumor), intraoperative U/S
111
``` ADRENAL GLAND INSULINOMA What is the medical treatment? P430 ```
Diazoxide, to suppress insulin release
112
``` ADRENAL GLAND INSULINOMA What is the surgical treatment? P430 ```
Surgical resection
113
ADRENAL GLAND INSULINOMA What is the prognosis? P430
≈80% of patients have a benign solitary | adenoma that is cured by surgical resection
114
GLUCAGONOMA What is it? P430
Glucagon-producing tumor
115
GLUCAGONOMA Where is it located? P430
Pancreas (usually in the tail)
116
GLUCAGONOMA What are the symptoms? P430
Necrotizing migratory erythema (usually below the waist), glossitis, stomatitis, diabetes
117
GLUCAGONOMA What are the skin findings? P430
Necrotizing migratory erythema is a red, often psoriatic-appearing rash with serpiginous borders over the trunk and limbs
118
GLUCAGONOMA What are the associated lab findings? P430
Hyperglycemia, low amino acid levels, | high glucagon levels
119
GLUCAGONOMA What is the classic finding on CBC? P430
Anemia
120
GLUCAGONOMA What is the classic nutritional finding? P430
Low amino acid levels
121
GLUCAGONOMA What stimulation test is used for glucagonoma? P430
Tolbutamide stimulation test: IV tolbutamide results in elevated glucagon levels
122
GLUCAGONOMA What test is used for localization? P430
CT scan
123
``` GLUCAGONOMA What is the medical treatment of necrotizing migratory erythema? P431 ```
Somatostatin, IV amino acids
124
GLUCAGONOMA What is the treatment? P431
Surgical resection
125
SOMATOSTATINOMA What is it? P431
Pancreatic tumor that secretes somatostatin
126
SOMATOSTATINOMA What is the diagnostic triad? P431
``` DDD: 1. Diabetes 2. Diarrhea (steatorrhea) 3. Dilation of the gallbladder with gallstones ```
127
SOMATOSTATINOMA What is used to make the diagnosis? P431
CT scan and somatostatin level
128
SOMATOSTATINOMA What is the treatment? P431
Resection (do not enucleate)
129
``` SOMATOSTATINOMA What is the medical treatment if the tumor is unresectable? P431 ```
Streptozocin, dacarbazine, or doxorubicin
130
ZOLLINGER-ELLISON SYNDROME (ZES) What is it? P431
``` Gastrinoma: non- islet cell tumor of the pancreas (or other locale) that produces gastrin, causing gastric hypersecretion of HCl acid, resulting in GI ulcers ```
131
ZOLLINGER-ELLISON SYNDROME (ZES) What is the incidence? P431
1/1000 in patients with peptic ulcer disease, but nearly 2% in patients with recurrent ulcers
132
ZOLLINGER-ELLISON SYNDROME (ZES) What is the associated syndrome? P431
MEN-I syndrome
133
ZOLLINGER-ELLISON SYNDROME (ZES) What percentage of patients with ZES have MEN-I syndrome? P431
≈25% (75% of cases of Z-E syndrome | are “sporadic”)
134
ZOLLINGER-ELLISON SYNDROME (ZES) What percentage of patients with MEN-I will have ZES? P431
≈50%
135
``` ZOLLINGER-ELLISON SYNDROME (ZES) With gastrinoma, what lab tests should be ordered to screen for MEN-I? P432 ```
1. Calcium level | 2. Parathyroid hormone level
136
ZOLLINGER-ELLISON SYNDROME (ZES) What are the signs/ symptoms? P432
Peptic ulcers, diarrhea, weight loss, | abdominal pain
137
ZOLLINGER-ELLISON SYNDROME (ZES) What causes the diarrhea? P432
Massive acid hypersecretion and | destruction of digestive enzymes
138
ZOLLINGER-ELLISON SYNDROME (ZES) What are the signs? P432
PUD (epigastric pain, hematemesis, melena, hematochezia), GERD, diarrhea, recurrent ulcers, ulcers in unusual locations (e.g., proximal jejunum)
139
ZOLLINGER-ELLISON SYNDROME (ZES) What are the possible complications? P432
GI hemorrhage/perforation, gastric outlet | obstruction/stricture, metastatic disease
140
``` ZOLLINGER-ELLISON SYNDROME (ZES) What is the differential diagnosis of increased gastrin? P432 ```
``` Postvagotomy Gastric outlet obstruction G-cell hyperplasia Pernicious anemia Atrophic gastritis Short gut syndrome Renal failure H(2) ```
141
ZOLLINGER-ELLISON SYNDROME (ZES) Which patients should have a gastrin level checked? blocker, PPI P432
Those with recurrent ulcer; ulcer in unusual position (e.g., jejunum) or refractory to medical management; before any operation for ulcer
142
ZOLLINGER-ELLISON SYNDROME (ZES) What lab tests should be performed? P432
Fasting gastrin level Postsecretin challenge gastrin level Calcium (screen for MEN-I) Chem 7
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ZOLLINGER-ELLISON SYNDROME (ZES) What are the associated gastrin levels? P432
NL fasting = 100 pg/ml ZES fasting = 200–1000 pg/ml Basal acid secretion; (ZES >15 mEq/hr, nl <10mEq/hr)
144
ZOLLINGER-ELLISON SYNDROME (ZES) What is the secretin stimulation test? P432
IV secretin is administered and the gastrin level is determined; patients with ZES have a paradoxic increase in gastrin
145
ZOLLINGER-ELLISON SYNDROME (ZES) What are the classic secretin stimulation results? P433
Lab results with secretin challenge: NL—Decreased gastrin ZES—Increased gastrin (increased by  >200 pg/ml)
146
``` ZOLLINGER-ELLISON SYNDROME (ZES) How can you remember the diagnostic stimulation test for Z-E syndrome? P433 ```
Think: “Secret Z-E GAS”: SECRETin | = Z-E GAStrin
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ZOLLINGER-ELLISON SYNDROME (ZES) What tests are used to evaluate ulcers? P433
EGD, UGI, or both
148
ZOLLINGER-ELLISON SYNDROME (ZES) What tests are used to localize the tumor? P433
``` Octreotide scan (somatostatin receptor scan), abdominal CT, MRI, endoscopic ultrasonography (EUS) ```
149
ZOLLINGER-ELLISON SYNDROME (ZES) What is the most common site? P433
Pancreas
150
ZOLLINGER-ELLISON SYNDROME (ZES) What is the most common NONpancreatic site? P433
Duodenum
151
ZOLLINGER-ELLISON SYNDROME (ZES) What are some other sites? P433
Stomach, lymph nodes, liver, kidney, ovary
152
ZOLLINGER-ELLISON SYNDROME (ZES) Define “Passaro’s triangle.” P433 (picture)
``` A.k.a. “gastrinoma triangle,” a triangle drawn from the following points: 1. Cystic duct/CBD junction 2. Junction of the second and third portions of the duodenum 3. Neck of the pancreas ```
153
``` ZOLLINGER-ELLISON SYNDROME (ZES) What percentage of gastrinomas are in Passaro’s triangle? P434 ```
≈80%
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ZOLLINGER-ELLISON SYNDROME (ZES) What is the next step if the tumor cannot be localized? P434
``` Exploratory surgery (if tumor is not in pancreas, open duodenum and look), proximal gastric vagotomy if not found ```
155
ZOLLINGER-ELLISON SYNDROME (ZES) What is the medical treatment? P434
H(2) blockers, omeprazole, somatostatin
156
``` ZOLLINGER-ELLISON SYNDROME (ZES) What is the surgical treatment needed for each of the following: Tumor in head of pancreas? P434 ```
1. Enucleation of tumor 2. Whipple procedure if main pancreatic duct is involved
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``` ZOLLINGER-ELLISON SYNDROME (ZES) What is the surgical treatment needed for each of the following: Tumor in body or tail of pancreas? P434 ```
Distal pancreatectomy
158
``` ZOLLINGER-ELLISON SYNDROME (ZES) What is the surgical treatment needed for each of the following: Tumor in duodenum? P434 ```
Local resection
159
``` ZOLLINGER-ELLISON SYNDROME (ZES) What is the surgical treatment needed for each of the following: Unresectable tumor? P434 ```
High selective vagotomy
160
ZOLLINGER-ELLISON SYNDROME (ZES) What percentage have malignant tumors? P434
66%
161
ZOLLINGER-ELLISON SYNDROME (ZES) What is the most common site of metastasis? P434
Liver
162
``` ZOLLINGER-ELLISON SYNDROME (ZES) What is the treatment of patients with liver metastasis? P434 ```
Excision, if technically feasible
163
``` ZOLLINGER-ELLISON SYNDROME (ZES) What is the surgical option if gastrinoma is in duodenum/head of pancreas and is too large for local resection? P434 ```
Whipple procedure
164
``` ZOLLINGER-ELLISON SYNDROME (ZES) What is the prognosis with the following procedures: Complete excision? P435 ```
90% 10-year survival
165
``` ZOLLINGER-ELLISON SYNDROME (ZES) What is the prognosis with the following procedures: Incomplete excision? P435 ```
25% 10-year survival
166
MULTIPLE ENDOCRINE NEOPLASIA What is it also known as? P435
MEN syndrome
167
MULTIPLE ENDOCRINE NEOPLASIA What is it? P435
Inherited condition of propensity to | develop multiple endocrine tumors
168
MULTIPLE ENDOCRINE NEOPLASIA How is it inherited? P435
``` Autosomal dominant (but with a significant degree of variation in penetrance) ```
169
MULTIPLE ENDOCRINE NEOPLASIA Which patients should be screened for MEN? P435
All family members of patients diagnosed | with MEN
170
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE I What is the common eponym? P435 ```
Wermer’s syndrome | Think: Wermer = Winner = #1 = type 1
171
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE I What is the gene defect in MEN type I? P435 ```
Chromosome 11 (Think: 11 = 1)
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``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE I What are the most common tumors and their incidences? P435 ```
``` “PPP”: Parathyroid hyperplasia ( ≈90%) Pancreatic islet cell tumors (≈66%) Gastrinoma: ZES (50%) Insulinoma (20%) Pituitary tumors (≈50%) ```
173
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE I How can tumors for MEN-I be remembered? P435 ```
Think: type 1 = Primary, Primary, Primary = PPP = Parathyroid, Pancreas, Pituitary
174
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE I How can the P’s associated with MEN-I be remembered? P435 ```
All the P’s are followed by a vowel: PA, | PA, PI
175
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE I What percentage of patients with MEN-I have parathyroid hyperplasia? P435 ```
≈90%
176
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE I What percentage of patients with MEN-I have a gastrinoma? P436 ```
≈50%
177
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE I What other tumors (in addition to PPP) are associated with MEN-I? P436 ```
Adrenal (30%) and thyroid (15%) | adenomas
178
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIa What is the common eponym? P436 ```
Sipple’s syndrome (Think: Sipple = | Second = #2 = type 2)
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``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIa What is the gene defect in MEN type IIa? P436 ```
RET (Think: reT = Two)
180
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIa What are the most common tumors and their incidences? P436 ```
``` “MPH”: Medullary thyroid carcinoma (100%); Calcitonin secreted Pheochromocytoma (>33%); Catecholamine excess Hyperparathyroidism (≈50%); Hypercalcemia ```
181
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIa How can the tumors involved with MEN-IIa be remembered? P436 ```
Think: type 2 = 2 MPH or 2 Miles Per Hour = MPH = Medullary, Pheochromocytoma, Hyperparathyroid
182
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIa How can the P of MPH be remembered? P436 ```
Followed by the consonant “H”— PHEOCHROMOCYTOMA (remember, the P’s of MEN-I are followed by vowels)
183
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIa What percentage of patients with MEN-IIa have medullary carcinoma of the thyroid? P436 ```
100%
184
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb What are the most common abnormalities, their incidences, and symptoms? P436 ```
``` “MMMP”: Mucosal neuromas (100%)—in the nasopharynx, oropharynx, larynx, and conjunctiva Medullary thyroid carcinoma (85%)— more aggressive than in MEN-IIa Marfanoid body habitus (long/lanky) Pheochromocytoma (50%) and found bilaterally ```
185
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb How can the features of MEN-IIb be remembered? P437 ```
MMMP (Think: 3M Plastics)
186
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb How can you remember that MEN-IIb is marfanoid habitus? P437 ```
Think: “TO BE marfanoid” = II B | marfanoid
187
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb What is the anatomic distribution of medullary thyroid carcinoma in MEN-II? P437 ```
Almost always bilateral (non–MEN-II | cases are almost always unilateral!)
188
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb What are the physical findings/signs of MEN-IIb? P437 ```
``` Mucosal neuromas (e.g., mouth, eyes) Marfanoid body habitus Pes cavus/planum (large arch of foot/ flatfooted) Constipation ```
189
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb What is the most common GI complaint of patients with MEN-IIb? P437 ```
Constipation resulting from | ganglioneuromatosis of GI tract
190
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb What percentage of pheochromocytomas in MEN-IIa/b are bilateral? P437 ```
≈70% (but found bilaterally in only 10% of all patients diagnosed with pheochromocytoma)
191
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb What is the major difference between MEN-IIa and MEN-IIb? P437 ```
``` MEN-IIa = parathyroid hyperplasia MEN-IIb = no parathyroid hyperplasia (and neuromas, marfanoid habitus, pes cavus [extensive arch of foot], etc.) ```
192
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb What type of parathyroid disease is associated with MEN-I and MEN-IIa? P437 ```
Hyperplasia (treat with removal of all parathyroid tissue with autotransplant of some of the parathyroid tissue to the forearm)
193
``` MULTIPLE ENDOCRINE NEOPLASIA MEN TYPE IIb What percentage of patients with Z-E syndrome have MEN-I? P437 ```
≈25%

Section II General Surgery P203Surgical Recall Sixth (33 decks)

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