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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
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What is the most common site of ectopic
ACTH-producing tumor?
P421
A

>66% are oat cell tumors of the lung

#2 is carcinoid

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26
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
How are the following tumors treated:
Adrenal adenoma?
P421
A

Adrenalectomy (almost always unilateral)

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27
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
How are the following tumors treated:
Adrenal carcinoma?
P421
A 
Surgical excision (only 33% of cases are
operable)
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28
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
How are the following tumors treated:
Ectopic ACTH-producing tumor?
P421
A

Surgical excision, if feasible

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29
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
How are the following tumors treated:
Cushing’s disease?
P421
A

Transphenoidal adenomectomy

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30
Q

ADRENAL GLAND
CUSHING’S SYNDROME
What medication must be given to a patient who is undergoing surgical correction of Cushing’s syndrome?
P422

A

Cortisol (usually hydrocortisone until PO

is resumed)

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31
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What medications inhibit
cortisol production?
P422
A
  1. Ketoconazole
  2. Metyrapone
  3. Aminoglutethimide
  4. Mitotane
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32
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
Give the mechanism of action:
Ketoconazole (an antifungal)
P422
A

Inhibits 11 ℬ–hydroxylase, c17-20 lyase,

and cholesterol side-chain cleavage

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33
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
Give the mechanism of action:
Aminoglutethimide
(an anticonvulsant)
P422
A

Inhibits cleavage of cholesterol side

chains

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34
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
Give the mechanism of action:
Mitotane
P422
A

Inhibits 11 ℬ-hydroxylase and cholesterol
side-chain cleavage; causes irreversible
adrenocortical cells (and thus can be
used for “medical adrenalectomy”)

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35
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
Give the mechanism of action:
Metyrapone
P422
A

Inhibits 11 ℬ-hydroxylase

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36
Q 
ADRENAL GLAND
CUSHING’S SYNDROME
What is a complication of BILATERAL
adrenalectomy?
P422
A

Nelson’s syndrome—occurs in 10% of

patients after bilateral adrenalectomy

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37
Q

ADRENAL GLAND
CUSHING’S SYNDROME
What is Nelson’s syndrome?
P422

A 
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
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38
Q

ADRENAL GLAND
ADRENAL INCIDENTALOMA
What is an incidentaloma?
P422

A

Tumor found in the adrenal gland
incidentally on a CT scan performed for
an unrelated reason

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39
Q 
ADRENAL GLAND
ADRENAL INCIDENTALOMA
What is the incidence of
incidentalomas?
P422
A

4% of all CT scans (9% of autopsies)

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40
Q 
ADRENAL GLAND
ADRENAL INCIDENTALOMA
What is the most common
cause of incidentaloma?
P423
A 
Nonfunctioning adenoma ( >75% of
cases)
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41
Q 
ADRENAL GLAND
ADRENAL INCIDENTALOMA
What is the differential
diagnosis of incidentaloma?
P423
A 
Nonfunctioning adenoma
Pheochromocytoma
Adrenocortical carcinoma
Aldosteronoma
Metastatic disease
Nodular hyperplasia
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42
Q 
ADRENAL GLAND
ADRENAL INCIDENTALOMA
What is the risk factor for
carcinoma?
P423
A

Solid tumor >6 cm in diameter

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43
Q

ADRENAL GLAND
ADRENAL INCIDENTALOMA
What is the treatment?
P423

A 
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
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44
Q 
ADRENAL GLAND
ADRENAL INCIDENTALOMA
What are the indications
for removal of adrenal
incidentaloma less than 6 cm?
P423
A 
MRI T2 signal >2
Hormonally active = hyperfunctioning
    tumor
Enlarging cystic lesion
Does not look like an adenoma
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45
Q 
ADRENAL GLAND
ADRENAL INCIDENTALOMA
What tumor must be ruled
out prior to biopsy or surgery
for any adrenal mass?
P423
A

Pheochromocytoma (24-hour urine for

catecholamine, VMA, metanephrines)

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46
Q

ADRENAL GLAND
PHEOCHROMOCYTOMA
What is it?
P423

A

Tumor of the adrenal MEDULLA and
sympathetic ganglion (from chromaffin
cell lines) that produces catecholamines
(norepinephrine > epinephrine)

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47
Q

ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the incidence?
P423

A

Cause of hypertension in ≈1/500
hypertensive patients (≈10% of U.S.
population has hypertension)

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48
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
Which age group is most
likely to be affected?
P423
A 
Any age (children and adults); average
age is 40 to 60 years
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49
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What are the associated risk
factors?
P423
A

MEN-II, family history, von Recklinghausen

disease, von Hippel-Lindau disease

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50
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What are the signs/
symptoms?
P424
A 
“Classic” triad:
    1. Palpitations
    2. Headache
    3. Episodic diaphoresis
Also, hypertension (50%), pallor →
flushing, anxiety, weight loss, tachycardia,
hyperglycemia
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51
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
How can the
pheochromocytoma
SYMPTOMS triad be
remembered?
P424
A 
Think of the first three letters in the
word PHEochromocytoma:
    Palpitations
    Headache
    Episodic diaphoresis
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52
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the most common
sign of pheochromocytoma?
P424
A

Hypertension

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53
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the differential
diagnosis?
P424
A 
Renovascular hypertension, menopause,
migraine headache, carcinoid syndrome,
preeclampsia, neuroblastoma, anxiety
disorder with panic attacks,
hyperthyroidism, insulinoma
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54
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What diagnostic tests should
be performed?
P424
A 
Urine screen: VanillylMandelic Acid
    (VMA), metanephrine, and
    normetanephrine (all breakdown
    products of the catechols)
Urine/serum epinephrine/
    norepinephrine levels
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55
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What are the other common
lab findings?
P424
A 
Hyperglycemia (epinephrine increases
    glucose, norepinephrine decreases
    insulin)
Polycythemia (resulting from intravascular
    volume depletion)
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56
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the most
common site of a
pheochromocytoma?
P424
A

Adrenal >90%

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57
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What are the other sites for
pheochromocytoma?
P424
A

Organ of Zuckerkandl, thorax

(mediastinum), bladder, scrotum

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58
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What are the tumor
localization tests?
P424
A

CT scan, MRI, I-MIBG, PET scan,

OctreoScan (In-pentetreotide scan)

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59
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What does I-MIBG
stand for?
P425
A

IodineMetaIodoBenzylGuanidine

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60
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
How to remember MIBG
and pheochromocytoma?
P425
A

Think: MIBG = My Big = and thus

“My Big Pheo” = MIBG Pheo

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61
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
How does the I-MIBG
scan work?
P425
A

I-MIBG is a norepinephrine analog
that collects in adrenergic vesicles and,
thus, in pheochromocytomas

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62
Q

ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the role of PET scan?
P425

A

Positron Emission Tomography is helpful
in localizing pheochromocytomas that do
not accumulate MIBG

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63
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the scan for
imaging adrenal cortical
pheochromocytoma?
P425
A

NP-59 (a cholesterol analog)

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64
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the localizing option
if a tumor is not seen on CT,
MRI, or I-MIBG?
P425
A

IVC venous sampling for catecholamines

gradient will help localize the tumor

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65
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the tumor site if
epinephrine is elevated?
P425
A

Must be adrenal or near the adrenal gland
(e.g., organ of Zuckerkandl), because
nonadrenal tumors lack the capability to
methylate norepinephrine to epinephrine

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66
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What percentage of patients
have malignant tumors?
P425
A

≈10%

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67
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
Can histology be used to
determine malignancy?
P425
A

No; only distant metastasis or invasion

can determine malignancy

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68
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the classic
pheochromocytoma “rule
of 10’s”?
P425
A 
10% malignant
10% bilateral
10% in children
10% multiple tumors
10% extra-adrenal
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69
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the preoperative/
medical treatment?
P425
A

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

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70
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
How can you remember
phenoxybenzamine as a
medical treatment of
pheochromocytoma?
P426
A

PHEochromocytoma =

PHEnoxybenzamine

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71
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the surgical
treatment?
P426
A

Tumor resection with early ligation of
venous drainage (lower possibility of
catecholamine release/crisis by tying off
drainage) and minimal manipulation

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72
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What are the possible
perioperative complications?
P426
A

Anesthetic challenge: hypertensive crisis
with manipulation (treat with nitroprusside),
hypotension with total removal of
the tumor, cardiac dysrhythmias

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73
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
In the patient with
pheochromocytoma, what
must be ruled out?
P426
A

MEN type II (almost all cases are

bilateral)

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74
Q 
ADRENAL GLAND
PHEOCHROMOCYTOMA
What is the organ of
Zuckerkandl?
P426
A 
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
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75
Q

ADRENAL GLAND
CONN’S SYNDROME
What is it?
P426

A

Primary hyperaldosteronism due to

high aldosterone production

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76
Q 
ADRENAL GLAND
CONN’S SYNDROME
How do you remember what
Conn’s syndrome is?
P426 (picture)
A

CONn’s disease = HYPERALdosterone

= “CON HYPER AL”

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77
Q 
ADRENAL GLAND
CONN’S SYNDROME
What are the common
sources?
P427
A

Adrenal adenoma or adrenal
hyperplasia; aldosterone is abnormally
secreted by an adrenal adenoma (66%)
> hyperplasia > carcinoma

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78
Q 
ADRENAL GLAND
CONN’S SYNDROME
What is the normal physiology for aldosterone
secretion?
P427
A 
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
Q 
ADRENAL GLAND
CONN’S SYNDROME
What is the normal
physiologic effect of
aldosterone?
P427
A

Aldosterone causes sodium retention for
exchange of potassium in the kidney, resulting
in fluid retention and increased BP

80
Q

ADRENAL GLAND
CONN’S SYNDROME
What are the signs/symptoms?
P427

A

Hypertension, headache, polyuria,

weakness

81
Q 
ADRENAL GLAND
CONN’S SYNDROME
What are the two classic
clues of Conn’s syndrome?
P427
A
  1. Hypertension

2. Hypokalemia

82
Q 
ADRENAL GLAND
CONN’S SYNDROME
Classically, what kind of
hypertension?
P427
A

Diastolic hypertension

83
Q 
ADRENAL GLAND
CONN’S SYNDROME
What are the renin levels
with Conn’s syndrome?
P427
A

Normal or decreased!

84
Q 
ADRENAL GLAND
CONN’S SYNDROME
What percentage of all
patients with hypertension
have Conn’s syndrome?
P427
A

1%

85
Q 
ADRENAL GLAND
CONN’S SYNDROME
What diagnostic tests should
be ordered?
P427
A
  1. Plasma aldosterone concentration

2. Plasma renin activity

86
Q 
ADRENAL GLAND
CONN’S SYNDROME
What ratio of these
diagnostic tests is
associated with primary
hyperaldosteronism?
P427
A

Aldosterone to renin ratio of >30

87
Q 
ADRENAL GLAND
CONN’S SYNDROME
What is secondary
hyperaldosteronism?
P428
A

Hyperaldosteronism resulting from
abnormally high renin levels (renin
increases angiotensin/aldosterone)

88
Q 
ADRENAL GLAND
CONN’S SYNDROME
What diagnostic tests should
be performed?
P428
A

CT scan, adrenal venous sampling for

aldosterone levels, saline infusion

89
Q 
ADRENAL GLAND
CONN’S SYNDROME
What is the saline infusion
test?
P428
A

Saline infusion will decrease aldosterone
levels in normal patients but not in
Conn’s syndrome

90
Q 
ADRENAL GLAND
CONN’S SYNDROME
What is the preoperative
treatment?
P428
A

Spironolactone, K⁺ supplementation

91
Q

ADRENAL GLAND
CONN’S SYNDROME
What is spironolactone?
P428

A 
Antialdosterone medication (works at the
kidney tubule)
92
Q 
ADRENAL GLAND
CONN’S SYNDROME
What are the causes of
Conn’s syndrome?
P428
A

Adrenal adenoma (66%)
Bilateral idiopathic adrenal hyperplasia
(30%)
Adrenal cancer ( < 1%)

93
Q 
ADRENAL GLAND
CONN’S SYNDROME
What is the treatment of the following conditions:
Adenoma?
P428
A

Unilateral adrenalectomy (laparoscopic)

94
Q 
ADRENAL GLAND
CONN’S SYNDROME
What is the treatment of the following conditions:
Unilateral hyperplasia?
P428
A

Unilateral adrenalectomy (laparoscopic)

95
Q 
ADRENAL GLAND
CONN’S SYNDROME
What is the treatment of the following conditions:
Bilateral hyperplasia?
P428
A

Spironolactone (usually no surgery)

96
Q 
ADRENAL GLAND
CONN’S SYNDROME
What are the renin levels in
patients with PRIMARY
hyperaldosteronism?
P428
A

Normal or low (key point!)

97
Q

ADRENAL GLAND
ADDISON’S DISEASE
What is it?
P428

A

Acute adrenal insufficiency

98
Q 
ADRENAL GLAND
ADDISON’S DISEASE
What are the electrolyte
findings?
P428
A

HYPERkalemia, hyponatremia

99
Q 
ADRENAL GLAND
ADDISON’S DISEASE
How do you remember what
ADDISON’s disease is?
P428
A

Think: ADDison’s disease = ADrenal

Down

100
Q

ADRENAL GLAND
INSULINOMA
What is it?
P428

A

Insulin-producing tumor arising from ℬ cells

101
Q

ADRENAL GLAND
INSULINOMA
What is the incidence?
P429

A 
#1 Islet cell neoplasm; half of ℬ cell
tumors of the pancreas produce insulin
102
Q 
ADRENAL GLAND
INSULINOMA
What are the associated
risks?
P429
A

Associated with MEN-I syndrome
(PPP = Pituitary, Pancreas, Parathyroid
tumors)

103
Q 
ADRENAL GLAND
INSULINOMA
What are the signs/
symptoms?
P429
A

Sympathetic nervous system symptoms
resulting from hypoglycemia:
palpitations, diaphoresis, tremulousness,
irritability, weakness

104
Q 
ADRENAL GLAND
INSULINOMA
What are the neurologic
symptoms?
P429
A

Personality changes, confusion,

obtundation, seizures, coma

105
Q

ADRENAL GLAND
INSULINOMA
What is Whipple’s triad?
P429

A 
1. Hypoglycemic symptoms produced by
    fasting
2. Blood glucose 50 mg/dL during
    symptomatic attack
3. Relief of symptoms by administration
    of glucose
106
Q 
ADRENAL GLAND
INSULINOMA
What is the differential
diagnosis?
P429
A 
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
Q 
ADRENAL GLAND
INSULINOMA
What lab tests should be
performed?
P429
A

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
Q 
ADRENAL GLAND
INSULINOMA
What diagnostic tests should
be performed?
P429
A

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
Q 
ADRENAL GLAND
INSULINOMA
What is the diagnostic fasting
insulin to glucose ratio?
P430
A

> 0.4

110
Q 
ADRENAL GLAND
INSULINOMA
What localizing tests should
be performed?
P430
A

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
Q 
ADRENAL GLAND
INSULINOMA
What is the medical
treatment?
P430
A

Diazoxide, to suppress insulin release

112
Q 
ADRENAL GLAND
INSULINOMA
What is the surgical
treatment?
P430
A

Surgical resection

113
Q

ADRENAL GLAND
INSULINOMA
What is the prognosis?
P430

A

≈80% of patients have a benign solitary

adenoma that is cured by surgical resection

114
Q

GLUCAGONOMA
What is it?
P430

A

Glucagon-producing tumor

115
Q

GLUCAGONOMA
Where is it located?
P430

A

Pancreas (usually in the tail)

116
Q

GLUCAGONOMA
What are the symptoms?
P430

A

Necrotizing migratory erythema
(usually below the waist), glossitis,
stomatitis, diabetes

117
Q

GLUCAGONOMA
What are the skin findings?
P430

A

Necrotizing migratory erythema is a red,
often psoriatic-appearing rash with serpiginous
borders over the trunk and limbs

118
Q

GLUCAGONOMA
What are the associated lab
findings?
P430

A

Hyperglycemia, low amino acid levels,

high glucagon levels

119
Q

GLUCAGONOMA
What is the classic finding
on CBC?
P430

A

Anemia

120
Q

GLUCAGONOMA
What is the classic nutritional
finding?
P430

A

Low amino acid levels

121
Q

GLUCAGONOMA
What stimulation test is used
for glucagonoma?
P430

A

Tolbutamide stimulation test:
IV tolbutamide results in elevated
glucagon levels

122
Q

GLUCAGONOMA
What test is used for
localization?
P430

A

CT scan

123
Q 
GLUCAGONOMA
What is the medical
treatment of necrotizing
migratory erythema?
P431
A

Somatostatin, IV amino acids

124
Q

GLUCAGONOMA
What is the treatment?
P431

A

Surgical resection

125
Q

SOMATOSTATINOMA
What is it?
P431

A

Pancreatic tumor that secretes somatostatin

126
Q

SOMATOSTATINOMA
What is the diagnostic triad?
P431

A 
DDD:
1. Diabetes
2. Diarrhea (steatorrhea)
3. Dilation of the gallbladder with
gallstones
127
Q

SOMATOSTATINOMA
What is used to make the
diagnosis?
P431

A

CT scan and somatostatin level

128
Q

SOMATOSTATINOMA
What is the treatment?
P431

A

Resection (do not enucleate)

129
Q 
SOMATOSTATINOMA
What is the medical
treatment if the tumor is
unresectable?
P431
A

Streptozocin, dacarbazine, or doxorubicin

130
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What is it?
P431

A 
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
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What is the incidence?
P431

A

1/1000 in patients with peptic ulcer
disease, but nearly 2% in patients with
recurrent ulcers

132
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What is the associated
syndrome?
P431

A

MEN-I syndrome

133
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What percentage of patients
with ZES have MEN-I syndrome?
P431

A

≈25% (75% of cases of Z-E syndrome

are “sporadic”)

134
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What percentage of patients
with MEN-I will have ZES?
P431

A

≈50%

135
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
With gastrinoma, what lab
tests should be ordered to
screen for MEN-I?
P432
A
  1. Calcium level

2. Parathyroid hormone level

136
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What are the signs/
symptoms?
P432

A

Peptic ulcers, diarrhea, weight loss,

abdominal pain

137
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What causes the diarrhea?
P432

A

Massive acid hypersecretion and

destruction of digestive enzymes

138
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What are the signs?
P432

A

PUD (epigastric pain, hematemesis,
melena, hematochezia), GERD, diarrhea,
recurrent ulcers, ulcers in unusual
locations (e.g., proximal jejunum)

139
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What are the possible
complications?
P432

A

GI hemorrhage/perforation, gastric outlet

obstruction/stricture, metastatic disease

140
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
What is the differential
diagnosis of increased
gastrin?
P432
A 
Postvagotomy
Gastric outlet obstruction
G-cell hyperplasia
Pernicious anemia
Atrophic gastritis
Short gut syndrome
Renal failure
H(2)
141
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
Which patients should have
a gastrin level checked? blocker, PPI
P432

A

Those with recurrent ulcer; ulcer in
unusual position (e.g., jejunum) or
refractory to medical management;
before any operation for ulcer

142
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What lab tests should be
performed?
P432

A

Fasting gastrin level
Postsecretin challenge gastrin level
Calcium (screen for MEN-I)
Chem 7

143
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What are the associated
gastrin levels?
P432

A

NL fasting = 100 pg/ml
ZES fasting = 200–1000 pg/ml
Basal acid secretion; (ZES >15 mEq/hr,
nl <10mEq/hr)

144
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What is the secretin
stimulation test?
P432

A

IV secretin is administered and the
gastrin level is determined; patients with
ZES have a paradoxic increase in gastrin

145
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What are the classic secretin
stimulation results?
P433

A

Lab results with secretin challenge:
NL—Decreased gastrin
ZES—Increased gastrin (increased by
>200 pg/ml)

146
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
How can you remember the
diagnostic stimulation test
for Z-E syndrome?
P433
A

Think: “Secret Z-E GAS”: SECRETin

= Z-E GAStrin

147
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What tests are used to
evaluate ulcers?
P433

A

EGD, UGI, or both

148
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What tests are used to
localize the tumor?
P433

A 
Octreotide scan (somatostatin receptor
scan), abdominal CT, MRI, endoscopic
ultrasonography (EUS)
149
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What is the most common
site?
P433

A

Pancreas

150
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What is the most common
NONpancreatic site?
P433

A

Duodenum

151
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What are some other sites?
P433

A

Stomach, lymph nodes, liver, kidney, ovary

152
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
Define “Passaro’s triangle.”
P433 (picture)

A 
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
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
What percentage of
gastrinomas are in Passaro’s
triangle?
P434
A

≈80%

154
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What is the next step if the
tumor cannot be localized?
P434

A 
Exploratory surgery (if tumor is not
in pancreas, open duodenum and
look), proximal gastric vagotomy if
not found
155
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What is the medical
treatment?
P434

A

H(2) blockers, omeprazole, somatostatin

156
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
What is the surgical treatment needed for
each of the following:
Tumor in head of pancreas?
P434
A
  1. Enucleation of tumor
  2. Whipple procedure if main pancreatic
    duct is involved
157
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
What is the surgical treatment needed for
each of the following:
Tumor in body or tail of
pancreas?
P434
A

Distal pancreatectomy

158
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
What is the surgical treatment needed for
each of the following:
Tumor in duodenum?
P434
A

Local resection

159
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
What is the surgical treatment needed for
each of the following:
Unresectable tumor?
P434
A

High selective vagotomy

160
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What percentage have
malignant tumors?
P434

A

66%

161
Q

ZOLLINGER-ELLISON SYNDROME (ZES)
What is the most common
site of metastasis?
P434

A

Liver

162
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
What is the treatment
of patients with liver
metastasis?
P434
A

Excision, if technically feasible

163
Q 
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
A

Whipple procedure

164
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
What is the prognosis with
the following procedures:
Complete excision?
P435
A

90% 10-year survival

165
Q 
ZOLLINGER-ELLISON SYNDROME (ZES)
What is the prognosis with
the following procedures:
Incomplete excision?
P435
A

25% 10-year survival

166
Q

MULTIPLE ENDOCRINE NEOPLASIA
What is it also known as?
P435

A

MEN syndrome

167
Q

MULTIPLE ENDOCRINE NEOPLASIA
What is it?
P435

A

Inherited condition of propensity to

develop multiple endocrine tumors

168
Q

MULTIPLE ENDOCRINE NEOPLASIA
How is it inherited?
P435

A 
Autosomal dominant (but with a significant
degree of variation in penetrance)
169
Q

MULTIPLE ENDOCRINE NEOPLASIA
Which patients should be
screened for MEN?
P435

A

All family members of patients diagnosed

with MEN

170
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE I
What is the common
eponym?
P435
A

Wermer’s syndrome

Think: Wermer = Winner = #1 = type 1

171
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE I
What is the gene defect in
MEN type I?
P435
A

Chromosome 11 (Think: 11 = 1)

172
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE I
What are the most common
tumors and their incidences?
P435
A 
“PPP”:
    Parathyroid hyperplasia ( ≈90%)
    Pancreatic islet cell tumors (≈66%)
       Gastrinoma: ZES (50%)
       Insulinoma (20%)
Pituitary tumors (≈50%)
173
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE I
How can tumors for MEN-I
be remembered?
P435
A

Think: type 1 = Primary, Primary,
Primary = PPP = Parathyroid, Pancreas,
Pituitary

174
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE I
How can the P’s associated
with MEN-I be remembered?
P435
A

All the P’s are followed by a vowel: PA,

PA, PI

175
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE I
What percentage of patients with MEN-I have
parathyroid hyperplasia?
P435
A

≈90%

176
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE I
What percentage of patients
with MEN-I have a gastrinoma?
P436
A

≈50%

177
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE I
What other tumors
(in addition to PPP) are
associated with MEN-I?
P436
A

Adrenal (30%) and thyroid (15%)

adenomas

178
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIa
What is the common
eponym?
P436
A

Sipple’s syndrome (Think: Sipple =

Second = #2 = type 2)

179
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIa
What is the gene defect in
MEN type IIa?
P436
A

RET (Think: reT = Two)

180
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIa
What are the most common
tumors and their incidences?
P436
A 
“MPH”:
    Medullary thyroid carcinoma (100%);
       Calcitonin secreted
    Pheochromocytoma (>33%);
       Catecholamine excess
    Hyperparathyroidism (≈50%);
       Hypercalcemia
181
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIa
How can the tumors
involved with MEN-IIa be
remembered?
P436
A

Think: type 2 = 2 MPH or 2 Miles
Per Hour = MPH = Medullary,
Pheochromocytoma, Hyperparathyroid

182
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIa
How can the P of MPH be
remembered?
P436
A

Followed by the consonant “H”—
PHEOCHROMOCYTOMA (remember,
the P’s of MEN-I are followed by vowels)

183
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIa
What percentage of
patients with MEN-IIa have
medullary carcinoma of the
thyroid?
P436
A

100%

184
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
What are the most common
abnormalities, their
incidences, and symptoms?
P436
A 
“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
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
How can the features of
MEN-IIb be remembered?
P437
A

MMMP (Think: 3M Plastics)

186
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
How can you remember
that MEN-IIb is marfanoid
habitus?
P437
A

Think: “TO BE marfanoid” = II B

marfanoid

187
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
What is the anatomic
distribution of medullary
thyroid carcinoma in
MEN-II?
P437
A

Almost always bilateral (non–MEN-II

cases are almost always unilateral!)

188
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
What are the physical
findings/signs of MEN-IIb?
P437
A 
Mucosal neuromas (e.g., mouth, eyes)
Marfanoid body habitus
Pes cavus/planum (large arch of foot/
    flatfooted)
Constipation
189
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
What is the most common
GI complaint of patients
with MEN-IIb?
P437
A

Constipation resulting from

ganglioneuromatosis of GI tract

190
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
What percentage of
pheochromocytomas in
MEN-IIa/b are bilateral?
P437
A

≈70% (but found bilaterally in only 10%
of all patients diagnosed with
pheochromocytoma)

191
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
What is the major difference
between MEN-IIa and
MEN-IIb?
P437
A 
MEN-IIa = parathyroid hyperplasia
MEN-IIb = no parathyroid hyperplasia
    (and neuromas, marfanoid habitus,
    pes cavus [extensive arch of foot],
    etc.)
192
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
What type of parathyroid
disease is associated with
MEN-I and MEN-IIa?
P437
A

Hyperplasia (treat with removal of all
parathyroid tissue with autotransplant of
some of the parathyroid tissue to the
forearm)

193
Q 
MULTIPLE ENDOCRINE NEOPLASIA
MEN TYPE IIb
What percentage of patients
with Z-E syndrome have
MEN-I?
P437
A

≈25%

Section II General Surgery P203Surgical Recall Sixth (33 decks)

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