Endocrine Flashcards
1
Q
Where is the drainage of the left adrenal vein?
A
Left renal vein
2
Q
Where is the drainage of the right adrenal vein?
A
IVC
3
Q
What is CRH?
A
Corticotropin-Releasing Hormone.
Released from anterior hypothalamus and causes released of ACTH from anterior pituitary
4
Q
What is ACTH?
A
AndrenoCorticoTropic Hormone.
Released from anterior pituitary, which in turn causes adrenal glands to release cortisol
5
Q
What feeds back to inhibit ACTH secretion?
A
Cortisol
6
Q
What is Cushing’s syndrome?
A
Excessive cortisol production.
7
Q
What is the most common cause of Cushing’s syndrome?
A
Iatrogenic (e.g. prednisone)
8
Q
What is the second most common cause of Cushing’s syndrome?
A
Cushing’s disease
9
Q
What is Cushing’s disease?
A
Cushing’s syndrome caused by excess production of ACTH by anterior pituitary
10
Q
What is an ectopic ACTH source?
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
11
Q
What are the signs and symptoms of Cushing’s syndrome?
A
Truncal obesity, hirsutism, moon facies, acne, buffalo hump, purple striae, hypertension, diabetes, weakness, depression, easy bruising, myopathy
12
Q
How can cortisol levels be indirectly measured over a short duration?
A
By measuring urine cortisol or 17-OHCS (breakdown product of cortisol)
13
Q
What is a direct test of serum cortisol?
A
Serum cortisol level (highest in morning)
14
Q
What initial tests should be performed in Cushing’s syndrome?
A
Electrolytes, serum cortisol, urine-free cortisol, urine 17-OHCS, low-dose dexamethasone suppression test
15
Q
What is the low-dose dexamethasone suppression test?
A
Dexamethasone is a synthetic cortisol that results in negative feedback on ACTH secretion and subsequent cortisol secretion in healthy patients
16
Q
After the dexamethasone suppression test, what is done next?
A
Check ACTH levels
17
Q
Can plasma ACTH levels be checked directly?
A
Yes
18
Q
In ACTH-dependent Cushing’s syndrome, how do you differentiate between a pituitary vs. an ectopic ACTH source?
A
High-dose dexamethasone test
19
Q
What is the test for equivocal results for differentiating pituitary vs. ectopic ACTH tumor?
A
Bilateral petrosal vein sampling, especially with CRH infusion
20
Q
What is the most common site of ectopic ACTH tumor?
A
66% are oat-cell tumors of the lung
21
Q
How is adrenal adenoma treated?
A
Adrenalectomy (usually unilateral)
22
Q
How is adrenal carcinoma treated?
A
Surgical excision (33% are operable)
23
Q
How is an ectopic ACTH-producing tumor treated?
A
Surgical excision, if feasible
24
Q
How is Cushing’s disease treated?
A
Transphenoidal adenomectomy
25
What medication must be given to a patient who is undergoing surgical correction of Cushing's syndrome?
Cortisol (usually hydrocortisone until PO resumed)
26
What medications inhibit cortisol production?
Ketoconazole, metyrapone, aminoglutethimide, mitotane
27
What is the mechanism of action of ketoconazole?
Inhibits 11 beta-hydroxylase, c17-20 lyase, and cholesterol side-chain cleavage
28
What is the mechanism of action of aminoglutethimide?
Inhibits cleavage of cholesterol side chains
29
What is the mechanism of action of mitotane?
Inhibits 11 beta-hydroxylase and cholesterol side-chain cleavage
30
What is the mechanism of action of metyrapone?
Inhibits 11 beta-hydroxylase
31
What is a complication of bilateral adrenalectomy?
Nelson's syndrome
32
What is Nelson's syndrome?
Functional pituitary adenoma producing excessive ACTH and mass effect producing visual disturbances, hyperpigmentation, amenorrhea
33
What is an adrenal incidentaloma?
Tumor found in the adrenal gland incidentally on a CT scan
34
What is the incidence of adrenal incidentaloma?
4% of CT scans
35
What is the most common cause of adrenal incidentaloma?
Non-functioning adenoma
36
What is the differential diagnosis of adrenal incidentaloma?
Non-functioning adenoma, pheochromocytoma, adrenocortical carcinoma, aldosteronoma, metastatic disease, nodular hyperplasia
37
What is the treatment for adrenal incidentaloma?
Small/medium-sized: surgery controversial
| Large (> 6 cm): resection
38
What are the indications for removal of adrenal incidentaloma less than 6 cm?
MRI T2 signal > 2, hyper-functioning tumor, enlarging cystic lesion, doesn't look like adenoma
39
What tumor must be ruled out prior to biopsy or surgery for any adrenal mass?
Pheochromocytoma (24-hour urine for catecholamine, VMA, metanephrines)
40
What is pheochromocytoma?
Tumor of the adrenal medulla and sympathetic ganglion (from chromaffin cell lines) that produces catecholamines
41
Which age group is most likely to have pheochromocytoma?
Any age
42
What are the associated risk factors for pheochromocytoma?
MEN-II, family history, von Recklinghausen disease, von Hippel-Lindau disease
43
What are the signs and symptoms of pheochromocytoma?
1. Palpitations
2. Headache
3. Episodic diaphoresis
Also, hypertension, anxiety, weight loss, tachycardia, hyperglycemia, polycythemia
44
How can the pheochromocytoma symptoms triad be remembered?
PHEochromocytoma:
| Palpitations, Headache, Episodic diaphoresis
45
What is the most common sign of pheochromocytoma?
Hypertension
46
What is the differential diagnosis of pheochromocytoma?
Renovascular hypertension, menopause, migraine, carcinoid syndrome, preeclampsia, neuroblastoma, anxiety disorder with panic attacks, hyperthyroidism, insulinoma
47
What diagnostic tests should be performed for pheochromocytoma?
Urine screen: VMA, metanephrine, normetanephrine (breakdown products of catecholamines)
Urine/serum epinephrine, norepinephrine
48
What are the possible sites for pheochromocytoma?
Adrenal gland, organ of Zuckerkandl, thorax, bladder, scrotum
49
What are the pheochromocytoma localization tests?
CT, MRI, I-MIBG, PET scan, OctreoScan
50
What does I-MIBG stand for?
Iodine-131 MetaIodoBenzylGuanidine
51
How does the I-MIBG scan work?
I-MIBG is a norepinephrine analog that collects in adrenergic vesicles and, thus, in pheochromocytomas
52
What is the role of PET scan in pheochromocytoma?
Helpful in localizing pheochromocytomas that do not accumulate MIBG
53
What is the scan for imaging adrenal cortical pheochromocytoma?
NP-59 (a cholesterol analog)
54
What is the localizing option if a pheochromocytoma is not seen on CT, MRI or I-MIBG?
IVC venous sampling for catecholamines (gradient will help localize tumor)
55
What is the pheochromocytoma tumor site if epinephrine is elevated?
Must be adrenal or near the adrenal gland, because non-adrenal tumors lack the capability to methylate norepinephrine to epinephrine
56
What percentage of patients with pheochromocytoma have malignant tumors?
10%
57
Can histology be used to determine malignancy of a pheochromocytoma?
No; only distant metastasis or invasion
58
What is the classic pheochromocytoma "rule of 10"?
```
10% malignant
10% bilateral
10% in children
10% multiple tumors
10% extra-adrenal
```
59
What is the preoperative/medical treatment for pheochromocytoma?
Increase intravascular volume with alpha-blockers (e.g. phenoxybenzamine, prazosin) to allow reduction in catecholamine-induced vasoconstriction and resulting volume depletion.
Treatment should start as soon as diagnosis is make.
60
What is the surgical treatment for pheochromocytoma?
Tumor resection with early ligation of venous drainage and minimal manipulation (lower possibility of catecholamine crisis)
61
What are the possible perioperative complications of pheochromocytoma surgery?
Hypertensive crisis with manipulation (treat with nitroprusside); hypotension with total removal of tumor; cardiac dysrhythmias
62
In the patient with pheochromocytoma, what must be ruled out?
MEN-II
63
What is the organ of Zuckerkandl?
Body of embryonic chromaffin cells around the abdominal aorta (near IMA).
Normally atrophies during childhood, but is the most common site of extra-adrenal pheochromocytoma
64
What is Conn's syndrome?
Primary hyperaldosteronism due to high aldosterone production
65
How do you remember what Conn's syndrome is?
CONn's disease = HYPERALdosteronism:
| CON HYPER AL
66
What are the common sources of Conn's syndrome?
Adrenal adenoma or hyperplasia
67
What is the normal physiology for aldosterone secretion?
1. BP in the renal artery is low.
2. Low sodium and hyperkalemia cause renin secretion from juxtaglomerular cells.
3. Renin then converts to angiotensinogen to angiotensin I.
4. ACE in the lung then converts angiotensin I to angiotensin II.
5. Angiotensin II then causes the adrenal glomerulosa cells to secrete aldosterone.
68
What is the normal physiologic effect of aldosterone?
Sodium retention for exchange of potassium in the kidney, resulting in fluid retention and increased BP
69
What are the signs and symptoms of Conn's syndrome?
Hypertension, headache, polyuria, weakness
70
What are the 2 classic clues of Conn's syndrome?
1. Hypertension
| 2. Hypokalemia
71
Classically, what kind of hypertension is seen in Conn's syndrome?
Diastolic hypertension
72
What are the renin levels with Conn's syndrome?
Normal or decreased
73
What percentage of all patients with hypertension have Conn's syndrome?
1%
74
What diagnostic tests should be ordered for Conn's syndrome?
1. Plasma aldosterone concentration
| 2. Plasma renin activity
75
What ratio of aldosterone to renin is associated with primary hyperaldosteronism?
> 30:1
76
What is secondary hyperaldosteronism?
Results from abnormally high renin levels
77
What diagnostic tests should be performed for secondary hyperaldosteronism?
CT, adrenal venous sampling for aldosterone levels, saline infusion test
78
What is the saline infusion test?
Saline infusion will decrease aldosterone levels in normal patients but not in Conn's syndrome
79
What is the preoperative treatment for Conn's syndrome?
Spironolactone, K supplementation
80
What is spironolactone?
Anti-aldosterone medication (works at kidney tubule)
81
What are the causes of Conn's syndrome?
Adrenal adenoma, bilateral idiopathic adrenal hyperplasia, adrenal carcinoma
82
What is the treatment for adrenal adenoma?
Unilateral adrenalectomy
83
What is the treatment for unilateral adrenal hyperplasia?
Unilateral adrenalectomy
84
What is the treatment for bilateral adrenal hyperplasia?
Spironolactone (no surgery)
85
What are the renin levels in patients with primary hyperaldosteronism?
Normal or decreased
86
What is Addison's disease?
Acute adrenal insufficiency
87
What are the electrolyte findings in Addison's disease?
Hyperkalemia, hyponatremia
88
What is an insulinoma?
Insulin-producing tumor arising from beta cells
89
What are the associated risk factors for insulinoma?
MEN-I
| PPP = Pituitary, Pancreas, Parathyroid tumors
90
What are the signs and symptoms of insulinoma?
Sympathetic nervous system symptoms resulting from hypoglycemia: palpitations, diaphoresis, tremulousness, irritability, weakness
91
What are the neurologic symptoms of insulinoma?
Personality changes, confusion, obtundation, seizures, coma
92
What is Whipple's triad?
1. Hypoglycemic symptoms produced by fasting.
2. Blood glucose < 50 mm/dL during symptomatic attack
3. Relief of symptoms by administration of glucose
93
What is the differential diagnosis for insulinoma?
Reactive hypoglycemia, functional hypoglycemia with gastrectomy, adrenal insufficiency, hypopituitarism, hepatic insufficiency, Munchausen syndrome (insulin self-injections), non-islet cell tumor causing hypoglycemia, surreptitious administration of insulin or OHAs
94
What lab tests should be performed for insulinoma?
Glucose and insulin levels during fast; C-peptide and proinsulin levels (if self-injection is a concern, as insulin injections have no proinsulin or C-peptides)
95
What diagnostic tests should be performed for insulinoma?
Fasting hypoglycemia with inappropriately high levels of insulin; 72-hour fast, then check glucose and insulin levels q6hr
96
What is the diagnostic fasting insulin:glucose ratio for insulinoma?
> 0.4
97
What localizing tests should be performed for insulinoma?
CT, A-gram, endoscopic U/S, venous catheterization (to sample blood along portal and splenic veins), intra-operative U/S
98
What is the medical treatment of insulinoma?
Diazoxide (suppress insulin release)
99
What is the surgical treatment of insulinoma?
Resection
100
What is the prognosis for insulinoma?
80% of patients have benign solitary adenoma that is cured by surgical resection
101
What is a glucagonoma?
Glucagon-producting tumor
102
Where is a glucagonoma located?
Pancreas, usually the tail
103
What are the symptoms of glucagonoma?
Necrotizing migratory erythema (usually below the waist), glossitis, stomatitis, diabetes
104
What are the skin findings with glucagonoma?
Necrotizing migratory erythema is a red, often psoriatic-appearing rash with serpiginous borders over the trunk and limbs
105
What are the associated lab findings with glucagonoma?
Hyperglycemia, low amino acid levels, high glucagon
106
What is the classic CBC finding with glucagonoma?
Anemia
107
What is the classic nutritional finding with glucagonoma?
Low amino acid levels
108
What stimulation test is used for glucagonoma?
Tolbutamide stimulation test: IV tolbutamide results in elevated glucagon levels
109
What test is used for localization of glucagonoma?
CT
110
What is the medical treatment of necrotizing migratory erythema?
Somatostatin, IV amino acids
111
What is the treatment of glucagonoma?
Surgical resection
112
What is a somatostatinoma?
Pancreatic tumor that secretes somatostatin
113
What is the diagnostic triad of somatostatinoma?
1. Diabetes
2. Diarrhea (steatorrhea)
3. Dilation of the gallbladder with gallstones
114
What is used to make the diagnosis of somatostatinoma?
CT and somatostatin levels
115
What is the treatment of somatostatinoma?
Surgical resection (no not enucleate)
116
What is the medical treatment for unresectable somatostatinoma?
Streptozocin, decarbazine, or doxorubicin
117
What is Zollinger-Ellison syndrome?
Gastrinoma: non-beta islet cell tumor of the pancreas (or other locale) that produces gastrin, causing gastric hyper secretion of HCl acid, resulting in GI ulcers
118
What syndrome is associated to ZES?
MEN-I
119
What percentage of patients with ZES have MEN-I syndrome?
25%
120
What percentage of patients with MEN-I will have ZES?
50%
121
With ZES, what lab tests should be ordered to screen for MEN-I?
1. Calcium level
| 2. Parathyroid hormone level
122
What are the symptoms of ZES?
Peptic ulcers, diarrhea, weight loss, abdominal pain
123
What causes diarrhea in ZES?
Massive acid hypersecretion and destruction of digestive enzymes
124
What are the signs of ZES?
PUD (epigastric pain, hematemesis, melena, hematochezia), GERD, diarrhea, recurrent ulcers, ulcers in unusual locations
125
What are the possible complications of ZES?
GI hemorrhage, GI perforation, gastric outlet obstruction/stricture, metastatic disease
126
What is the differential diagnosis of ZES?
Post-vagotomy, gastric outlet obstruction, G-cell hyperplasia, pernicious anemia, atrophic gastritis, short gut syndrome, renal failure, H2 blocker, PPI
127
Which patients should have a gastrin level checked?
Recurrent ulcer; ulcer in unusual position; ulcer refractory to medical management; before any ulcer operation
128
What lab tests should be performed for ZES?
Fasting gastrin level; post-secretin challenge gastrin level; Ca (MEN-I); BMP
129
What are the associated gastrin levels in ZES?
Normal fasting = 100 pg/mL
| ZES fasting = 200-1000 pg/mL
130
What is the secretin stimulation test?
IV secretin is administered and the gastrin level is determined.
Patients with ZES have a paradoxic increase.
131
What are the classic secretin stimulation results?
```
Normal = decreased gastrin
ZES = increased gastrin
```
132
What tests are used to evaluate ulcers?
EGD, UGI, or both
133
What tests are used to localize a ZES tumor?
Octreotide scan (somatostatin receptor scan); abdominal CT; MRI; endoscopic U/S
134
What is the most common site of a ZES tumor?
Pancreas
135
What is the most common non-pancreatic site of a ZES tumor?
Duodenum
136
What are possible sites for a ZES tumor?
Pancreas, duodenum, stomach, lymph nodes, liver, kidney, ovary
137
What is the next step if the ZES tumor cannot be localized?
Exploratory surgery (pancreas, duodenum); proximal gastric vagotomy if not found
138
What is the medical treatment for ZES?
H2 blockers, omeprazole, somatostatin
139
What is the surgical treatment for gastrinoma in the head of the pancreas?
Enucleation of tumor; Whipple procedure if main pancreatic duct is involved
140
What is the surgical treatment for gastrinoma in the body or tail of the pancreas?
Distal pancreatectomy
141
What is the surgical treatment for gastrinoma in the duodenum?
Local resection
142
What is the surgical treatment for unresectable gastrinoma?
High selective vagotomy
143
What percentage ZES tumors are malignant?
66%
144
What is the most common site of metastasis in ZES?
Liver
145
What is the treatment for ZES patients with liver metastasis?
Excision, if feasible
146
What does MEN stand for?
Multiple Endocrine Neoplasia
147
What is MEN syndrome?
Inherited condition of propensity to develop multiple endocrine tumors
148
How is MEN inherited?
Autosomal dominant (but with varying penetrance)
149
Which patients should be screened for MEN?
All family members of patients diagnosed with MEN
150
What is Wermer's syndrome?
MEN-I
151
Where is the gene defect in MEN-I?
Chromosome 11
152
What are the most common tumors and incidences in MEN-I?
```
PPP:
Parathyroid hyperplasia (90%)
Pancreatic tumors (ZES, insulinoma) (66%)
Pituitary tumors (50%)
Also, adrenal and thyroid adenomas
```
153
What is Sipple's syndrome?
MEN-IIa
154
What is the gene defect in MEN-IIa?
RET
155
What are the most common tumors and incidences in MEN-IIa?
MPH:
Medullary thyroid carcinoma (calcitonin) (100%)
Pheochromocytoma (33%)
Hyperparathyroidism (50%)
156
What are the most common abnormalities, incidences and symptoms in MEN-IIb?
```
MMMP:
Mucosal neuromas (100%): nasopharynx, oropharynx, larynx, conjunctiva
Medullary thyroid carcinoma (85%)
Marfanoid body habitus
Pheochromocytoma (50%)
```
157
What is the anatomic distribution of medullary thyroid carcinoma in MEN-IIb?
Almost always bilateral
158
What is the most common GI complaint of patients with MEN-IIb?
Constipation resulting from ganglioneuromatosis of GI tract
159
What is the major difference between MEN-IIa and MEN-IIb?
MEN-IIa: parathyroid hyperplasia
| MEN-IIb: neuromas, marfanoid habitus, pes cavus
