Endocrine pt 2

Question 1

Describe the pathogenesis of pituitary adenomas

Answer 1

Endocrine tissue proliferates more readily than does neural
1) Therefore, most pituitary tumors are regulated to the ant. Organ
2) Pituitary adenoma are MC cause of hyper/hyposecretion syndromes in adults
-Account for 7 cases per 100,000 people, usually 3rd to 6th decade of life
3) 5 types of neoplasm arising from 5 cell types:
-Lactotropes, corticotropes, thyrotropes, somatotropes, gonaditropes

Question 2

Pituitary adenomas: Descr. classification based on size

Answer 2

Macroadenoma > 1cm
Microadenoma < 1cm

Question 3

Describe Hormone secreting/ “functional” pituitary adenoma symptoms

Answer 3

Symptoms of hormone excess* sex may influence this
Because they are symptomatic, likely discovered while small
Microadenomas < 1cm

Question 4

Describe Non-hormone secreting/ “hypofunctioning”
pituitary adenomas

Answer 4

1) Sx: none regarding hormone release
2) Therefore, the tumor grows to become large
Macroadenoma > 1cm
Instead, the symptoms are structural i.e. from compression of optic chiasm
Bitemporal hemianopsia

Question 5

Pituitary Adenoma: Describe the pt if functioning or nonfunctioning adenomas

Answer 5

1) Nonfunctioning Adenoma: Accounts for 1/3 of adenomas
Asymptomatic, if small
Produce no clinically distinct secretory syndrome
2) Functioning Adenoma
Clinical picture of hyper/hyposecretion

Question 6

Pituitary adenoma:
1) Dx?
2) Tx?

Answer 6

1) Diagnosed with MRI
But if they are small and nonfunctioning, they are often discovered incidentally on MRI!
Check visual fields on exam!
2) Microadenomas get followed with serial MRI
Macroadenomas are candidates for transsphenoidal resection

Question 7

What are some complications of pituitary adenomas? Why?

Answer 7

1) Tumor size and degree of invasiveness determine surgical complications
2) Transient DI and hypopituitarism occur in 20% of patients
Why? Because of additional damage to the post. pituitary

Question 8

Pituitary adenomas: Describe cabergoline Tx for if the pt can’t get surgery

Answer 8

Oral dopamine agonist can be given in some cases
Successful for tumors that secrete prolactin and even GH
Yes, they will shrink 1/3 of acromegaly associated pituitary tumors
Safe during pregnancy

Question 9

Pituitary adenomas: Describe radiation Tx for if the pt can’t get surgery

Answer 9

Takes years to work
Patient may require interim medical therapy
Radiation will damage function of hypothalamic-pituitary axis with high risk of additional disease
Not optimal therapy

Question 10

Hyperprolactinemia: List the many causes (pathogen)

Answer 10

1) Prolactinoma is MC
2) Hypothyroidism
3) Acromegaly
4) Antipsychotics
5) Cirrhosis
6) Renal failure
7) Physiologic causes
-Pregnancy
-Stress
-Exercise

Question 11

Hyperprolactinemia:
1) Describe the presentation in women
2) Describe the presentation in men

Answer 11

1) Amenorrhea > oligomenorrhea
-Infertility
-Vaginal dryness
-Galactorrhea (relatively rare)
-Hypogonadism
2) Infertility
-Erectile dysfunction
-Decreased libido
-Gynecomastia (rare)
-Hypogonadism

Question 12

What 3 Sxs do men and women have in common with hyperprolactinemia?

Answer 12

-If adenoma; may have bitemporal hemianopsia
-Hypogonadism
-Infertility

Question 13

Hyperprolactinemia: How do you Dx?

Answer 13

1) Check pregnancy test
2) Check med list
3) Check TSH
4) Then check:
-Prolactin (high)
-FSH/LH (low)
-GH, ACTH (either high or low - prolactinomas can cause hyper/hyposecretion of other hormones)
5) MRI is diagnostic for adenoma

Question 14

Hyperprolactinemia:
1) How do you Tx if med induced?
2) How do you Tx if Adenoma or other?
3) What if this Tx doesn’t work?

Answer 14

1) Stop, reduce, or change the med
-1st and 2nd gen antipsychotics
-Metoclopramide, promethazine, prochlorperazine
2) Dopamine agonists: Cabergoline or bromocriptine are both first line; Cabergoline is better tolerated
3) Transsphenoidal surgery if adenoma is greater than 3cm
-Radiation rarely used

Question 15

GH tumor: Describe the pathogenesis and demographic

Answer 15

1) Pituitary adenoma hypersecreting GH
2) 40% of these come from genetic mutation, usually sporadic
3) Most often occurs 30’s-50’s

Question 16

Pathological IGF-1 levels have what affects on:
1) Heart
2) Bones
3) Liver and spleen
4) Metabolism

Answer 16

1) Diastolic heart failure
2) Hypertrophy and linear growth (if growth plates are not fused)
3) Hepatosplenomegaly
4) HTN, DM, HLD, obesity

Question 17

GH tumor: Describe gigantism

Answer 17

1) If hypersecreting tumor occurs before puberty, pt will be tall, and proportionally developed
2) They will still develop the pathological states of internal organs

Question 18

GH tumor: Describe acromegaly

Answer 18

1) If hypersecreting tumor occurs after puberty, the epiphysial plates will be closed and pt will have bone growth without elongation
2) Jaw, hands, feet, will all continue to thicken

Question 19

Describe the Sx in a pt with a GH tumor (many)

Answer 19

1) Diabetes or glucose intolerance
2) Enlargement of soft tissues, cartilage and bone
-Hands, feet, skull, tongue, forehead, jaw
-Carpal tunnel
-OSA
-Increased spacing between teeth
3) Headache is common
4) Deepened voice
5) Weight gain
6) Arthralgia
7) Hypertension
8) Kidney stones
9) Colonic polyps!
10) Heart failure
11) Bitemporal hemianopsia

Question 20

How do you initially Dx a GH tumor?

Answer 20

Insulin-like growth factor test
You may do this at random because it’s release is pulsatile
Check a prolactin too, because there is so often overlap

Question 21

How do you follow-up Dx a GH tumor (after initial Dx)?

Answer 21

1) Oral glucose suppression test; growth hormone levels increase in acromegaly
-A normal response would be GH suppression
2) How does this work again?
a) Normal: Glucose goes in > blood glucose goes up > insulin goes UP > GH goes down
b) Abnormal: Glucose goes in > blood glucose goes up > insulin goes UP > GH does what it wants. . .
It stays up because of the tumor

Question 22

How do you confirm Dx of GH tumor?

Answer 22

MRI will be confirmatory

Question 23

How do you Tx GH tumors? Explain

Answer 23

1) Transsphenoidal surgery is preferred
2) Medical management:
-Either in addition to surgery or when surgery is not possible
-Octreotide or Lanreotide is first line: Somatostatin inhibits GH release
-Sometimes pegvisomant is given (GH receptor antagonist)
3) Radiation therapy is not optimal & is reserved for other treatment failure

Question 24

GH Deficiency: What are the 3 etiologies?

Answer 24

Genetic mutation
Pituitary infarct
Radiation therapy

Question 25

GH deficiency: How does it present in children?

Answer 25

1) Less than the 3rd percentile 2) Radiographs show: Bone age > 2yr behind chronologic age 3) Delayed puberty 4) Fasting hypoglycemia

Question 26

GH deficiency: How does it present in adults?

Answer 26

1) Mild-moderate obesity, increased blood pressure, dyslipidemia 2) Decreased bone mass, decreased cardiac output, muscle wasting 3) Increased inflammatory markers

Question 27

GH Deficiency: How is it diagnosed?

Answer 27

1) MRI of head to assess for pituitary tumor 2) X-ray of wrist and hand for bone age assessment 3) Arginine and sleep stimulation test -No change in GH release 4) Random measurements of GH hormone are not helpful

Question 28

GH Deficiency: How is it treated?

Answer 28

Growth hormone supplementation before the growth plates close

Question 29

Hypopituitarism: Describe the 3 methods of chronic pathogenesis

Answer 29

1) Tumor -Nonfunctioning adenoma -Craniopharyngioma 2) Infiltrative disease -Sarcoidosis -Other autoimmune destruction -Radiation therapy 3) Adaptation to injury -Pituitary will prioritize which hormones to secrete TSH, ACTH -It will “cut out” GH, FSH/LH/prolactin

Question 30

Hypopituitarism: Describe the 3 methods of acute pathogenesis

Answer 30

1) Infection, infarction, hemorrhage, iatrogenic (resection/rads) 2) Pituitary apoplexy: pre-existing adenoma that hemorrhages 3) Sheehan's: Post partum hemorrhage > hypotension > pituitary infarct

Question 31

Can adaptation to acute hypopituitarism occur?

Answer 31

Cannot occur; pt has hypotension, lethargy, coma, or death

Question 32

Describe the pt in chronic and acute hypopituitarism

Answer 32

1) Chronic: Clinical manifestations of hormone deficiencies, dwarfism, metabolic derangements, hypothyroidism, or bitemporal hemianopsia 2) Acute: Female with post partum hemorrhage -Head trauma patient -Hypotension, AMS, unstable

Question 33

Hypopituitarism: 1) Dx? 2) Tx?

Answer 33

1) Target hormones AND pituitary hormones decreased in the same direction 2) Lifelong hormone replacement to give them what they lack

Question 34

Pituitary Apoplexy is related to?

Answer 34

Hypopituitarism

Question 35

Hypercortisolism: Describe the 2 methods of pathogenesis

Answer 35

1) Pituitary hypOcortisolism -Probably panhypopituitarism -Low ACTH -Atrophy of adrenals (However, zona glomerulosa will still respond to Angiotensin II and to hyperkalemia, so Aldosterone will still be made) -Low cortisol 2) Adrenal insufficiency: This will be covered in the next section

Question 36

Hypercortisolism: 1) What 2 things will you see in the pt? 2) What is the Dx/ Tx?

Answer 36

1) Low BP and lethargy 2) Low cortisol/low ACTH Give them what they lack Glucocorticoids

Question 37

Hypercortisolism: Describe the pt presentation/ Sx

Answer 37

1) Central/truncal obesity 2) Moon facies: roundly shaped, puffy face 3) Skin atrophy, striae, easy bruising bleeding, decreased wound healing 4) Acanthosis nigricans: Epidermal hyperplasia and thickening of skin around neck and armpit with hyperinsulinemia 5) Hypertension 6) Androgen excess = hirsutism, oily skin, acne

Question 38

Describe cortisol under normal function

Answer 38

1) Hypothalamus > corticotropin releasing hormone > corticotropes release > adrenocorticotropic hormone > fasciculata releases cortisol 2) ACTH inhibits CRH 3) Cortisol inhibits ACTH and CRH

Question 39

List and describe 3 main effects of hypercortisolism

Answer 39

1) **HTN** -Alpha-1 agonism -Production of vasopressors 2) **DM**: Hyperglycemia 3) **Obesity**: Central obesity “Purple Striae, buffalo hump, moon facies”

Question 40

1) Describe cortisol synthesis 2) What does this have to do with testing for hypercortisolism? 3) What is an alternative test?

Answer 40

1) Levels peak in early morning, usually just after waking Therefore, cortisol levels should be lowest late at night 2) Therefore, patients with refractory symptoms of HTN, DM, Central obesity should have their late-night cortisol checked -Why? Because this is when it should be low; an elevated level is thus more diagnostic 3) Alternative testing is 24-hour urine is most specific

Question 41

1) What test should you do for hypercortisolism after one of the first two comes back with high cortisol? 2) Why? 3) How do you interpret the results?

Answer 41

1) Give low-dose dexamethasone suppression test 2) Giving this artificial glucocorticoid should suppress the hypothalamus and the anterior pituitary -This, in turn, should suppress the adrenal gland’s production of cortisol 3) If there is not suppression of cortisol, they have an illness of hypercortisolism -If there is suppression of cortisol, they have no such illness, and your work up is done

Question 42

So, the low-dose test didn’t suppress cortisol. Now what?

Answer 42

1) They have hypercortisolism, but you don’t know where in the axis it is coming from 2) Check ACTH: a) If it is low, then the axis is responding to the dexamethasone suppression, and you know the cortisol is coming from somewhere outside the axis -Adrenal adenoma! b) If ACTH is still high, then the illness is ACTH dependent, meaning ACTH production is the cause! -Pituitary adenoma -Paraneoplastic disease

Question 43

So, the ACTH remains high after dexamethasone suppression, now what? Explain how to interpret

Answer 43

1) The endocrine axis will respond to a high enough dose of counterregulatory hormone. 2) This means that a high-dose dexamethasone suppression test will cause a drop in ACTH if the source is a pituitary adenoma -If the source is a small-cell lung cancer, the ACTH will be high no matter what!

Question 44

Describe Primary hypercortisolism

Answer 44

1) This is the patient with adrenal tumor 2) Because the cortisol is always high, there is consistent negative feedback decreasing CRH and ACTH 3) Because ACTH is therefore low, the remaining adrenal tissue atrophies due to lack of stimulation

Question 45

Describe Secondary Hypercortisolism

Answer 45

ACTH dependent illness 1) Whether from pituitary adenoma or malignancy, this patient will have very high levels of ACTH 2) Because ACTH is always high, the adrenal glands will hypertrophy 3) Because ACTH is always high, melanocytes will be stimulated and will demonstrate hyperpigmentation Image the lungs to evaluate tumor  confirmed with biopsy

Question 46

Hypercortisolism: How to Tx pituitary adenoma?

Answer 46

Find it and cut it out MRI  petrosal sinus sampling if needed  transsphenoidal resection

Question 47

Hypercortisolism: How to Tx adrenal adenoma?

Answer 47

Find it and cut it out

Question 48

Hypercortisolism: How to Tx endocrine secreting tumor?

Answer 48

Find it and cut it out If nonresectable, ketoconazole or metyrapone can be given

Question 49

Hypercortisolism: How to Tx (Other) Exogenous use of steroid?

Answer 49

Because these patients have high levels of cortisol which will decrease the hormones produced upstream This will drop their ACTH and atrophy of the adrenal glands This is why patients on long term steroids need to be tapered Treatment is to d/c steroid

Question 50

HypOcortisolism / HypOaldosteronism: Describe primary adrenal insuficiency (Addison's)

Answer 50

Autoimmune destruction of the adrenal glands 80% of US cases Aldo and cortisol will both be low

Question 51

HypOcortisolism / HypOaldosteronism: List and describe some causes other than Addison's

Answer 51

1) Infection: more common in developing countries (TB, HIV) 2) Thrombosis or hemorrhage of the adrenal gland Waterhouse-friderichsen syndrome 3) Other: trauma, metastatic disease 4) Abrupt withdrawal of long-term steroids 5) Meds: Ketoconazole (inhibits cortisol and aldosterone synthesis)

Question 52

Primary adrenal insufficiency: What are the 2 main types? Describe

Answer 52

1) Chronic = Addison’s disease 2) Acute = almost dead (Addison’s crisis) Lethargy  coma  death Hypotension  shock  death

Question 53

Primary adrenal insufficiency: Describe the pathogenesis

Answer 53

Primary adrenal insufficiency = Adrenal glands not working 1) Low cortisol Hypotension Lethargy 2) Low aldosterone Salt-wasting Decreased blood volume Hyperkalemia

Question 54

HypOcortisolism / HypOaldosteronism: 1) Describe the pt if chronic 2) Describe the pt if acute

Answer 54

1) Hypotensive, Fatigued -Because the adrenals have failed and cortisol will be low, ACTH will be high, so hyperpigmentation occurs 2) Hypotensive > You give fluids > you give pressors = Still hypotensive

Question 55

HypOcortisolism / HypOaldosteronism: Describe the 4 steps of Dx of chronic disease

Answer 55

1) **8am cortisol and renin levels** In primary adrenal insufficiency, renin will be elevated (trying to juice the adrenals) 2) **ACTH** Will be elevated in primary Will be decreased in secondary disease (that’s hypopituitarism) 3) **CMP** Hypoglycemia Hyponatremia, hyperkalemia, non-anion gap metabolic acidosis due to decreased aldosterone 4) **High-dose ACTH cosyntropin stim test** Normal response is for cortisol to rise if ACTH is given Adrenal insufficiency if insufficient or absent rise in serum cortisol after ACTH administration

Question 56

HypOcortisolism / HypOaldosteronism: 1) How to Tx chronic? 2) How to Tx acute?

Answer 56

1) Glucocorticoid replacement: hydrocortisone Mineralocorticoid replacement: fludrocortisone 2) High dose hydrocortisone!

Question 57

HypOcortisolism / HypOaldosteronism: Describe further management

Answer 57

1) Because cortisol is a stress hormone, patients will need IV glucocorticoids and IV fluids before and after surgical procedures This is to mimic the body’s natural response 2) During illness/surgery/fever, oral dosing needs to be adjusted as well 3) They need a medical alert tag

Question 58

Hyperaldosteronism: Describe the primary pathogenesis

Answer 58

1) Idiopathic bilateral adrenal hyperplasia; most common 60% 2) Conn syndrome (adrenal aldosteronoma) 40% 3) Rarely: unilateral adrenal hyperplasia, familial, or ectopic aldosterone secreting tumor

Question 59

Hyperaldosteronism: List the secondary pathogenesis

Answer 59

1) Renal artery stenosis 2) Renal hypoperfusion: CHF, renal failure

Question 60

Describe the Sx of hyperaldosteronism

Answer 60

1) Often asymptomatic 2) Triad: Hypertension, hypokalemia, metabolic alkalosis 3) Hypokalemia: Muscle weakness, polyuria (nephrogenic DI), fatigue, constipation, decreased DTR, hypomagnesemia 4) Hypertension: Headache, flushing

Question 61

Hyperaldosteronism Dx: 1) What should you check? Why? 2) What will you see on CMP? 3) What will you see on EKG?

Answer 61

1) Check renin and aldosterone levels Ratio of aldo to renin > 20:1 indicates primary disease CMP 2) Hypokalemia with metabolic alkalosis -Aldosterone promotes sodium retention at the expense of potassium and hydrogen ion excretion 3) Get an EKG -Hypokalemia = T wave flattening and prominent U wave

Question 62

Hyperaldosteronism Dx cont.: 1) Normally, giving salt would decrease aldosterone. . . therefore what would be seen in primary? 2) Why should you do CT or MRI? 3) What sampling should you do? Why?

Answer 62

1) Oral sodium loading test: high urine aldo in primary Saline infusion test: no suppression of aldo levels in primary 2) CT or MRI looking for adrenal or extra-adrenal mass 3) Venous sampling from the adrenal glands -Differentiates adenoma vs adrenal hyperplasia

Question 63

Hyperaldosteronism: 1) What is the first line Tx? 2) What is the second line Tx? 3) What else should you do? 4) What should you do in Conn's syndrome?

Answer 63

1) 1st Spironolactone -Blocks aldosterone 2) ACE inhibitors, CCB 3) Correct electrolyte abnormalities 4) Find it and cut it out

Question 64

What does ADH do normally?

Answer 64

1) It is secreted by the post. pituitary 2) It is received by ADH receptors in the kidney 3) It up regulates transcription of aquaporin proteins 4) These allow the kidney to resorb water

Question 65

SIADH “Syndrome of inappropriate ADH”: 1) What is it? 2) What is the main effect?

Answer 65

1) An excess of ADH upregulates aquaporin production and exaggerates the effect of normal ADH function 2) Namely, concentrated urine and dilute blood

Question 65

SIADH: Describe the 3 main etiologies (pathogen)

Answer 65

1) CNS: Subarachnoid hemorrhage, stroke, head trauma, meningitis, CNS tumor, hydrocephalus 2) Pulmonary: small cell lung cancer, legionella, HIV 3) Meds: Desmopressin, anticonvulsants, carbamazepine, HCTZ, antidepressants (TCA, SSRI), IV cyclophosphamide, ecstasy (MDMA)

Question 66

SIADH: How does the pt present?

Answer 66

If progressed far enough, you may see the effects of hyponatremia and cerebral edema -Confusion, AMS, lethargy > Coma

Question 67

SIADH Dx: Describe the diluteness of the blood

Answer 67

These patients will have dilute blood Blood is dilute because of the excess ADH How can you tell their blood is dilute? They will have a low “serum osmolality” on lab

Question 68

SIADH Dx: Describe the blood volume

Answer 68

These patients will have a normal blood volume Volume is normal because aldosterone is functioning normally They will not have increased JVP, crackles, edema or other signs of volume overload A patient with normal volume status is said to be “euvolemic”

Question 69

SIADH Dx: 1) Describe the sodium. 2) Why?

Answer 69

1) These patients will have low sodium 2) Why? This has nothing to do with salt resorption. Salt is low because the excess water resorption is diluting the salt. When a patient has low serum osm and low sodium, this is called “hypoosmolar hyponatremia”

Question 70

SIADH: When patient has low sodium, low serum osmolality, and normal blood volume, they have “________________________________”

Answer 70

euvolemic hypoosmolar hyponatremia

Question 71

SIADH: How do you Tx?

Answer 71

1) Treat underlying cause 2) Correct Hyponatremia -Mild: water restriction -Mod-severe: ADH receptor antagonists – conivaptan, tolvaptan -Severe hyponatremia: IV hypertonic saline + furosemide

Question 72

What occurs with ADH under normal function?

Answer 72

ADH is released from post. pituitary It is then received by receptors on the kidney

Question 73

There are thus two mechanisms for injury causing DI; explain

Answer 73

1) Central DI: Loss of production from pituitary 2) Nephrogenic DI: Loss of function of the receptor

Question 74

DI: 1) Describe the pt 2) Describe the pt if severe

Answer 74

1) Polyuria up to 20L per day + polydipsia -High volume nocturia 2) Severe: -Dehydration, hypotension, rapid vascular collapse -Hypernatremia: Confusion, lethargy, disorientation, seizures, coma

Question 75

DI: How is it diagnosed?

Answer 75

1) Increased serum osm 2) Decreased urine osmolality and specific gravity 3) Increased urine volume 4) +/- hypernatremia

Question 76

DI Dx cont.: Patients with very high urine output and dilute urine are put on water restriction; what will the results of this tell you?

Answer 76

1) If urine osmolality rises, they have psychogenic polydipsia. -Tx: “stop doing that” 2) If urine remains dilute, they have DI. But which type? -Give ADH. If this concentrates the urine, the problem was central -If ADH is given and the urine does NOT concentrate, the problem is nephrogenic.

Question 77

DI: 1) How do you Tx central? What is 1st line + what is 2nd? 2) How do you Tx nephrogenic?

Answer 77

1) Desmopressin first line – intranasal injection or oral -Second line is carbamazepine 2) Correct underlying cause; sodium and protein restriction -Hydrochlorothiazide, indomethacin, amiloride (for lithium induced)

Question 78

Pheochromocytoma: What is the pathogenesis and epidemiology?

Answer 78

1) Catecholamine-secreting adrenal tumor of chromaffin cells 2) Responsible for 0.1-0.5% of HTN, so rare -Nonetheless it is the most common adrenal tumor in adults 4) Associated with MEN syndromes 5) 90% benign  rule of 10s 10% malignant 10% bilateral 10% seen in children 10% extra-adrenal (paraganglioma)

Question 79

Pheochromocytoma: Explain the rule of 10s

Answer 79

90% benign  rule of 10s 10% malignant 10% bilateral 10% seen in children 10% extra-adrenal (paraganglioma)

Question 80

Pheochromocytoma: Describe the pt presentation

Answer 80

1) Hypertension! 2) PHEochromocytoma Palpitations Headache (most common symptom) Excessive sweating Chest or abdominal pain, weakness, fatigue, weight loss with increased appetite, pallor

Question 81

Pheochromocytoma: Describe the 2 steps of Dx

Answer 81

1) Labs Plasma fractionated metanephrines confirmed by 24-hour fractionated catecholamines Positive if increased metanephrines and vanillylmandelic acid 2) Imaging MRI or CT of abdomen/pelvis Done after laboratory testing

Question 82

Pheochromocytoma Dx: What if labs and imaging are both negative

Answer 82

Nuclear isotope scanning (MIBG scanning) Finds tumors outside the adrenal gland

Question 83

Pheochromocytoma: DO NOT initiate therapy with _________________ because of unopposed alpha constriction during catecholamine release which can lead to hypertensive crisis

Answer 83

beta-blockade

Question 83

Pheochromocytoma: What is the initial therapy? What do you do next?

Answer 83

1) Nonselective alpha-blockade is initial therapy PHEnoxybenzamine or PHEntolamine for 1-2 weeks Both of these block alpha cascade 2) Then beta blockers or CCB to control blood pressure

Question 84

Pheochromocytoma: How do you Tx patients in hypertensive crisis?

Answer 84

Phentolamine, nitroprusside, nicardipine can all acutely lower blood pressure

Question 85

MEN I (Also called Wermer’s syndrome): Describe the pathogenesis

Answer 85

Endocrine gland tumor Parathyroid, pancreas, pituitary Associated with Menin gene defect Usually benign

Question 86

MEN I: Describe the pt

Answer 86

Parathyroidism, usually hyperparathyroidism Pancreatic tumor: gastrinomas (Zollinger Ellison syndrome), insulinomas, glucagonomas, VIPomas, somatostaninomas Prolactinomas Others: carcinoid, lipomas, other nonmalignant tumors

Question 87

MEN 1: 1) Dx 2) Tx 3) F/u

Answer 87

1) Genetic testing 2) Tumor specific 3) Screen PTH, Calcium, gastrin, prolactin

Question 88

MEN II: 1) Pathogenesis? 2) What will you see in the pt in MEN IIa? 3) What abt in MEN IIb?

Answer 88

1) Inherited disorder of endocrine gland tumors RET proto-oncogene 2) Medullary thyroid cancer, pheochromocytoma, hyperparathyroidism 3) Medullary thyroid carcinoma, pheochromocytoma, neuromas/marfanoid habitus

Question 89

MEN II: 1) Dx 2) Tx 3) F/u

Answer 89

1) Genetic testing 2) Tumor specific 3) Screen calcitonin, epinephrine, PTH, calcium