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35 Cards in this Set

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what is meant by incidentalomas?
Approximately 10% of people undergoing pituitary imaging will have abnormalites seen in the anterior pituitary.

Adenomas
Empty sella- can't visualize on MRI
Uncertainties
Galactorrhea in women
Hypogonadism in women or men

pituitary Hormone involved?
Prolactin
Cushing’s disease (one of the causes of Cushing’s Syndrome).
Adrenal insufficiency

pituitary Hormone involved?
ACTH
Giantism/Acromegaly
Short stature

pituitary Hormone involved?
GH
hypogonadism
precocious puberty

pituitary Hormone involved?
LH/FSH
Hyperthyroidism or hypothyroidism

pituitary Hormone involved?
TSH
which hormone is most important to fix the regulation of first?

*
ACTH

(cortisol)
All pituitary hormones (all hormones everywhere for that matter) are secreted in response to a stimulus to do so except for one...which one is that?
PROLACTIN .

The secretion of prolactin is under static inhibition by constant release of dopamine from the hypothalamus.

Anything that inhibits that outflow results in elevated levels of prolactin (meds are common culprits).

if you have a growth on the tumor >1cm then the tumor is probably pinching on the stalk that is releasing DA (thus preventing inhibition of prolactin)
What is normally the cause of hyperprolactinemia?
DRUGS
44 y/o woman has an MRI of her brain done to evaluate headaches. Her history includes depression for which she takes Prozac. She also has noticed occasional breast discharge and amenorrhea for the past year. MRI reveals a 5 mm adenoma. Her prolactin level is 90 ng/ml. Is this a PROLACTINOMA?
MAYBE. The adenoma is kinda small, and she is on meds...though to tell which is the cause

NOT LIKELY THOUGH... Prolactinomas that are large enough to see on imaging studies result in prolactin levels >150 ng/ml
44 y/o woman has noticed occasional breast discharge and amenorrhea for the past year. She has a history of depression for which she takes Prozac. Laboratory evaluation reveals a prolactin level of 90 ng/ml. What is the likely cause of her hyperprolactinemia? Should you order an imaging study (MRI) of her pituitary?
What is the likely cause of her hyperprolactinemia?
Always consider pregancy, medications, chest wall trauma or stimulation and hypothyroidism as the cause
Most likely cause here is medication

Should you order an imaging study (MRI) of her pituitary?
YES – but keep in mind that as many as 10% of normal individuals have an abnormal pituitary MRI
Nonfunctioning pituitary tumors cause problems only by their ...
MASS EFFECT (dun dun dun)

start pushing on the optic chiasm..visual field defects
If causing a problem the treatment of choice for nonfunctioning tumors is?
surgery

note: If not causing a problem, repeat MRI in 6 months. If it’s growing, take it out. If stable, continue to observe periodically for growth.
most common “functioning” pituitary tumors produce prolactin at levels...
>150
Hyerprolacinemia can cause hypogonadism no matter what the cause. Why?
Interferes with GnRH Pulsations.
Interferes with gonadotropin action on the gonads.
The treatment of choice for prolactinomas is
MEDICAL (dopamine agonists).

Most prolactinomas respond favorably to dopamine agonists with normalization of prolactin and a reduction of the tumor size.
Approximately 50% of prolactinomas recur after surgery.
indications for surgery with a prolactinoma?
Severe visual field defects.
Lack of response/continued growth while on medical therapy.
Intolerance to medical therapy.
What is Cushing's Disease?
OVERSECRETION OF ACTH due to pituitary tumor

May cause diabetes, HTN, obesity (central), hirsuitism, osteoporosis, proximal muscle weakness, etc.
good way to screen for Cushing's Disease? 2
24 hour urinary free cortisol

Dexamethasone suppression test (ppl without cushings are easily suppressible with low levels of dexamethasone, this drug is used because it is not measured in the blood as cortisol, less lab confusion)

REMEMBER: ACTH and cortisol are released in a pulsatile fashion. Random levels rarely helpful. Lack of evening fall in cortisol and ACTH may be.
Tx for Cushing's disease?
remove the ant pituitary adenoma (surgery)

Radiotherapy of surgery is not curative

Medical therapy as necessary to prevent cortisol production.
Ketoconazole, mitotane, metyrapone
Coarse facial features
Skeletal and soft tissue changes
Hypertension
Cardiomegaly
Glucose intolerance/Diabetes Mellitus
Increased risk of various tumors, especially colon.
50% mortality at 25 years compared to 70% in matched population.

are clinical findings of what?
Acromegaly
what is the diagnostic test for acromegaly?
Failure to suppress GH levels during oral glucose tolerance test (OGTT).
GH < 0.4 ng/ml with 2-site IRMA or ICMA.
GH < 1.0 ng/ml using coventional RIA.

Others:
Elevated IGF-1 level (age and gender matched).

MRI to identify size and location of tumor.
>60% are macroadenomas.
Tx options for acromegaly?
Transphenoidal surgery
Octreotide to control growth hormone levels
A few GH-secreting tumors will respond to dopamine agonists (cabergoline, bromocryptine).
Actually monitor IGF-1 levels.
Radiotherapy if above not successful
33 y/o woman presents complaining of tachycardia, irritability, trouble sleeping and dypsnea on exertion. Physical exam is normal except for a modest goiter.
Laboratory evaluation reveals a TSH of 2.2 and a free T-4 of 4.2.
THS is normal, free T4 is elevated

this is hyperthyroid
Pt presents with no problems, but on CBC she has high T4 levels..what is likely going on?
Familial Hyperalbuminemic Hyperthroxinemia
LH-omas cause what problems?
Delayed puberty/hypogonadism
Precocious puberty
If a pituitary tumor or injury to the pituitary is found or suspected it is ESSENTIAL to evaluate the patient for deficiencies of all other anterior pituitary hormones...especially?
ACTH

look for adrenal insufficiency!!
please list the problem associated with the deficiency listed below

Prolactin –
T.S.H –
L.H and F.S.H –
Growth Hormone –
A.C.T.H. –
A.D.H. -
Prolactin – inability to produce breast milk
T.S.H – hypothyroidism
L.H and F.S.H – hypogonadism
Growth Hormone – short stature
A.C.T.H. – adrenal insufficiency
A.D.H. - diabetes insipidus
what hormone is likely to be lost first in pituitary hormone deficiency
GH
What is the gold standard test for confirming GH deficiency?
Insulin-induced hypoglycemia
What is Kallmann's Syndrome?

**
Secondary hypogonadism

genetic mutation is the cause

isolated gonadotropin deficiency

often associated with anosmia (cant smell)

classically affects males
16 y/o male is brought to your office by his father because of lack of pubertal development. The patient is relatively short for his age. Laboratory evaluation reveals Testosterone which is not detectable and LH level which is normal.
Does this represent primary or secondary hypogonadism?
This is secondary. In this case the LH is inappropriately normal. If this were primary hypogonadism, the LH would be elevated.

give testosterone
16 y/o male comes to see you because of delayed puberty. Physical exam reveals very small, firm testicles. Laboratory evaluation reveals a testosterone below detectable limits and an LH of 150.
Is this primary or secondary hypogonadism?
What is the most likely diagnosis?
pituitary is doing its job, but the target gland is not making hormone in response to appropriate stimulus...so PRIMARY

this is Klinefelter's syndrome (XXY genotype)
What is Klinefelter's Syndrome?
Most common genetic cause of primary hypogonadism

Have XXY genotype
______ Syndrome:

Classic form os Secondary Hypogonadism

Isolated GONADOTROPIN deficiency (no progress into puberty)

- Associated with ANOSMIA
Kallmann's Syndrome