DSA 37: Pituitary (MLM)

most common type of pituitary macro adenoma.

Gonadotroph (FSH/LH) adenomas

Everytime we see someone with macroadenomas we need to check their?

Optic fields

C4 Q3: What are the two most common symptoms of acromegaly? Acral enlargement and maxillofacial changes Excessive sweating and arthralgia Headache and visual deficit Fatigue and weight gain

Acral enlargement and maxillofacial changes

ID

As the face changes shape, the jaw may protrude, the nose may enlarge, and the lips may thicken Acromegaly

___ is the only FDA approved Dopamine Agonist During Pregnancy

Bromocriptine

Side effects of bromocriptine

Bromocriptine adherence can be challenging. When initiated, it is associated with orthostasis and lightheadedness, and patients can have dizziness, nausea, and headache during treatment.

ID

Buffalo hump

C4Q4: You checked IGF1, which was very high for her age and gender. How should you confirm growth hormone excess? By checking random GH, which should be > 50 ng/ml By repeating IGF1 By failure to suppress GH to < 1 ng/ml with OGTT By doing MRI of the pituitary gland By failure of GH to increase with Insulin

By failure to suppress GH to < 1 ng/ml with OGTT

Pituitary tumor invading sphenoid sinus, what can you see in PE?

CSF leaking out the nose Have them dribble on a tissue paper and if the first drop forms a clear halo it suggests CSF

Much better tolerated and more effective drug that normalizes prolactin and tumor shrinkage

Cabergoline

Consequences of extensive prolactin and decreased androgens and estrogens On bone

Decreased bone density

Clinical manifestations of prolactinomas in men

Decreased libido, impotence, infertility, gynecomastia or rarely galactorrhea

C1Q4: The patient desires to become pregnant. The medication used to treat is related to what neurotransmitter? pt has prolactinemia Glutamate Somatostatin Dopamine Substance P Threonine

Dopamine

ID

Elevated prolactin In Primary Hypothyroidism, you get elevated TRH. TRH also stimulates the production of Prolactin. Prolactin is inhibited by Dopamine from the Hypothalamus

ID

Enlarged hands as seen in acromegaly

TX of gonadotroph adenomas

FSH/LH pituitary adenomas are typically asymptomatic and are treated similarly to nonfunctioning adenomas because they either do not secrete functional gonadotropins or do not secrete enough FSH or LH to produce a clinical syndrome. Usually they are macro adenomas and are diagnosed because of mass effect or because they are causing central hormonal gonadotropic insufficiency because of the compression of the normal Hypophyseal/gland

Excess ___ can lead to colon cancer

GH

C6Q2: The onset of pan hypopituitarism is often insidious. Which of the following is the usual sequence in which anterior pituitary hormones are lost? GH, LH/FSH, TSH, ACTH FSH/LH, TSH, ACTH, GH FSH/LH, TSH, ACTH, GH ACTH, TSH, LH/FSH,GH

GH, LH/FSH, TSH, ACTH

ID

Gigantism Excess Growth Hormone prior to epiphyseal closure Robert Wadlow - 8 ft 11 in

C4Q2: All of the following are common findings in acromegaly EXCEPT: Osteoporosis Diabetes type 2 Carpal tunnel Glioma of the brain Frontal bossing

Glioma of the brain

Symptoms & Clinical Presentation of Hypercortisolism

Nonspecific symptoms: fatigue, weight gain, hirsutism, proximal muscle weakness, hypertension, hyperglycemia, hypokalemia, loss of bone mineral density Clinical findings that are highly specific: centripetal obesity, facial plethora, abnormal fat deposition in the supraclavicular or dorsocervical ("buffalo hump") areas, wide (>1 cm) violaceous striae

A 45-year-old man presents for evaluation of elevated prolactin level. He is healthy and does not take any medications. He does not have any erectile dysfunction or reduced libido, but he feels depressed and fatigued. The comprehensive metabolic panel, thyroid function tests and complete blood count are normal. His morning testosterone and free testosterone twice were normal. Prolactin is 39 ng/ml (ref = 5-20). He is not under stress, and he is not exercising excessively, neither does he have any weight changes. C3Q1: What is the most appropriate diagnostic test? Obtain pituitary MRI to rule out pituitary tumor Request prolactin level on serial dilutions to rule out hook effect Order a macro prolactin level Check FSH and LH Repeat TSH at 8 am

Obtain pituitary MRI to rule out pituitary tumor

Which test confirms acromegaly?

Oral glucose tolerance test

Differentiate between a prolactinoma and PCOS?

PCOS: Hypertestosterone state. Prolactinoma: interferes with FSH, LH, may end up with estrogen deficiency

Mrs. Wallace is a 48-year-old woman seen for weakness, fatigue and progressive inability to complete ADL's. She is unable to state when the problem actually began, but believes she is definitely worse over the past year. She has secondary amenorrhea. She takes no medications. She has had no major illnesses. She has noticed increased urination and thirst in the last 3 months. She also complains of mild headache and visual impairment that started in the last 3 months. Also she was found having osteoporosis. She does not smoke or drink alcohol and there is no FH of premature osteoporosis. PE BP = 110/58 HR = 54 RR = 16 Ht = 64" Wt = 110# General appearance: Thin, pale, fatigued HEENT: Sclera clear, nares patent, fine wrinkles face skin Skin: Pale, with wrinkles There is a loss of the pubic hair Labs: low FSH/LH and low estradiol normal TSH with Low FT4 morning cortisol of 2.8 mcg/dL (ref = 7-28 mcg/dL) and low normal ACTH. Her ACTH stimulating test led to increased cortisol in 60 minutes (2.8 mcg/dl to 8 mcg/dl). IGF1 was low for her age and gender. (Growth hormone response to stimulation has yet to be tested) C6 Q1- What is the most likely diagnosis? Primary hypogonadism Primary hypothyroidism Secondary hypothyroidism Primary hypocortisolism Pan hypopituitarism

Pan hypopituitarism

Response to therapy (for prolactinomas) can be monitored by

checking serum prolactin levels 1 month after initiating therapy and then every 3 to 4 months

Pituitary adenomas that are clinically nonfunctioning or "silent"- 80 to 90 percent of these are

gonadotrophic adenomas

In Gonadotroph adenomas, the most common clinical hormone deficiency is

impaired secretion of gonadotropins resulting in secondary hypogonadism with infertility and sexual dysfunction

DA therapy should be discontinued when the

pregnancy is diagnosed

Physiologic causes of prolactinemia

prolactin is released during pregnancy and postpartum to cause lactation. Nipple stimulation such as during sex can cause mild hyperprolactinemia (serum prolactin <40 ng/mL [40 μg/L]). Nipple piercing can transiently elevate prolactin to high levels. Physiologic stress, coitus, and exercise can also increase prolactin levels.

LH and FSH commonly present

without any symptoms

A 32-year-old female has had menstrual problems for one year, with oligo-amenorrhea, mild hirsutism, and weight gain of 5 pounds. She has less energy than in the past and feels cold. She takes no medications. Her blood pressure and heart rate are normal. She has mild central obesity and she does not have galactorrhea. On physical exam the skin is dry and cold, and she has delayed relaxation phase on DTR's. Her prolactin level is 53 ng/ml (ref = 5-20). MRI of the pituitary is negative, and a pregnancy test is negative The most appropriate test to find out what can explain the patient's symptoms and laboratory findings is Ovarian USG Serum FSH Serum LH Serum testosterone Serum TSH

Serum TSH

Hypopituitarism can occur due to postpartum pituitary infarction because of excessive postpartum uterine hemorrhage causing hypotension and hypo-perfusion to the pituitary. will not lactate because of prolactin deficiency. Patients can present immediately or several years after childbirth.

Sheehan syndrome

Pituitary adenomas that are hypersecreting other than prolactinomas are treated with

Sphenoidal surgery

How is pituitary adenoma different in terms of treatment to other secreting adenomas. Gold treatment for secreting tumors?

Surgery Prolactinomas have to be treated medically first

Prolactinomas are treated with dopamine agonists (DA) -

bromocriptine and cabergoline. Dopamine agonists typically decrease the size and hormone production of prolactinomas rapidly.

ID

Acromegaly

First test that you order if you suspect acromegaly

IGF-1

After Diagnosis of Acromegaly needs to be done:

1. Colonoscopy needs to be done to rule out polyps of the colon or other neoplasia of the colon 2. Thyroid gland assessment - associated with cancer of the thyroid 3. Check lipid panel, Glucose tolerance 4. Check Prolactin level - up to 50% of patients with Acromegaly are co secreting Prolactin! 5. Rule out hypopituitarism- check 8 a.m. ACTH, cortisol, TSH, FT4, FSH, LH, 8 a.m. testosterone, estradiol- the GH secreting tumors are usually macro adenomas , because the diagnosis is usually made late. 6. Visual field evaluation if any change in the vision.

SUMMARY OF THE TREATMENT OPTIONS FOR ACROMEGALY

1. First line- Trans sphenoidal surgery of the GH secreting Adenoma or transcranial resection if the tumor is big. Usually macro adenoma. If residual tumor - reoperation if possible! 2. Second line - if 1 can not control the disease- Somatostatin analogues- Octreotide, lanreotide , Pasireotide. Side effects- GI with abdominal pain, diarrhea, increased BS- decreases the secretion of Insulin especially with Pasireotide and Gall bladder stones 3.Dopamine agonists especially if co secretion of PRL. Can control less efficiently then 1,2 usually small tumors- micro adenomas 4. Pegvisomant- GH receptor blocker (peripheral). Monitor for growth of the GH secreting adenoma. Usually happens in 3-5% of the cases. Controls IGF1 in 85% of the cases. Side effect is LFT elevation. Usually very effective is Somatotropin analogue plus Pegvisoman- controls the disease and keeps IGF1 in normal ranges in more then 95% of the patients. 5. Stereotactic radiosurgery- in difficult to treat disease

INCREASED MORTALITY IN PATIENTS WITH ACROMEGALY

1. Increased risk of Cardiovascular diseases which are the major number one risk for morbidity and mortality in patients with acromegaly but also increased risk of Diabetes mellitus, sleep apnea and cancers- esophageal, gastric, colonic adenocarcinomas, thyroid cancer, melanoma. 2. Early detection is the key- in every patient with pituitary adenoma the screening test using IGF-1 should be done. Frequently if unsure need to be checked old photographs of the patient for comparison. Rare disease with very serious complications!

Diagnosis of Cushing Disease

1st line biochemical screening for hyper cortisolism based on high clinical suspicion: 24 hour urine free cortisol. Needs to be repeated if increased and if 2 values are increased the test is positive Late night (LN) salivary cortisol also needs to be repeated if increased, and if 2 values are increased the test is positive Low dose dexamethasone suppression test (LDST): In the standard LDST, dexamethasone is administered every 6 hours for 48 hours and serum cortisol is measured at 9am on the 3rd day. In the overnight LDST, dexamethasone is administered late night, and serum cortisol is measured the next morning. With either test, serum cortisol will typically be suppressed to less than 1.8 μg/dL (55 nmol/L) in normal people. In contrast to the two tests above, the LDST does not need to be repeated. One abnormal suppression is considered abnormal In hypercortisolism (Cushing's Syndrome) - at least 2 of the first line tests should be abnormal Then check ACTH - If ACTH is (low ACTH levels on 2 separate occasions ) -----> screen for adrenal adenomas/carcinomas (CT /MRI adrenals)

Incidentally Noted Pituitary Masses When a pituitary tumor is incidentally noted, investigation must determine whether it is causing a mass effect whether it is secreting excess hormones whether it has a propensity to grow and cause problems in the future After a thorough history and physical examination, biochemical testing can be undertaken in a targeted fashion based on the patient's clinical signs and symptoms. Initial tests could include measurement of

8 AM cortisol TSH, free (or total) thyroxine (T4) Prolactin Insulin-like growth factor 1 (IGF-1) -Most frequently secreted in excess - the Prolactinomas and GH secreting adenomas are the most commonly co-occuring

After you order a IGF-1 test and after you do an oral glucose tolerance exam on someone who you suspect has acromegaly, what should be done?

A pitutary MRI should be obtained once GH excess is confirmed biochemically.

Ms. Mendel is a 45-year-old woman seen because her family has noticed changes in her appearance. She has no complaints. Physical examination reveals... Ms. Mendel is a 45-year-old woman seen because her family has noticed changes in her appearance. She has no complaints. Physical examination is significant for coarse facial features and big hands and feet. She has not noticed the changes but does relate that her ring does not fit her fingers anymore. She also needed a larger hat for the winter months. Also she complains of excessive sweating and headache, and has recent onset of Type 2 diabetes mellitus and HTN. C4Q1: What disease will most likely be diagnosed in Ms. Mendel? Gigantism Acromegaly Paget's Acromegaloidism Cushings

Acromegaly

DX of Growth Hormone Excess

Acromegaly is diagnosed biochemically. Because GH is pulsatile throughout the day, it is not useful for diagnosis Screening for Acromegaly is, instead, done by measurement of serum IGF-1 is used instead Has longer half life. If IGF-1 is above normal for the age and gender, do a Glucose Suppression test for GH to confirm Normal: Glucose suppresses GH levels ≤1 ng/mL (1 μg/L) or bellow 0.3 ng/ml on ultrasensitive assay. GH Excess: GH levels ≥1 ng/mL (1 μg/L) or equal or greater then 0.3 ng/ml on ultrasensitive assay A pituitary MRI should be obtained once GH excess is confirmed biochemically. Consultation with an endocrinologist is recommended if IGF-1 is elevated. Also ruling hypopituitarism should be done when macro adenoma is confirmed!

Diagnosis & Treatment of Hypopituitarism

After the clinical and biochemical diagnosis of hypopituitarism has been established, a radiographic study id indicated to determine whether a mass or other abnormality is present. The most informative imaging study of the pituitary gland is a gadolinium enhanced MRI. Patients with Panhypopituitarisim require lifelong replacement thyroxine(T4), cortisol, and antidiuretic hormone because these deficiencies are life threatening. Replacement of other hormones (growth hormone, estrogen, testosterone) is variable. If fertility is desired, Gonadotropin Hormonal replacement is needed

Explain the high dose dexamethasone suppression test

After we determine that there is something abnormal via the LDST, we can identify the source via a high dose suppression test Give patient 8mg dexamethasone If tumor is in pituitary: Cushings disease, pituitary adenoma is producing ACTH. Giving enough dexamethasone will suppress cortisol production. Normal or slightly decreased ACTH, cortisol is supressed If tumor is in adrenals: Decreased ACTH Cortisol will be high If tumor is ectopic: Increased ACTH Increased cortisol

Why is increased testosterone seen in some cases of gonadotroph adenomas?

Although gonadotroph adenomas are thought to occur most commonly in middle-aged men, clinical findings due to hypersecretion of intact LH or FSH are rare in this group. However, elevated serum testosterone concentration due to hypersecretion of intact LH and testicular enlargement due to FSH hypersecretion have been described.

Describe silent gonadotrophic adenomas

Although gonadotrophic adenomas are considered to be "nonfunctioning", most do produce intact gonadotropins or their subunits. However, these adenomas are typically poorly differentiated and inefficient producers/secretors and do not raise serum gonadotropin concentrations. Thus, they are usually clinically "silent" and cannot be distinguished from other clinically nonfunctioning adenomas until immunohistochemistry is performed after pituitary surgery. The majority of gonadotroph adenomas that secrete intact gonadotropins occur in middle aged adults and do not result in a clinical syndrome. In postmenopausal women, for example, a gonadotroph adenoma that secretes intact gonadotropins would not result in a clinical syndrome, because gonadotropin levels are already high, and a postmenopausal ovary cannot be stimulated to produce follicles or estrogen.

What is Pituitary Apoplexy?

An endocrine and neurosurgical emergency An acute hemorrhage into the pituitary gland often at the site of a preexisting pituitary adenoma (typically a macroadenoma). Can cause acute pituitary hormone deficiency and mass effect Present with headache, vomiting, opthalmoplegia, visual field loss, hypotension, meningismus, fever and decreased consciousness. Evaluation should include an MRI scan and neurological assessment with formal visual field testing. Patients with vision changes or loss associated with apoplexy require urgent surgical decompression. Acute ACTH deficiency is common and can be life-threatening. If suspected, high dose (stress-dose) glucocorticoid replacement should be initiated emergently.

A 34-year-old man is evaluated for a 2-year history of decreased libido and severe erectile dysfunction, including absence of morning erections. He and his female partner would like to conceive. His medical history is otherwise unremarkable, and he takes no medications. He has headaches and visual problems. Vital signs are normal. BMI is 27. Atrophic testes are noted bilaterally. MRI of the pituitary reveals a 3.5 cm anterior pituitary mass consistent with a macro-adenoma with extension to the optic chiasm and cavernous sinus on the left. His FSH and LH are low, and morning testosterone levels on two separate occasions are low. TSH is normal. PRL level is 1,000 ng/dl. C2Q1: Which of the following is the most appropriate treatment for this patient? Cabergoline and obtain eye specialist consultation Sildenafil Testosterone replacement therapy Clomiphene citrate Trans-sphenoidal surgery

Cabergoline and obtain eye specialist consultation Pt has prolactinoma

C4 Q7-Which is the number one cause of morbidity and mortality in patients with Acromegaly Cancer of the colon Cancer of the thyroid Diabetes-mellitus-related microvascular complications Obstructive sleep apnea Cardiovascular (macrovascular) complications

Cardiovascular (macrovascular) complications Increased lipids, impaired glucose tolerance, heart disease

What other test needs to be done on this patient? Colonoscopy to rule out colonic neoplasia MRI of the spine to rule out osteosarcoma CT of the kidneys to rule out kidney neoplasia Serum free metanephrines (because the patient has HTN to rule out MEN syndrome) X-ray of the Hands

Colonoscopy to rule out colonic neoplasia

Ms. Volta is a 22-year-old woman presenting with diabetes mellitus and hypertension. She does not have depression, alcoholism and is not under stress, does not have anorexia nervosa. She is not a smoker. Physical examination is reveals.... C7Q1: What is the suspected diagnosis? Acromegaly Hypothyroidism Cushing Syndrome Polycystic Ovarian Syndrome Addison's Disease

Cushing Syndrome

A 45-year-old man presents for evaluation of elevated prolactin level. He is healthy and does not take any medications. He does not have any erectile dysfunction or reduced libido, but he feels depressed and fatigued. The comprehensive metabolic panel, thyroid function tests and complete blood count are normal. His morning testosterone and free testosterone twice were normal. Prolactin is 39 ng/ml (ref = 5-20). He is not under stress, and he is not exercising excessively, neither does he have any weight changes. C3Q2: What is the condition we are suspecting? Prolactinoma Thyrotropinoma Gonadotroph Adenoma Craniopharyngioma Growth Hormone Adenoma

Gonadotroph Adenoma Remember that GH tumors can also co-secrete prolactin Prolactinomas would have a much increased value of PRL than the one reported in this case.

Failure of GH to increase insulin

Growth hormone deficiency We give patient insulin to determine if there is a growth hormone deficiency. If GH does not increase in response to insulin then there is growth hormone deficiency.

C6Q4: Which of the following hormones MUST be given for survival if this patient has pan hypopituitarism? Estrogen Growth hormone Hydrocortisone, Levothyroxine and Desmopressin if Central DI Dihydroepiandosterone LH and FSH

Hydrocortisone, Levothyroxine and Desmopressin if Central DI

centripetal obesity, facial plethora, abnormal fat deposition in the supraclavicular or dorsocervical ("buffalo hump") areas, wide (>1 cm) violaceous striae

Hypercortisolism

Prolactinomas and Pregnancy

Hyperprolactinemia is a frequent cause of infertility because of the effect on gonadotropin release. DA therapy lowers prolactin, normalizing gonadotropin regulation and allowing normal ovulation. DA therapy should be discontinued when the pregnancy is diagnosed. The pituitary increases in size during normal pregnancy, and prolactinomas can increase in size as well. The risk for significant tumor expansion is negligible in patients with micro prolactinomas. Women with macro prolactinomas are at risk for clinically significant tumor growth or vision compromise during pregnancy. If the tumor is very large or abuts the optic chiasm, patients should be counseled on risk of tumor growth during pregnancy, as well as the risks and benefits of surgical resection of the tumor before pregnancy. DA therapy is sometimes continued during pregnancy if the patient has a history of visual field defect.

Describe Sheehan syndrome

Hypopituitarism can occur due to postpartum pituitary infarction (Sheehan syndrome) because of excessive postpartum uterine hemorrhage causing hypotension and hypo-perfusion to the pituitary. A patient with Sheehan syndrome will not lactate because of prolactin deficiency. Patients can present immediately or several years after childbirth. Patients who may have Sheehan syndrome should be emergently tested and treated for secondary cortisol deficiency. Other hormone deficiencies can be evaluated 6 weeks after delivery.

Hyperprolactinemia

Hypothyroidism can cause diffuse swelling of the pituitary gland that on imaging may resemble enlargement due to a pituitary adenoma. Therefore, a patient with primary hypothyroidism and hyperprolactinemia should be treated with thyroid hormone replacement with retesting of the prolactin level once the TSH has normalized. Further evaluation is indicated if the hyperprolactinemia does not correct when hypothyroidism is treated. If pituitary imaging has noted pituitary enlargement prior to treatment of hypothyroidism, repeat MRI should be obtained when the TSH is normal.

In GH tumors: Remember that morbidity and mortality is associated with the level of

IGF1 after treatment - needs to be for the age and gender and GH needs to be less then 1 ng/ml after OGTT- Oral Glucose tolerance test

C1Q7: If the pituitary tumor is not causing mass effect and there is no evidence of hormone excess in incidentally found pituitary tumor when should we repeat the pituitary MRI to assess for growth? In 12 months for the macro adenomas In 18 months for micro adenomas In 18 months for macro adenomas In 12 months for micro adenomas None Required

In 12 months for micro adenomas 6 months for macroadenomas

Screening for ACTH secreting Pituitary tumors is done only in appropriate clinical settings!

Incidentally Noted Pituitary Masses If the tumor is not causing mass effect and there is no evidence of hormone excess, a pituitary MRI should be repeated in 6 months for a macro adenoma and 12 months for a micro adenoma to assess for growth. If no growth occurs, MRIs should be repeated every 1 to 2 years for the next 3 years and then intermittently thereafter. In a patient at risk for cancer or with a history of cancer, metastatic disease must be excluded. Patients with macro adenomas need to be checked upon diagnosis for visual field defect and then in 6- months for hormonal suppression of the remaining pituitary gland, visual fields and MRI of the HP gland.

Clinical manifestations of prolactinomas in women

Infertility, oligomenorrhea, amenorrhea or rarely galactorrhea

ID

Known formally as Maurice Tillet, a Russian-born Frenchman, the man rose to prominence as a wrestling star in the 1930s and '40s. Born in 1903, Tillet is said to have been called "angel" by his boyhood friends because of his blonde hair and angelic face. At the age of 17, however, doctors diagnosed Tillet with acromegaly.

C7Q2: Which are 1st line tests for Cushing's Syndrome 24 hour Urine Free Cortisol & High Dose Dexamethasone Suppression Test Low & High Dose Dexamethasone Suppression Test Late night Salivary Cortisol & CRH Stimulation Test Low Dose Dexamethasone Suppression Test and Late Night Salivary Cortisol 24 hour Urine Free Cortisol & CRH Stimulation Test

Low Dose Dexamethasone Suppression Test and Late Night Salivary Cortisol

A 45-year-old man present with complaints of headache and vision changes. Physical examination is unremarkable. An MRI of the brain is abnormal and an MRI of the pituitary shows a 2 cm mass. Is this a Microadenoma Macroademona

Macroademona Macroadenomas are greater than 1 cm

Prolactinomas typically present in men as

Macroadenomas Since men don't breast feed, these tend to present later

Why is elevated PRL in some cases of gonadotroph adenomas?

Macroadenomas often compress the pituitary stalk and obstruct the normal inhibitory hypothalamic influence on the prolactin producing cells, resulting in modestly elevated serum prolactin concentrations (usually <100 ng/mL but sometimes as high as 200 ng/mL).

Mass effects due to a prolactinoma are more often seen in

Men

Prolactinomas typically present in women as

Microadenomas

ID

Moon facies Plethora

Clinical Presentation & diagnosis of Gonadotroph adenomas

Most often diagnosed when mass effects are present The three most common presentations include the following : Neurologic symptoms, most commonly visual symptoms; less commonly headache. A pituitary mass that is discovered as an incidental finding when an imaging procedure is done for reasons other than pituitary symptoms or disease. Pituitary hypofunction due to compression of normal pituitary tissue by the adenoma. The most common clinical hormone deficiency is impaired secretion of gonadotropins resulting in secondary hypogonadism with infertility and sexual dysfunction Patients suspected of having these adenomas should undergo pituitary MRI as well as testing of pituitary function

Gonadotroph adenomas: The three most common presentations include the following :

Neurologic symptoms, most commonly visual symptoms; less commonly headache. A pituitary mass that is discovered as an incidental finding when an imaging procedure is done for reasons other than pituitary symptoms or disease. Pituitary hypofunction due to compression of normal pituitary tissue by the adenoma.

C1Q5: What is the most common cause of hyperprolactinemia? Physiologic causes Prolactinoma Growth-hormone-secreting tumors TSH-secreting tumors Dopamine antagonist medications

Physiologic causes The most common cause of hyperprolactinemia is physiologic; prolactin is released during pregnancy and postpartum to cause lactation. Nipple stimulation such as during sex can cause mild hyperprolactinemia (serum prolactin <40 ng/mL [40 μg/L]). Nipple piercing can transiently elevate prolactin to high levels. Physiologic stress, coitus, and exercise can also increase prolactin levels. Clinical breast examination should not raise prolactin levels above the reference range, unless evaluation for milk production is performed, but if desired, palpation of the breast can be deferred until after a serum prolactin level is measured.

Given the slow development of symptoms of pan hypopituitarism in this patient, which of the following diseases is most likely causing this condition in Mrs. Wallace Injury Infarction Pituitary adenoma Infection

Pituitary adenoma

Do a high dose supression test: Tumor is most likely in this location: ACTH: Low Cortisol: Low

Pituitary adenoma Cushings disease

What is the most common tumor identified on MRI of the Pituitary?

Pituitary adenomas are the most common pituitary lesion with a prevalence of approximately 10% -38% of the patients who underwent MRI of the brain. Most common are the non functioning incidentally found adenomas. From the ones which secrete excess of hormones the most common are: Prolactinomas most common (60%) GH secreting adenomas (15%) ACTH secreting (6%) LH, FSH or TSH are very rare

Gonadotroph (FSH/LH) adenomas

Pituitary adenomas that are clinically nonfunctioning or "silent"- 80 to 90 percent of these are gonadotrophic adenomas, making them the most common type of pituitary macro adenoma. FSH and LH are complex hormones with 2 units that must be glycosylated and then combined in appropriate ratio - which is not a very efficient process in making biologically active hormones by this adenoma; this is why they are usually clinically non-functional, but they stain positive for these hormones or their subunits in immunostaining

an cause acute pituitary hormone deficiency and mass effect Present with headache, vomiting, opthalmoplegia, visual field loss, hypotension, meningismus, fever and decreased consciousness.

Pituitary apoplexy

In primary hypothyroidism you get elevated TRH. TRH stimulates the production of TSH and _____

Prolactin

Some GH producing tumors also co-secrete

Prolactin

C1Q6: Which hormones need to be checked in Incidentally noted Pituitary micro-adenomas Prolactin, IGF1, Morning cortisol, TSH and FT4 LH and FSH Testosterone ACTH, 24-hour urine for cortisol, and low dose dexamethasone suppression test in every patient with pituitary microadenoma Dopamine, TRH, Cosyntropin

Prolactin, IGF1, Morning cortisol, TSH and FT4

C1Q2: What is the most common functioning pituitary tumor? Prolactinoma Thyrotropinoma Craniopharyngioma ACTH producing adenoma GH producing tumor

Prolactinoma

ID

Purple striae

You have confirmed acromegaly. The pituitary MRI confirmed pituitary macro adenoma invading the sphenoid sinus. Her other pituitary hormones were normal and prolactin was normal. The patient does not have dyslipidemia or thyroid problems, and there are no visual field defects. How should you treat this patient? Initiate growth hormone receptor antagonist Start dopamine receptor antagonist to decrease both hormonal production and size of the tumor Start the somatostatin analogue to decrease both hormonal production and size of the tumor Radiation therapy Refer for trans sphenoidal resection of the tumor

Refer for trans sphenoidal resection of the tumor

C2Q2: All of following are indications for surgery of a pituitary prolactinoma EXCEPT The patient is unable to tolerate medication There is progression of the tumor with medication The patient doesn't respond to medication The patient is female, with a microadenoma and desires pregnancy The patient has a macroadenoma (>1cm)

The patient has a macroadenoma (>1cm) Prolactinoma gets medical therapy initially. Doesnt matter if it is macro or micro. We go to surgery if patient who do not respond to treatment or those who show progression after an initial response to medical treatment. We also do surgery in women who have a microadenoma, desire pregnancy and cannot tolerate medical treatment.

ID

Transphenoidal surgery is tx of choice for acromegaly in most patients. When surgery fails, medical therapy should be initiated (octreotide or lanreotide). These drugs suppress pituitary GH secretion adn block the synthesis of IGF-1 When surgery and somatostatin analogues fail, use pegvisomant, a GH receptor antagonist Drug acts peripherally, but does not affect the pituitary secretion of GH, it does not lower GH or reduce tumor size. Radiotherapy slows tumor growth but its effects are delayed and hypopituitarism is a common side effect.

Clinical Presentation of Hypopituitarism

When symptoms are acute in onset, an event such as pituitary apoplexy should be considered. GH and gonadotropin deficiencies often occur early when the pituitary gland is damaged by tumor, radiation, surgery, or hemorrhage because the cell lines that synthesize GH (somatotrophs) and LH and FSH (gonadotrophs) are most sensitive to injury. Secondary hypothyroidism (TSH deficiency) and secondary cortisol deficiency (ACTH deficiency) often occur later in the disease process. GH & LH/FSH ---> TSH & ACTH

C1Q3: How does a prolactinoma present in women? Elevated Diastolic Blood Pressure, Moon Facies, Buffalo Hump With oligo-menorrhea, galactorrhea, +/- hirsutism, infertility, osteoporosis Wt. loss, cervical fat pad, infertility Oligo-menorrhea, FSH/LH excess, hirsutism Pigmented skin, striae, cervical fat pad

With oligo-menorrhea, galactorrhea, +/- hirsutism, infertility, osteoporosis

Explain the Low dose dexamethasone suppression test (LDST)

You give dexamethasone to a patient the night before. Check the levels the next morning. If Cortisol levels are reduced then this is a normal response. If cortisol levels are not reduced then we have a problem. One abnormal suppression is considered abnormal