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162 Cards in this Set
- Front
- Back
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what is suggested by tumors seen in pituitary,pancreas, parathyroid?
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MEN1
multiple endocrine neoplams type 1 |
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MEN 1 caused by what gene
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menin
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MEN 2 caused by what gene
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RET protooncogene
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what is seen in almost always in MEN2
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meduallay thryoid carcinoma
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how can you differentiate btw MEN 2a and 2b?
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2b has neuro ganglioma and other nerve neoplasia
2a doesnt |
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what is a recomendation in reguards to the thyroid in pt with MEN2?
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prophylatic thyroidectomy
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what is the prognosis of tumors found in MEN2?
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worse than people with normal versions of those same tumors
tumors are multifocal appear at a younger age. |
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what predisposes people to papillary thyroid carcinoma?
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radiation
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where is pituitary located?
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beneath sella turcica very close to optic nerves
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what are the hormones released by the pituitary?
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prolactin
ACTH TSH GH LH/FSH ADH Oxytocin |
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what is the difference in blood supply to the anterior and posterior pituitary?
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anterior has single blood supply via portal system
posterior has dual supply both artiers/viens and the portal system. |
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which part of the pituitary is most vulnerable to ischemia?
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the anterior
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what are the functions of the anterior and post. pit.
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ant.-adenohypophysis glandular portion
releases hormones. post-neurohypophysis all modified glial cells and axonal processes. releases neurtransmitters as hormones |
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what are the cells of the anterior pituitary?
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somatotrophs
lactotrophs corticotrophs thrytorhops gonaodotrohps |
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what does somatotrophs produce?
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growth hormone
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what does lactotrophs produces?
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prolactin
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what does corticotrophs produce?
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adrenocotiocotrophic homrone(ACTH_
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what does thrytophs produce?
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TSH
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what do gnoadotrophs produce?
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FSH and LH
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how does immunohistochem work?
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if you use a stain for something the cells containing that substance will light up on the slide. this can be used to visualize the different cell populations in the ant. pit.
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what is produced in the neurohypophysis?
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ADH (vasopressin)
Oxytocin |
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what is the usual cause of hyperpituitarism?
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excess sectrtion usually by an adenoma
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what is the cause of hypopituitarism?
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hormone defcit usually bc of destructive proccess like ischemia, surgery, radiation, inflammation
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what are the mass effects of a pituitary tumor?
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visual feild deficit(bilarteral temporal anopsia)
elevated intracranial pressure pituitary apoplexy(massive hemmorhage at the gland) |
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what are the two catagories of adenomas of the pituitary?
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functional
nonfunctional |
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what is typical about functional adenomas of the pituitary?
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they produce only one hormone. usually but can produce more
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what is typical about the nonfunctional pituitary adenomas?
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they do not produce hormone/hormones dont get released/homrones produced are not functional
can destroy surrrounding tissue leading to hypopituitarism. |
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what is a genetic compentent with pituitary adenoma of somatotroph cells
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mutation of gene encoding the alpha subunit of the Gs
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what is the cut off for microadenoma and macroadenoma?
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microadenoma-less than 1cm
macroadenoma ->than 1cm |
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what is the typical appearance of pituitary adenoma on histo?
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a homogenous appearance of one cell type
atypial, hemorrhage, and necrosis may be present but dont imply malignancy decreased reticulin network(black outlines) now only seen around vessels normal pituitary histo is very diverse with different colors and glands this is not its all homogenous |
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what type of adenoma usually gets big?
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nonfunctioning
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what is another cause of hyperprolactin other than an adenoma?
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stalkeffect/lactotroph hyperplasia,
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what is lactotroph hyperlasia
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normally lactorphs want to produce tons of prolactin but that action is inhibited by dopamine released from hypothalmus if that is inhibited then lactotrophs will produce in excess
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what is stalk effect
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lactroph hyperplasion caused by tumor in the suprasellar compartment that compresses the stalk and prevent dopamine from getting down to pituitary.
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what are some properties of the prolactinomas?
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most are macroadenomas
can be found with imunostain for PrL serum PrL tend to correlate with size of adenoma |
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what is the most common functioning pituitary tumor?
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prolactinomas 30% of all adenomas
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how is prolactinomas present clinically?
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amenorrhea, galactorrhea, loss of libido, infertility,
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what is the treatement for prolactinomas?
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surgury and bromocripitine(dopamine style drugs that inhibit lactotrohps from secretion)
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what is the second most comon functioning adenoma?
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somatotroph cell growth hormone adenoma
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what is the genetic cause of somatotroph adenoma?
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40% with mutant GTPase defeicient alpha subunit of G protien Gs
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what is seen clinically in somatotroph cell adenomas?
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children before epphyseal closure-gigantism increased total body size
adults after epiphyseal closure-acromegaly |
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what is the oral glucose challenge test in GH adenoma?
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failure to suppress GH production with oral glucose is a very sensitive test for acromegaly
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what is corticotroph cell adenomas also called?
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cushing disease
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what is the difference btw cushing disease and cushing syndrome?
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cushing syndrome is any time a pt has high levels of cortisol
cushing disease is when that syndrome is caused by adenoma in the pituitary. |
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what is nelsons syndrome?
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removal of the adrenals= loss of inhibitons of cortisol on ACTH= development of large adenomas= mass effects and hyperpigmentatino of skin.
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why does hyper ACTH lead to hyperpigmentation?
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ACTH is complexed to form preopiomelanocorticotropin(POCM) has a stimulatory effect on melanocytes
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what are signs of gonatroph adenoma?
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not usually detected till the start causing neuro symptoms by mass effect
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what are the two types of nonfunctioning adenomas?
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null cell- generate no detectable hormonal product
nonsecretory variant= silent adenoma more common of the two hormone is being produced but no secreted |
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what is likelyhood of pituitary carcinoma?
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very very rare
must have demonstation of metastases to call it carcinoma bc necrosis atypia and all that occur in the adenoma |
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how much of the gland must be destroyed for there to be hypopituitarism?
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75% must be gone before hypofunction occurs
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what is normal presentation of pituitary apoplexy?
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sudden rapid enlargment of tumor which causes really bad headache, diplopia, hypopituitaritarim, and can even have sudden death
sudden enlargment caused by hemorrhage into the pituitary usually the result of damage from an adenoma |
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what is the main cause of ischemic necrosis to the pituitary?
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Sheehan syndrome
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what is sheehan syndrome?
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postpartum necrosis of the anterior pituitary secondary to sudden infarction b/c of obstetic hemoorhage/shock
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why is sheehan syndrome specific to pregnancy as opposed to other cuases of shock?
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bc in pregnancy ant. pituitary is hyperplastic due to gearing up for elevated secretion of prolactin, but there is not increased blood flow to support this increased tissue. It is common to lose a lot of blood during delivery so the pituitary is the most vulnerable thing during this time it becomes ischemic and then necrotic.
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what is empty sella syndrome?
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enlarged, empty sell turcica lacking pituitary tissue
primary- anatomic defect in which CSF is able to leak into pituiatary space and compress it leading to hypopituitarism secondary- surgury done that removes tumor and results in hole that allows CSF in. |
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where do the cells of the post. pit. orginate from?
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supraoptic and paraventricular nuclei of the hypothalmus
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what is the effect of oxytocin?
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stims contraction of uterine smooth muscle in gravid uterus and cells of lactiferous ducts in mammary glands
no clinically recognized diseases that are affected by this. |
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what are effects of ADH deficiency?
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poluria
polydispsia hypernatremia |
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what can cause ADH deficiency?
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spontaneous
result of head trauma tumors, inflammatory disorders or surgical procedures of hypothalmus/pituitary |
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what are the two types of Diabetes insipidus?
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lack of ADH secondary to pituitary is central DI
when kidney doesn't respond to ADH its nephrogenic DI |
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what is SIADH?
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syndrome of inappropriate ADH secretions
causes increased free water resportion. |
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what are effects of SIADH?
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hyponatremia
cerebral edema neurologic dysfunction |
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what is often a cause of SIADH?
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paraneoplastic syndrome with tumor that secretes ectopic ADH.
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what tumor is most often associated with SAIDH? from paraneoplastic syndrome
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small cell carcinoma of the lung
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what are nonneoplastic causes of SIADH?
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lung disease, local injury to hypothalmus/pituitary.
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what is the pituitary effect of hypothalamic suprasellar tumors?
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can cause hypo or hyperfunction of pituitary
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what are the two hypothalamic suprasellar tumors?
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glioma and craniopharyngioma
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what are the characteristics of the craniopharngioma?
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rathke's pouch
slow growing |
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who does craniopharyngioma affect most often and how does it present in them?
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duel peak incidicne in children and adults in their 60s
children present with endorcrine deficiency epseacially growth retardation adults present with visual disturbance due to mass effect |
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what are histologic features of craniopharyngiomas?
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cycstic tumor with calcifications
mixture of squamous and stroma MACHINE OIL appeareance of wet keratin |
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what is normal histo for parathyroids?
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composed of mainly cheif cells which are basaloid neuclei with pink cytoplasm
and stromal fat that dots all over it also there are oxyphil cells with are pink puffy cells the gland is small and surround by thyroid tissue |
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what is the hormone released form the paraythyroid?
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PTH
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what is PTH released in response to?
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low serum Ca
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what is the action of PTH?
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raises CA by:
causing calcium reabsorption by renal tubules converting vit. D to active form in kidney increaseing phosphate excretino in kidney augmenting GI absoptino of calcium activatino osteoclasets to release bone calcium. |
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what are the causes of hypercalcemia?
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parathyroid adenoma
renal disease(causes secondary hyperparathyroidism) bone destroying malignancyies(multiple myeloma, leukemia) maltignancies that produce PTH like hormone sarcoidosis paget disease hypervitaminosis D |
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what is the PTH like hormone and what does it do?
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PTHrP affects RANKL/ostoprotegrins axis shifting bone homeostatis toward osteoclastogenesis
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what are the most common sites of neoplasms secreteing PTHrP?
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small cell carcinoma of lung
breast or kidney |
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what is the most common cause of hypercalcemia that is clinically appareent(ie shows symptoms)
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malignancy
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what is the most common cause of hypercalcemia that is picked up on routine labs and does not show other symptoms?
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parathyroid adenoma.
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what are the parathyroid pathologies?
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adenoma-produces PTH
carcinoma primary hyperplasia-seen in MEN secondary hyperplasia agenesis- accidental-from surgery autoimmune |
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what is the cause of secondary hyperplasia?
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gland stimulated to produce PTH by low serum calcium usually caused by renal failure
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what conditions often involves agenesis of the parathyroids
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diGeorge syndrome
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what is the most common cause of primary hyperparathyroidism?
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adenomas
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what is the genetics associated with parathyroid adenomas?
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cyclinD1 and MEN1
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what are the familial conditions associated with primary hyperparathyroidism?
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MEN syndromes
familial hypocalciuric hypercalcemia |
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what is familal hypocalciuric hypercalcemia?
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parathyroid gland has decreased sensitivity to extracellular calcium bc of mutation in the calcium sensing receptors(CASRs)
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what are the features of the parathyroid adenoma?
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almost always solitary involving only one gland remaining glands are shrunken
compsed of sheets, trabeculae or follicucles of cheif cells STOMAL FAT DECREASED-halmark feature of adenomas and primary hyperplasia |
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what are features of primary hyperplasia?
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involves all glands
cheif cells are hyperpalstic in a diffuse or multinodular pattern stromal fat decreased |
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what are the features of carcinoma of parathyroid gland?
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can cause hyperparathyroidism
only one gland affected diagnosis based on local invasion/metasteses or both. |
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what are the systemic effects of hyperparathyroidism?
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skeletals-bone resorption
osteitis fibrosa cystica- marrow increased fibrosis, hemorrhage and cycst formation brown tumors- aggregates of osteoclasts, reacivte gaint cells and hemoorhagic debris, somtimes mistaken for malignancy Renal effects: nephrolithiasis nephrocacinosis-cacification of interstiuti and tubueles OTHER metastatic calcifications of other tisue |
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what are the two ways in which hyperparathyroidism can present?
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asymptomatic-
presents for routine test shows serum Ca elevation, additional tests show elevated PTH in the presence of high serum calcium levels symptomatic- constellatio of symptoms: bones, stones, moans, and groans. 1. bone pain, fractures secondary to osteoporosis 2.renal nephrolithiasis 3. GI ulcers-increased gastrin bc of hypercalcemai, pacreatitis, gallstones 4. CNS depression, lethargy, eventual seizures. |
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what is the moa of secondary hyperparathyroidism?
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chronic renal disease with phosphate retention depresses serum caclium levles
all 4 parathyroids become hyperplastic and secrete PTH to restore serum calcuim resutls i renal osteodystrophy(bone changes like primary but less severe) vasular calcificiation causes painful debilitating skin necrosis (calciphylaxis) |
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what is tertiary hyperparathyroidism?
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when parathyroid gland becomes autonomous in the setting of secondary hyperPTH
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what gene is affected in autoimmune diseases that cause hypoparathyroidism?
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APS1/AIRE gene
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what are the signs of hypocalcemia?
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neuromuscular excitabiilty
postive chvostek and trousseau signs hyperreflexia, muscle cramps tetany with laryngospasm mental status changes-irritabilty, psychosis, anxiety CALCIFICATIONS of ocular lens(cataracts) basal ganglia parkinsonian like symptoms increased ICP/papilledema cardiac conduction defects leading to QT interval prolongation |
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what is pseudohypoparathyroidism?
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genetic condition in which there is end organ resistance to PTH in target tissues
loss of calcium from kidney with resultant hypocaclemia and hyperphosphatemia serum levels of PTH are normal or high pts. may have short stature, round face, short neck, short metacarpals/tarsals. |
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what is the genetic defect in pseudohypoparathyroidism
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GNAS1 mutations
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what is produced in the adrenal medulla?
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epinephrine and norepinephrine(the catecholameines)
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what are the secretory cells of the adrenal medulla?
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chromaffin cells
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what is the most common cause of hypercortisolism?
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exogenous GC(physcian given steroids)
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what is the main cause of hypercortisolism endogenously?
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pituitiary produces excess ACTH(cushings disease)
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what is character of cushing's disease?
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bilateral hyperplastic adrenal glands
crook hyaline chagne seen in ACTH prodcuing basophils elavated ACTH and cortisol |
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what is crook hyline change?
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dense eosinohpilc stain around edeges of a cells in the pituitary, occurs in response to hypercortisolism
composed of intermediate keratin filaments |
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what are the characteristics of primary (adrenal) hypercortisolism?
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manly due to neoplasms
ACTH is low adenomas and carcinomas adults equal number of adenomas and carcinama children its predominatnly carcinoma hypercortisolism is more marked during carcinoma, contralateral adrenal cotex is atrophic |
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what is the characteristics of cortisol released from carcinoma?
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usually its not as effective as normal cortisol its malformed, but the carcinoma produces so much it over comes that fact.
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what is the one endocrine condition that is more common in men than women?
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ectopic ACTH secretion by nonpituitary tumors.
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what is the most common site associated with ectopic ACTH secretion ?
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small cell cancer of lung
also seen in carcinoid tumors medullary ca of the thyroid islet cell tumros of pancrease |
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what is the state of adrenals in ectopic ACTH secretions?
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adrenals are bilaterally hyperplastic
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what zone of the adrenal cortex is usually within normal limits during adrenal cortical atrophy due to exogenous cortisol release?
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the zona glomerulosa bc mineralacorticoids are under control of renin angiotnesin system.
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what is the complete list of symptoms in cushing syndrome?
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central obesity
moon facies weakness and fatigability hirsutiism plethora glucose intolerance/diabetes osteoporosis neuropsychiatric abnormalities mentrual abnormalities cutanous striae delayed wound healing/bruising |
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what is the response of pituitary cushings to dexamethasone suppression test?
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low dose- no effect
high dose- will decrease ACTH and urinary steroids are reduced |
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what is the response of extopic ACTH to dexamethasone supression test?
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no resprponse to low or high dose.
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what is the normal response to dexamethasone suppression test?
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low dose will produce lowering in ACTH levels and urinary steroids are reduced
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how will an adrenal tumor respond to dexamethasone suppression test?
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doesnt respond at all HOWEVER ACTH will be low even before administering any dose
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what are the effects of aldosterone?
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sodium retention adn potassium excretion
causeing hypertensino and hypokalemia |
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what are the causes of primary hyperaldosteroneism?
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aldosterone prodcuing noeplasm
primary adrenocortical hyperplasia glucocorticoid remediable hyperaldosteronism |
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what is the most common cause of hyperaldosteronism?
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prmary adrenocortical hyperplasia(idopathic)
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what is a possible gene defect in hyperaldosteronism?
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CYP11B2
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what is Conn syndrome?
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hyperaldosterone caused by a solitary adenoma in the adrenal gland
35% of cases of hyperaldosteronism |
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what are the features of glucocorticoid remediable hyperaldosteronism?
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uncommon familial disorder
chimeric gene leads to hybrind steroids under ACTH control will react to dexamethasone suppression test. |
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what are the secondary causes of hyperaldosteronism?
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decreased renal perfusion
arterial hypoolemia pregnancy all these will cause increase in renin angiotensin activity |
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WHAT CONTROLS ALDOSTERONE RELEASE?
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ONLY RENIN ANGIOTENSIN SYSTEM THE ONLY TIME ACTH AFFECTS IT IS IN GLUCOCOTICOID REMEDIABLE HYPERALDOSTERONISM.
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what are key features about hyperaldosteronism adneomas?
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more common on left side
bright yellow bc of lipids(true of all the adrenal adenoma) PAS reactive cytoplasmic inclusions= spironolactone bodies |
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what are clinical signs of hyperaldosteronism?
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hypertension-main
-secondary to expanded ECF due to soduim retention hypokalemia -weakness -parasthesias -visual disturbances -teteny |
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what is the main adrenogential syndromes?
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androgen secreting neoplasms are rare
the most common cause by far is Congenital Adrenal Hyperplasia(CAH) |
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what is CAH?
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group of AR, inihereted metabolic errors of a deficieny to total lack of a particular enzyem in the biosyntesis of cortical steroids.
steroidiogenesis is channeled into other pathways usually the androgen pathway which leads to virilization |
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what is the most common/most dangerous variation of CAH?
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classic salt wasting form caused by 21alpha -hydroxylase defeciency.
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what pathways are blocked in 21-alphahydroxylase defiency?
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blocks aldosterone and cortisol funneling steroid synthesis into the androgens
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what is the presentation of 21-alphahydroxylase deficincy?
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lack of mineralocorticoid production leads to hyponatremai, hyperkalemia, acidosis, hypotension, CV collaspe, and death
excess androgen leads to virilzation seen at birth in females but not until electrolyte imbalance occurs in males lack of cortisol leads to bylateral adrenal hyperplasia which allows for normal cortisol levels. |
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what is gross appeance of adrenals in CAH classic salt wasting form?
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bilaterally hyperplastic adrenal glands that are brown on cut surface due to lack of lipids
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what are the less common forms of CAH?
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simple virilizing- only causes the virilization
nonclassic- same as classic except with late onset in adulthood. ALL cases have the bilateral hyperplastic adrenal glands with brown surface |
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when can acute adrenocortical insufficiency occur?
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can occur in chronic insuffienct pts that have some sort of crisis occur that requirs immediate steroid output that the glands cant compensate for ie trauma, surgery, illness etc
can occur in rapid withdrawal from exogenous prescribed steroids that were taken for an extended period. or if acute crisis occurs while on those steroid treatments massive adrenal hemorrhage |
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what are the most common cases of massive adrenal hemorrhage/
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newborns with traumatic/hypoxic delivery(complicated by lack of prothrombin that newborns have)
post surgical pt maintained on anticoags with DIC bacteremic adrenal hemorrhage also known as waterhouse friderchisne syndrome. |
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what is waterhouse freiderichsen syndrome?
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overwhelmign bacterial infections associated with neiseria meningitidis
rapid hypotension leads to shock DIC with widespread purpura, espeaically skin rapidly developing adrenocortical insufficiency associated with massive bilateral hemorrhage -hemorrhage begins in medulla cause not understood most common in children treat with antibiotics and hormone replacement quickly. |
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what is chronic adrenal insufficnecy also called?
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addisons disease
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what % of the cortex must be lost for symptoms of adrenal insuffiency to manifest?
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90%
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what are the causes of addisons disease?
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autoimmune adrenalitis-most common
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what are the 3 types of autoimmune adrenalitis?
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autoimmune polyendocrinopathy syndrome type (APS1)
APS2 isolated autoimune addisons disease |
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what is the gene associated with APS1?
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AIRE1
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what is APS1 symptoms?
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chronic mucocutaneous candidiasis, ectodermal dysplasia, hypoadrenal, hypoparathyroid, hypogona, pernicious anemia
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what is APS2 symptoms?
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adrenal insuffieciency and autoimmune thyroiditis or type 1 diabtes
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What are the non autoimmune conditions that can cause addisons?
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infections- espeacially TB and fungal AIDs at increased risk
metastatic neoplasms from breast or lung rare genetics disorders |
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what is morphology of addisions disaease?
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usually small atrophic glands
lipid depletion of the cortex with lympohocytic infiltrate medulla spared |
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what is hyperpigmentation associated with in regaurds to endocrine?
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only seen in addisons not in any ectopic releases of ACTH.
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what are the clinical signs of addisons?
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hyperkalemia, hyponatremia, hypotension
hyperpigmentation nausea, anorexia, vomiting , weightloss, diarrhea glucocortiod deficiency with resultan hypoglycemia minor stress can cause acute fatal adrenal crisi |
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what is treatement in addisons?
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hormone replacent
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what is secondary chronic adrenal insufficiency?
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problem with pituitary
loss of ACTH release |
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what occurs in secondary chronic adrenal insufficiency?
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adrenal cortex atrophies
deficiency of glucocoticoids and sex hormones but no defcit of mineralocorticoids so no hyperkalmeia and hyponatremia(renin) no hyperpigmentation |
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what is treatment for secondary chronic adrenal insufficiency?
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give ACTH so that cortex will respond
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what are the traits of neoplasms in adrenals?
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poorly encapsulated
adenoma not functioning carcinoma more likely to be functional than adenomas carcinoma determined by invasion into vasuclar channels, mets to LNs and viscers espeacially the lungs are common. |
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what is a pheochromocytoma?
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functional tumor of the adrenal medulla producing cetecholamines causing HTN.
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what is the rule of 10s?
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most stats in reference to pheochromocytoma are 10%
10% bilateral 10%malignant 10% extra adrenal(occuring in paraganglion system called paragangliomas) 10% arise in childhood 10% associated with hypternsion 25% associated with famililal syndromes liek MENIIA and B, NF1 von hippel lindau, and sturge-weber |
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what is a unique feature of pheochromocytoma?
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turns brown or black when placed in a dichromate fixative bc of oxidatinoof catecholamines
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what is seen micro for pheochromocytoma?
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zellballen-small nests and alveolar groups of cells.
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how is malignancy determined in pheochromocytoma?
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only by presence of metasteses.
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what is clinical features of pheochromocytoma?
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hypertension is the dominent feature, but the high BP is abrupt and paroxysimal.
associated with tachycardia, palpitations, headache, sweating, tremor, and sense of apprehension cause by paraoxsymal catecholamine release cardiac complications due to ischemic damage secondary to catecholaming induced vasoconstiriction |
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what is the lab finding in pheochromocytoma?
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increased urinary catecholamine and their metablites-vanillymandelic acid and metanephrines.
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what are the features of the MEN syndromes?
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neoplasm arise at a younger age than spordic tumors
can arise either synchronoously or metachoronously tumors often multifocal even in one organ tumors usually preceded by an asymptomatic stage of endocrine hyperplasia tumors are more aggressive and recur more than sporadic tumors ****************************** know this |
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what are the specific features of MEN1?(wermer)
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3 Ps
parathyroid, pancrease, pituitary tumors pancreatic tumors aggressive and leading cause of morbidity and mortality duodenal tumors also seen mutation in MENIN gene |
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what are specific features of MEN2a?(Sipple)
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pheochromocytoma, thryoid medullary ca, parathyroid hyperplasia
germline mut in RET protooncogene-gain of function |
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what are the features of MEN2B(multiple mucosal neuromas)
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muliple neuromas or ganglioneuromas in skin,mouth,conjuctiva, GI and respiratory tract.
marfanoid body habitus pheocrhomocytoma medually thryoid ca 100% of the time single amino acid mutation of RET |
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Familial medually thyroid cancer
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varient of MEN2A without the other features.
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