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Path Endocrine
KYCOM Block 16
273
Pathology
Graduate
05/13/2014

Additional Pathology Flashcards

 


 

Cards

Term
most common causes of hyperfunction 4
Definition
adenoma - most common
acute inflammation
hyperplasia
cancer
Term
most common caues of hypofunction 6
Definition
autoimmune destruction - most common
destruction: infection, surgery, radiation
decreased stimulation
enzyme deficiency
neoplasia
congenital disorder
Term
how does adenoma cause hyperfunction
Definition
mass of tumor causes unregulated release of hormone from anaplastic cell due to genetic changes
release of hormones from non-endocrine tissue is paraneoplastic
Term
how does neoplasia cause hypofunction
Definition
tumor cells derived from non-functional components of endocrine gland compress functional tissue
hormones dont release due to genetic changes in the cell
Term
explain how the pituitary recieves blood and endocrine stimulation
Definition
ap gets blood from portal venous
pp gets blood from its own artery
ap is stimulated by hypothalamus to make hormones via portal vein delivery
pp recieves hormones through stalk into gland
Term
6 anterior pituitary cells and what they make
Definition
lactotroph > prolactin
mammosomatotroph > GH, prolactin
somatotroph > GH
thyrotroph > thyrotropin (TSH)
gonadotroph > FSH/LH
corticotroph > corticotropin (ACTH)
Term
6 anterior pituitary hormones and what regulates them
Definition
prolactin +TRH +GNRH -dopamine -somatostatin

GH +GNRH - somatostatin

TSH +TRH

FSH and LH +GNRH

ACTH +CRH
Term
2 posterior pitutiary hormones and their functions
Definition
ADH: regulates water in CD
oxytocin: mediates uterine contraction and release of breast milk when sucking
Term
causes of hyperpituitarism 5
Definition
adenoma - most common
hyperplasia
cancer
non-pituitary tumor
hypothalamus
Term
causes of hypopotuitarism 9
Definition
destruction: ischemia, surgery, radiation, inflammation

nonfunctioning adenoma: compress and destory functional tissue

craniopharyngioma: most common in kids

hypothalamic

sheehan syndrome

rathke cleft cyst

empty sella syndrome

genetic defects

specific hypofunctions
Term
pituitary local mass effect signs 3
Definition
silent tumors are most likley to have mass effect

lesion near sella: compress optic chiasm and cause bitemporal hemianopia (lateral visual deficit)

elevated ICP: headache, nausea, vomiting

pituitary apoplexy
Term
pituitary apoplexy: cause, signs 3, prognosis
Definition
acute hrmorrhage: rapid enlargement causes lesion to outgrow blood supply and hemorrhage into adenoma

headache, hypopituitary, diplopia

surgical emergency, can cause sudden death
Term
5 types of pituitary adenoma, cell type, which is most common, 1nd most common
Definition
prolactinoma - lacrotroph - 1st
growth hormone - somatotroph - 2nd
cortitroph - corticotroph
gonadotroph - LH/FSH
thyrotroph - TSH
Term
if someone has multiple cell types or lesions in their pituitary adenoma what should you suspect
Definition
multiple endocrine neoplasia 1
Term
which is more common hyper or hypopituitary
Definition
hyperpityitary
Term
genetic abnormalities associated with pituitary adenoma 4
Definition
monoclonal (even plurihumoral tumors)

some come from pituitary stems cells and differentiate

MEN1 mutation in non-sporatic

RAS activation with c-MYC in agressive

GNAS1 phosphorlyation mutation in somaatototroph, corticotroph, TSH, PTH
Term
what are 2 diseases that have a GNAS1 mutation
Definition
pituitary adenoma: somatotroph, cortioctroph, TSH, PTH

intraductal mucinous pancreatic andeoma
Term
if there is a plurihumoral pituitary adenoma what is the most common combination
Definition
growth hormone and prolactin
Term
which pituitary adenoma is often silent, what is it called
Definition
gonadotroph cell adenoma, null cell adenoma is silent form
Term
causes of prolactin elevation 8
Definition
intrasellar tumor producing prolactin
nonfunctioning pit tumor compresses stalk
hypothalamic neoplasm
head trauma
dopamine blocking drugs
pregnancy/breast feeding
renal failure decreases clearance
primary hypothyroidism (inc TRH)
Term
what is a craniopharyngioma
Definition
hypothalamic neoplasm
Term
what drugs block dopamine
Definition
dopamine antagonist: metoclopramide, phenothiazine, butyrophenone

delivery blocker: MAOI, TCA

antiHTN: verapmil, reispine, methyldopa
Term
signs of prolactinoma in perimenopausal women 5
Definition
amenorrhea
infertility
galactorrhea
oligomenorrhea
seen after DC or OC often
Term
signs of prolactinoma in postmenopausal women and men 7
Definition
mass effect: headache, neuro signs, impotence, infertility, decreased lobido, galactorrhea, gynecomastia
Term
diagnostic of prolactinoma: 2 tests, 3 things to rule out
Definition
serum prolactin: mild elevation (pulsatile so not always high)

MRI is conformation test, usually mass is small

biopsy is not needed

rule out pregnancy, thyroid, renal dysfunction
Term
signs of a prolactinoma biopsy 4
Definition
dystrophic calcification
plasmomma bodies: microcalcification
pituitary stones
gross calcification of tumor
Term
treatment of prolactinoma
Definition
dopamine agonist: bromocryptine
inhibits prolactin and shrinks tumor
Term
GH elevation: causes 5
Definition
pituitary adenoma
cancer
lymphoma
pancreatic islet cell tumors
paraneoplastic syndrome
Term
explain how a pituitary adenoma releases GH
Definition
mutation of GTPase a-subunit of Gs.
proliferation of somatotroph and mamosomatotroph cells causing release of GH that induces IGF-1 which induces cell proliferation and stops apoptosis
Term
2 types of GH adenoma
Definition
dense granulated: slow growing, more in adults >50yo

sparse granulated: fast growing, more in young
Term
signs of GH adenoma in adults 10
Definition
7-10y until diagnosis
acromeagly: big soft tissue, distal bones (feet, hands, jaw, ears, nose, visceral organs, heart),
thick skin
DEGENERATIVE ARTHRITIS
HEART FAILURE - #1 COD
HTN
headache
visual disturbance
sleep apnea
SECONDARY DIABETES: GH INCREASES GLUCOSE UPTAKE INTO CELLS
Term
signs of GH adenoma in kids
Definition
gigantism: linear bone growth increases body size, long arm snad legs
Term
how is GH adenoma diagnosed 3
Definition
elevated GH and IGF-1: GH goes to liver and causes increased IFG-1 which mediates tissue growth

AUTONOMOUS SECRETION OF GH: MOST SENSITIVE - FAILURE TO SUPRESS GH WITH ORAL GLUCOSE LOAD

MRI with contrast: most sensitive for determining source of GH
Term
treatment of GH adenoma 2
Definition
octerotide: blocks response in anterior pituitary to GNRH
GH receptor antagonist
Term
causes of increased ACTH 4
Definition
cortiticotroph adenoma
cushing syndrome
cushing disease: ACTH made due to tumor
post-adrenalectomy: nelson syndrome
Term
nelson syndrome: cause
Definition
pre-existing corticotroph tumor grows rapidly after surgical removal of adrenal gland due to loss of inhibitor effect of adrenal corticosteroids

corticotrophs pump out ACTH to help body demand for cortisol and adenoma grows large because adrenal glands are gone

NO HYPERCORTISOLISM
Term
nelson syndrome: signs
Definition
mass effect
hyperpigmentation: POMC > ACTH + MSH + B-endorphin
Term
signs of gonadotroph adenoma 3
Definition
neurologic symptoms due to mass effect
reduced testosterone in men decreases energy and libido
amenorrhea in premenopausal women
Term
cause of gonadotroph tumor symptoms
Definition
HYPOFUNCTION (NOT HYPERFUNCTION): non-functioning clonal cells compress functioning tissue
Term
how can you differentiate pituitary adenoma and pituitary carcinoma
Definition
histologically similar, diagnosis needs demonstration of metastasis
Term
what is the definition of hypopituitary
Definition
insufficient hormones when >75% of the pituitary parenchyma is lost
Term
non-functioning pituitary adenoma: signs 3
Definition
compresses pituitary causing loss of function
apoplexy: bleeding causing it to rapidly grow and compress
bitemporal hemianopsia
Term
what is the most common ischemic necorosis of the pituitary
Definition
sheehan syndrome
Term
cause of sheehan syndrome
Definition
in pregnancy there is high demand for pituitary hormones so it enlarges 2x but blood supply does not increase, anterior pituitary uses portal venous system so if there is blood loss (obstretic shock or hemorrhage) it causes infarct
Term
signs of sheehan syndrome 7
Definition
poor lactation
loss of pubic hair (LH)
ovarian failure
amenorrhea
adrenal insurfficiency
hypothyroid (later)
pallor (MSH deficiency)
Term
rathke cleft cyst cause
Definition
anterior pituitary is embryologically derived from rathke pouch
cysts accumulate proteinaceous fluid and expand, compormising normal gland
Term
empty sella syndrome 2 types
Definition
condition that destorys part of all of the pituitary

primary: diaphragm sella defect allows arachnoid and CSF to hermiate compressing pituitary in obese and women with multiple pregnancies

secondary: surgical removal of pituitary due to mass or pituitary necrosis from loss of function
Term
genetic defects that cause deficiencies of pituitary hormones 2
Definition
pit01 mutation
a pituitary transcription factor
Term
specific pituitary hormone deficiencies and their signs 4
Definition
GH: pituitary dwarfism (proportional)
TSH: hypothyroid
ACTH: hypoadrenalism, pallor
prolactin: no postpartum lactation
Term
DI definition
Definition
kidney cannot resorb water from urine causing large volume (polyuria) dilute urine (LOW SPECIFIC GRAVITY), polydypsia, thirst, hyponatremia
Term
2 types of DI and how to diagnostially distinguish
Definition
central: water deprivation test, cannot concentrate urine

nephrogenic: no response to desmopressin
Term
cause of central DI 4
Definition
head trauma
tumor
infection
inflammation of hypothalamus/pituitary
Term
treatment of central DI
Definition
desmopressin ADH analog
Term
nephrogenic DI: cause and pathogenesis
Definition
inherited mutation or drug (lithium) makes tubules not respond to ADH
Term
SIADH causes 4
Definition
malignant neoplasm: small CC
non-neoplastic diease of lung COPD, pulmonary infection
hypothalamus or post. pituitary injury
drugs
Term
signs of SIADH 5
Definition
ADH causes reabsorption of water and hyponatremia, cerebral edema, neurological dysfunction (mental status change, seizure)
Term
tx SAIDH 2
Definition
water restriction
dimethylcycline (ADH blocker)
Term
explain how the thyroid makes hormones
Definition
stimulated by TSH from anterior pituitary
takes up tyrosine which turns into thyroglobin and goes into follicle lumen
takes up iodine which is OXIDIZED with peroxidase into I2
I2 and thyroglobin ORGANIFIED with peroxidase to monodiotyrosine or diididothyrosine
mono and di COUPLED via peroxidase to T3 (most powerful)
di and di COUPLED via peroxidase to T4 (most prevelant)
T4 binds to TBG
Term
what are some things that effect the TBG levels 3, how does this change other thyroid marker levels
Definition
estrogen increases it
steroids and nephrotic syndrome decrease it
affects total T4 not free T4
Term
causes of primary hypothyroid 4
Definition
thyroprivic: radiation or thyroidectomy
goiterous: increased TSH enlarges thyroid
cretinism
myexedema
Term
causes of secondary hypothyroid
Definition
pituitary abnormality decreases TSH
Term
causes of tertiary hypothyroid
Definition
hypothalamus abnormality decreases TRH
Term
causes of goiterous hypothyroid 4
Definition
iodine deficiency
AUTOIMMUNE: HASHIMOTOS
drugs
inborn errors of thyroid metabolism
Term
what is the most common cause of hypothyroid in iodine deficient and iodine prevalent areas
Definition
deficient: iodine deficiency
prevalent: HASHIMOTOS
Term
4 drugs causing hypothyroid
Definition
methimazole
PTU
lithium
aminosalicylic acid
Term
6 thyroiditis
Definition
hashimotos
painless postpartum
painless sporatic
painful subacute
suppurative
ridels
Term
2 subacute thyroiditis
Definition
painless postpartum
painless sporatic
Term
3 chronic lymphocytic thyroiditis
Definition
painless postpartum
painless sporatic
hashimotos
Term
causes of primary hyperthyroidism 3
Definition
hyperplasia GRAVES DISEASE
hyperfunction multinodular goiter
hyperfunction adenoma of thyroid
Term
causes of secondary hyperthyroidism
Definition
pituitary abnormality increases TSH
Term
causes of tertiary hyperthyroidism
Definition
hypothalamic abnormality increases TRH
Term
2 thyroid goiter diseases
Definition
diffuse nontoxic simple gouter
multinodular goiter
Term
2 benign thyroid neoplasms
Definition
thyroid nodules
follicular adenomas
Term
4 thyroid carcinomas, which is most common
Definition
papillary - most common
follicular
medullary
anaplastic
Term
4 causes of cretinism
Definition
inborn error of thyroid metabolism
maternal hypothyroid: before fetus develops
thyroid agenesis in infant
dyshormonogenic goiter: unable to make TH due to peroxidase deficiency
endemic iodine deficiency area
Term
signs of cretinism 6
Definition
severe mental retardation: christ like
short stature
skeletal abnormalities
coarse facial features
enlarged protruding tongue
umbilical hernia
Term
who gets cretinism
Definition
INFANT TO EARLY CHILDHOOD
Term
who gets myexedema
Definition
MANIFESTATIONS MORE SEVERE IN YOUNG BUT MORE COMMON IN OLDER CHILDREN AND ADULTS
Term
cause of myexedema 4
Definition
iodine deficiency
hashimotos
lithium
thyroid ablation or removal
Term
signs of myexedema 19
Definition
decreased BMR and SNS

fatigue, apathy, mental slug
depression
muscle weakness
EDEMA
broad face
enlarged tongue
voice deepens
cold interolance
cool pale skin
weight gain (normal appetite)
reduced CO
breathlessness
decreased exertion capacity
bradycardia
SOA
constipation
decreased sweating
oligomenorrhea
hypercholesterolemia
Term
how can some thyroiditis syndomes make thyrotoxicosis and hypothyroidism
Definition
TH is stored in gland
in thyroiditis inflammatory cell damages follicles which release TH into circulation causing thyrotoxicosis
as stores deplete and thyroid heals hypothyroidism results untils cells replaced and enough TH is made
(rare occasions hypothyroid persists)
Term
6 thyroditis age ranges
Definition
hashimotos: all, peak 30-50
painless postpartum: childbearing
painless sporatic: all, peak 30-40
painful subacute: 20-60
suppurative: kids, 20-40
riedels: 30-60
Term
6 thyroditis gender predominances
Definition
hashimotos: male
painless postpartum: female
painless sporatic: female
painful subacute: female
suppurative: even
riedels: male
Term
6 thyroiditis causes
Definition
hashimotos: autoimmuune
painless postpartum: autoimmune
painless sporatic: autoimmune
painful subacute: viral resp infection
suppurative: infection
riedels: unk
Term
6 thyroditis pathological findings
Definition
hashimotos: lymphocyic infiltrate, germinal centers, fibrosis
painless postpartum: lymphocytic infiltrate
painless sporatic: lymphocytic infiltrate
painful subacute: giant cells, granulomas
suppurative: abscess
riedels: dense fibrosis
Term
6 thyroditis thyroid function
Definition
hashimotos: hypo
painless postpartum: tox > hypo
painless sporatic: tox > hypo
painful subacute: tox > hypo
suppurative: eithroid
riedels: euthroid
Term
6 thyroditis TPO antibodies
Definition
hashimotos: high
painless postpartum: high
painless sporatic: high
painful subacute: low or none
suppurative: no
riedels: usually
Term
6 thyroditis ESR
Definition
hashimotos: normal
painless postpartum: normal
painless sporatic: normal
painful subacute: high
suppurative: high
riedels: normal
Term
6 thyroditis iodine uptake
Definition
hashimotos: variable
painless postpartum: low
painless sporatic: low
painful subacute: low
suppurative: normal
riedels: low or normal
Term
who gets hashimotos
Definition
45-65 yo F>M
major cause of non-endemic goiter in children
Term
what are the three areas of hashimotos pathogenesis
Definition
autoimmune thyroid antigens
immunologic mechanism
genetic susceptability
Term
3 autoimmune thyroid antigens in hashimotos
Definition
anti-TSH receptor
anti-thyroglobulin
anti-thyroid peroxidase
Term
explain the immunologic mechanism of hashimotos
Definition
thyroid epithelium sensitizes T cells to thyroid antigen
T helper differentiates into CD4/8
Th activates plasma cells which make auto Ab
CD4 activates macrophages
CD8 causes T cell mediated toxicity
Term
what is some proof for the genetic connection in hashimotos 4
Definition
60% concordance in monozygotics
50% asymptomatic relatives have anti-thyroid Ab
associated with turner syndrome and down syndrome
Term
explain the pathological progression of hashimotos
Definition
initiall hashitoxicosis: current follicles destoryed, TH leaks into blood

then hypothyroid: cells gone, T4 decreases while TSH increases
Term
what markers can we use for hashimots and their significance 2
Definition
anti-thyroGLOBIN and anti-microsomal Ab
signs of thyroid damage
do not mediate disease
only used as markers
Term
1 complication of hashimotos and why
Definition
B cell lymphoma: increased germinal centers make post germinal center B cells that generate maginal zone which leads to lymphma and thyroid enlargement
Term
morphological characteristcs of hashimotos 5
Definition
thyroid gradually replaced by mononuclear cell infiltrates and fibrosis

chronic inflammation and formation of germinal centers

diffuse thyroid enlargement

HURTHLE CELLS: metaplastic response of follicular eithelium to injury with eosinophilic, granular cytoplasm

lymphocytes
Term
signs of hashimotos
Definition
PAINLESS ENLARGEMENT OF THYROID
middle aged woman
hashitoxicosis
hypothyroidism
Term
autoimmune diseases associated with hashimotos 3
Definition
SLE
myasthenia gravis
sjogerns
Term
signs of hyperthyroidism 30, which 3 are specific to graves only
Definition
increased BMR and SNS

weight loss (increased appetite)
warm skin
flushing
peripheral vasodilation
heat intolerance
sweating
TACHYCARDIA
increased cardiac contractility
increased peripheral O2 requirements
arrhythmia
tremor
hyperactivity
emotional liability
anxiety
insomnia
thyroid myopathy: decreased muscle mass b/c its used to make proteins
ocular staring gaze - graves
lid lag - graves
exothalamus - graves
hypermotility
malabsorption
diarrhea
OSTEOPEROSIS: bone reabsorption stimulated
fracture
hypercalcemia
oligomenorrhea
hyperchlesterolemia
hyperglycemia: TH causes gluconeogenesis
Term
what is the most common feature of hyperthyroidism, who is most likley to get them
Definition
cardiac manifestations
arrhythmia in the elderly
Term
who gets graves
Definition
women 20-40yo
Term
2 parts to the pathognesis process in graves
Definition
genetic susceptability
auto IgG thyroid effect thyroid growth and hormone release
Term
3 factors of genetic susceptability in graves
Definition
HLA-B8
HLA-DR4
polymorphism in CD8 T cell CTLA-4 gene
Term
3 auto-antibodies possible graves and what each does
Definition
TSI: IgG mimics TSH, binds TSH receptor, stimulates adenylyl cyclase and causes hormone release

TGI: activates TSG receptors causing proliferation of follicles

TBII: mimic TSH to cause hyperfunction OR inhibit TSH binding causing HYPOfunction
Term
what are the possible combinations of autoantibodies in graves
Definition
HAS TO HAVE TSI which is SPECIFIC TO GRAVES ONLY

could also have TGI or TBII

both forms of TBII (hyper/hypo function) can be in the same person
Term
morpholigcal characteristics of the graves thyroid 2
Definition
hypertrophy and hyperplasia of follicular epithelium

scalloping of colloid: white area of colloid around thyroid follicles
Term
graves disease triad of symptoms
Definition
hyperplasia of thyroid causes hyperthyroidism
infiltrative opthalmopathy
localized infiltrative dermopathy
Term
prognosis of graves
Definition
permant fibrous deposition may cause persistance of signs over life
Term
hi i'm out of order. why is there edema like broad facial features, enlarged tongue, hoarsness etc in myexedema
Definition
accumulation of matrix (GAGs, hyaluronic acid) in sin, SC tissue, visceral sites (esp larynx)
Term
graves triad: signs of hyperplasia 2
Definition
increased blood flow causes bruit
diffuse goiter
Term
graves triad: signs of infiltrative opthalmopathy and why
Definition
EXOPTHALMOS - GRAVES SPECIFIC
orbit perdipocyte fibroblasts have TSH receptors so IgG and T cell cytokines stimulate them to make more GAG causing myexedema
volume of extraocular muscles is increased displacing eyeball forward and weakening muscles
Term
graves triad: signs of infiltrative dermopathy and why
Definition
usually on shins (pretibial myexedema)
scaky thickening orange peel pigmented papule/nodules
fibroblasts of shin have TSH receptors so in response to IgG they secrete GAG causing tibial myexedma and dough like feel
Term
what is the most common cause of thyroid storm
Definition
graves disease
Term
cause and signs 4 of thyroid storm
Definition
abrupt hyperthyroidism releases excess catecholamines during sress

arrhythmia, hyperthermia, vomiting, hypovolemic shock
Term
tx and prognosis of thyroid storm
Definition
medical emergency, arrhythmia popssible

PTU stops oxidation, organification, and coupling in thyroid production and stops conversion of T4>T3
Term
lab signs of graves disease 4
Definition
increased T4
decreased TSH: T4 down regulates receptors on anterior pituitary
hypercholesterolemia
hyperglycemia
Term
tx graves disease 4
Definition
increase B adrenergic tone
decrease thyroid hormone synthesis: PTU blocks peroxidase, radioiodine ablation, thyroidectomy
Term
why do goiters form
Definition
impaired TH synthesis increases TSH causing hypertrophy and hyperplasia of follicular cells

many overcome TH deficiency making patient euthyroid but with goiter
some dont causing goiterous hypothyroid

proportional to level of TH deficiency
Term
cause of diffuse nontoxic goiter
Definition
goiterous diet: interferes with TH synthesis

brassica, cruciferae (cabbage, caluliflower, turnips, cassava) inhibit iodine transport
Term
morphological signs of diffuse nontoxic goiter 3
Definition
diffuse involvement of entire gland
no nodularity
follicles filled with colloid
Term
2 types of diffuse nontoxic goiter and their causes
Definition
endemic: soil, water, and food has low iodine

sporatic: goitrogen diet, hereditary enzyme defects (dyshormonogenic goiter)
Term
2 conditions caused by dyshormonogenic goiter
Definition
cretinism
diffuse nontoxic goiter - sporatic
Term
who is most likley to get a dyshormongenic goiter
Definition
puberty/young female
Term
cause of multinodular goiter
Definition
activating mutation in TSH signaling causes better response to trophic hormones in some nodes causing enlarged thyroid with nodules due to relative iodine reficiency
Term
morphological signs of multinodular goiter 5
Definition
uneven accumulation of colloid: causes tension, hemorrhage, scars, calcifications, increased nodularity

if unredolved recurrent hyperplasia causes involution
Term
2 types of multinodular goiter, which is most common
Definition
nontoxic euthyroid - most common
plummer syndrome thyrotoxicosis
Term
plummer syndrome: pathogenesis, morphology, signs
Definition
regions become TSH independent and secrete TH without TSH control
hyperactive nodules concentrate radioactive iodine (hot)
causes hyperthyroid symptoms
Term
other than being odd, what is the clinical significance of a multinodular goiter 3
Definition
mass effect: airway obstruction, dysphagia, compresses vessels in neck and thorax
Term
who gets thyroid nodules
Definition
4x more in women
higher in endemic goiter regions
Term
prognosis of thyroid nodules
Definition
majority not neoplastic
usually indolent (self limited) with 90% survival at 20y
Term
morphology of thyroid nodules
Definition
solitary nodule
palpable discrete swelling
normal in thyroid gland
Term
clues that a thyroid nodule has a high chance of being neoplastic 8
Definition
solitary
<20 >65yo
rapid growth
male
signs of invasion: dysphagia, neck pain, hoarsness
hx radiation to head or neck
family hx or cancer or polyposis (gardners syndrome)
hot nodule more likley benign and cold 10% malignant
Term
follicular adenma pathognesis
Definition
activation mutation in TSH receptor G protein a-subunit on follicular epithelium
increases cAMP and follicular cell proliferation and hormone release
non-functional, some make TH causing thyrotoxicosis (toxic adenoma)
Term
morphology of follicular adenoma 7
Definition
3cm
neoplastic: WELL DEFINED CAPSULE, local atypia, clear cells, mitotic figures
intact capsule distinguishes these as benign
HURTHLE CELLS: possible
Term
what are 2 diseaes that have hurthle cells
Definition
follicular adenoma
hashimots thyroiditis
Term
clinical signs of follicular adenoma 3
Definition
SOLITARY PAINLESS MASS
COLD NODULE NON-FUNCTIONAL
hemosiderin pigment
Term
who gets thyroid carcinoma
Definition
female adults
if in kid or late adult life male rate equals female
Term
who gets papillary thyroid carcinoma
Definition
20-40yo female exposed to ionizing radiation as kid (acne tx)
Term
genetic changes in papillary thyroid carcinoma 5
Definition
RET or NTRK1 rerrangement
BRAF oncogene activating mutation
RAS mutations

general: MAP KINASE PATHWAY ACTIVATED VIA TYROSINE KINASE RECEPTORS INCREASING GROWTH AND DIFFERENTIATION
Term
morphology of papillary thyroid carcinoma 6
Definition
solitary or multifocal
encapsulated or ill defined
papillae: fibrovascular stalk covered with cuboidal epithelium
nuclei: dispersed chromatin, clear, GROUND GLASS / ORPHAN ANNIE NUCLEI
PLASMOMMA (SIMMONA) BODIES: layers of calcification
Term
signs of papillary thyroid carcinoma 9
Definition
asymptomatic
freely moving nodule
cold mass
CERVICAL NODE MASS 1ST SIGN
hoarsness
dysphagia
cough
dyspnena
hematogenous metastasis to lung
Term
prognosis of papillary thyroid carcinoma
Definition
excellent
10y survival 95% even if spread to local nodes
worse if <40yo or distant metastasis
Term
pneumonic to remember papillary thyroid carcinoma
Definition
papillary
popular
papillae
palpable nodes
pupil nuclei
psammoma bodies
pediatric pt
positive prognosis
Term
hi, im out of order. pneumonic for thyroid storm
Definition
storm HITS girls cAMP
hyperthyroidism
infection/illness
trauma
surgery
girls more common
cAMP tx involves BB which stop cAMP
Term
follicular thyroid carcinoma: who gets it
Definition
40-60yo women
increased in areas of iodine deficiency
Term
follicular thyroid carcinoma: morphology 3
Definition
minimally invasive or very invasive
plenty of colloid (looks like papillary adenoma somtimes)
NO CAPSULE OR INVASION THROUGH CAPSULE
Term
follicular thyroid carcinoma: mutations 2
Definition
RAS oncogene mutation
PAX8-PPARyI fusions
Term
follicular thyroid carcinoma: signs 4
Definition
slowly enlarging painless nodule
cold usually on scan
rare invades lymphatics
hematogenous dissemination to bone, lung, liver
Term
why do you use fine needle aspiration on thyroid and not normal biopsy
Definition
it bleeds lots
Term
prognosis of follicular thyroid carcinoma
Definition
poor if widley invasive form
10y survival 50%
Term
4 carcinomas that disseminate hematogenously instead of the normal nodes first
Definition
follicular of thyroid
renal cell
hepatocellular
choriocarcinoma
Term
who gets medullar thyroid carcinoma
Definition
40-60yo
Term
medullar thyroid carcinoma: 5 causes
Definition
MEN2A
MEN2B
activating RET mutation seen in MEN2A/B too
familial medullary thyroid carcinoma
80% sporatic
Term
what mutation permits prophylactic thyroidectomy, what diseases is it associated with
Definition
MEN2A/B and medullary thyroid carcinoma
activating RET mutations
Term
medullar thyroid carcinoma: pathogenesis 5
Definition
PARAFOLLICULAR C CELL HYPERPLASIA - SUSPICIOUS FOR FAMILIAL SYNDROMES

calcitonin secretion: does not caucse hypocalcemia our calcitonin sucks

secrete somatostatin, serotonin, VIP causing VIP diarrhea
Term
medullar thyroid carcinoma: morphology 4, and how it tells us the cause of the cancer
Definition
sporatic: solitary nodule
familial: multiple lesions, C-cell hyperplasia
stroma: amyloid deposits from altered calcitonin
Term
medullar thyroid carcinoma: signs
Definition
neck mass
dysphagia
hpoarsness
PARANEOPLASTIC VIP DIARRHEA
SINGLE COLD NODULE
Term
medullar thyroid carcinoma: prognosis
Definition
MEN2-RET mutation has poor prognosis
prophylactic thyroidectomy needed
Term
anaplastic thyroid carcinoma: who gets it
Definition
>65yo!
Term
anaplastic thyroid carcinoma: signs 3
Definition
HX MULTINODULAR GOITER
hx of thyroid cancer
rapidly enlarging bulky neck mass (compression and invasion symptoms)
Term
anaplastic thyroid carcinoma: morphology
Definition
malignant cells that look nothing like thyroid tissue
Term
anaplastic thyroid carcinoma: prognosis
Definition
agressive
mortality 100%
no effective therapy
often invades local structures causing dysphagia and respiratory compormise
Term
explain the normal embryology and function of the parathyroid and the hormone functions
Definition
derived from pharyngeal pouches, descends from thymus so parathyroid tissue can be anywhere from thyroid to thymus
chief cells: regulate free Ca via PTH secetion
PTH increases osteoblasts which increase osteoclast which reasorb bone and release Ca and P, increase SI absorption of Ca and P, increase renal Ca reabsorption and decrease P reabsorption in kidney
Term
digeorge syndrome sign and cause
Definition
agenesis of parathyroid due to improper descent of pharyngeal arches
hypocalcemia
Term
causes of primary hyperparathyroid 3, which is most common
Definition
parathyroid adenoma - most common
primary hyperplasia diffuse or nodular
parathyroid carcinoma
Term
causes of secondary hyperparathyroid 4, which is most common
Definition
renal failure - most common
dietary Ca deficiency
stratorrhea
vitamin D deficiency
Term
causes of hypercalcemia, which is most common
Definition
immobilization: promotes bone loss
thiazide diuretics
vitamin D toxicity
sarcoidosis and OTHER GRANULOMATOUS DISEASES which activate vitamin D and Ca
thyrotoxicosis: T3 STIMULATES OSTEOCLAST BONE REABSORPTION
MALIGNANCY - MOST COMMON
Term
2 malignancies that cause hypercalcemia
Definition
osteolytic metastasis: cancer erodes bone
paraneoplastic: SQUAMOUS cell carcinoma of the lung makes PTH
Term
causes of hypoparathyroid 5
Definition
surgery
congenital parathyroid absence
familial hypoparathyroid: autoimmune polyendocrine syndrome 1
idiopathic hypoparathyroid: autoimmune Ab against CASR
digeorge syndrome
Term
causes of pseudohypoparathyroid
Definition
mutation in G-protein a-subunit GNAS1 stops PTH signal on organs

GNAS1 is selectivly imprinted on different tissues

pituitary and kidney paternal allele silenced so mutation in mom expressed

other tissues: no imprinting so 50% decrease in function when there is a mutation
Term
possible mutations causing familial primary hyperparathyroidism 3
Definition
MEN1 OR RET ACTVATING MUTATIONS
Ca sensing receptor (CASR) mutation

familial hypocaluric hyperclacemia: Ca receptors in PT cannot sense Ca so it release PTH, Ca receptors in kidne are deficient and percieve low Ca, PTH stimulates reabsorption of Ca in kidney (less Ca in urine)
Term
possible mutations causing sporatic primary hyperparathyroidism
Definition
MEN1 OR RET ACTIVATING MUTATIONS

sporatic parathyroid adenoma (PRAD1): mutation in cyclin D1
Term
what is more common hyper/hypo parathyroid benign/malignant
Definition
hyperparathyroid
benign: cancer of thyroid is rare more likley adenoma
Term
causes of asymptomatic primary hyperparathroidism 2
Definition
sporatic parathryoid adenoma 1
familial hypocalcuric hypercalcemia
Term
how does morphology of primary hyperparathyroid distinguish between causes
Definition
adenoma involves 1 gland
hyperplasia involves 4 glands (familial)
PT carcinoma cannot be distinguished from adenoma
Term
how do you diagnose parathyroid carcinoma
Definition
PT carcinoma cannot be distinguished from adenoma and metastasis or local invasion is only proof
Term
signs of primary hyperparathyroid 15
Definition
asymptomatic hypercalcemia
nephrolithiasis: Ca oxylate kidney stones
nephrocalcinosis: metastasic calficiation, polyuria
constipation
nausea
peptic ulcers
gall stones
acute pancreatitis: Ca activates enzymes
depression
lethargy
seizures
weakness of muscles
fatigue
heart valve calcifications
osteofibrosis cystica: fibrosis, cyst, hemorrhage in bone due to increased PTH
Term
pneumonic for primary hyperparathyroid
Definition
painful bones
renal stones
abdominal groans
psychic moans
Term
lab signs of primary hyperparathyroid
Definition
increased PTH, Ca
decreased P
increased urinary cAMP
increased alkaline phosphatase
Term
why is there increased urinary cAMP in primary hyperparathyroid
Definition
PTH binds tubular cells of kidney activating Gs then adenylylate cyclase which converts ATP to cAMP which mediates PTH
Term
why is there increased alkaline phosphatase in primary hyperparathyroid
Definition
PTH does not directly activate osteoclast
osteoblast activated first and lays down bone making alkaline phosphatas ebefore osteoclast breaks down bone
Term
how does renal failure cause secondary hyperparathyroid
Definition
decreased P excretion causes hyperphosphatemia
Ca and P can percipitate and are taken up into bone causing hypocalcemia
PT sees less Ca so it increaes PTH from ALL 5 GLANDS
decreased a1-hydroxylases for Vitamin D activation
Term
lab signs of secondary hyperparathyroid 4
Definition
increased PTH
Ca low
increased P
increased alkaline phosphatase
Term
signs of secondary hyperparathyroid
Definition
renal osteodystrophy
calciphylaxis: ventricular calcifications cause ischemic damage to skin and organs
Term
cause of tertiary hyperparathyroid
Definition
secondary hyperparathyroid leads to autonomous excessive parathyroid production
Term
signs of hypoparathyroid 24, which is the hallmark
Definition
tetany - hallmark
muscle apasm
numbness
tingling
chovsteck sign: tap cheek causes facial muscle spasm
trosseau sign: BP cuff induces carpal spasm
laryngospasm
seizure
emotional instability
anxiety
depression
confusion
psychosis
hallucinations
calcification of basal galgnia causes parkinson like movement
increased ICP
papilledema
cataracts
conduction defecit prolonged QT
dental abnormalities if occurs early in development
dental hypoplasia
failed eruption of teeth
defective enamel and root
abraded carious teeth
Term
labs of hypoparathyroid 2
Definition
low PTH
low Ca
Term
cause of pseudohypoparathyroidism 1A
Definition
mutation in maternal allele which is only one in pituitary and kidney decreases stimulation by anything (not just PTH) that uses cAMP

so TSH, GNRH, PTH
Term
cause of pseudohypoparathyroidism 1B
Definition
mutation is on paternal allele but maternal is fine, so no multihormone resistance
Term
signs of pseudohypoparathyroidism 1A (5)
Definition
multihormone resistance: short stature, short 4th-5th digits, obesity, mental deficits

albright hereditary osteodystrophy: skeletal and developmental defects
Term
signs of pseudohypoparathyroidism 1B (1)
Definition
albright hereditary osteodystrophy: skeletal and developmental defects
Term
adrenal medulla: embryology, hormone release and breakdown
Definition
neural crest cells > chromaffin cells > catecholamines

epinepherine > meanephrine + MAO > VMA > trash

NE > normetanepherine + MAO > VMA > trash
Term
how does the adrenal cortex make hormones and in what locations
Definition
cholesterol > pregneolone...

... + 21-hydroxylase + 11-hydroxylase > ALD in glomerulus

... +17-hydroxylase + 21-hydroxylase > weak mineral corticoid > 11-hydroxylase > glucacorticoids (cortisol in fasiculata)

... + 17-hydroxylase > sex streoids in reticularis
Term
functions of ALD 2
Definition
principal cells od CD: retain Na, excrete K

a-intercalated cells of CD: excrete H
Term
3 adrenocortico hyperfunction syndromes
Definition
hypercortisolism
hyperaldosteronism
androgenital syndromes
Term
causes of hypercortisolism, which is most common
Definition
cushing disease - most common endogenous

ACTH independent cushing syndrome (adrenal cushing syndrome)

iratogenic cushing syndrome - most common

paraneoplastic cushing syndrome
Term
causes of primary hyperaldoseronism 4
Definition
adrenocortical neoplasm (conn syndrome, adrenocortical carcinoma)

primary adrenocorticol hyperplasia (idopathic hyperaldosteronism)

glucocorticoid-remediable hyperaldoseronism
Term
causes of secondary hyperaldosteronism
Definition
decreased renal perfusion

arterial hypobolemia and edema: CHF, cirrhosis, nephrotic syndrome

pregnancy: estrogen induced increase in plasma renin
Term
4 causes and how does decreased renal perfusion cause secondary hyperaldoseronism
Definition
renal artery stenosis, arteriolar nephrosclerosis, fibromuscular dysplasia of renal artery, athlerosclerosis of renal artery (eso old men)

decreases flow and increases renin
Term
2 adrenogenital syndromes
Definition
adrenocortical neoplasm associated with virilization

congenital adrenal hyperplasia
Term
4 adrenocorticol hypofynction diseases
Definition
adrenal crisis: primary acute adrenocortical insufficiency

addisons: primary chronic adrenocortical insufficiency

secondary adrenal insufficiency
Term
causes of adrenal crisis 3
Definition
rapid withdrawl of steroids

stress in pt with chrnic adrenocortical insufficiency

massive adrenal hemorrhage
Term
causes of addisons disease 10
Definition
90% autoimmune adrenalitis, TB, AIDS, metastic lung cancer
10% lymphoma, amyloidosis, sarcoidosis, hemochromatosis, fungal, adrenal hemorrhage
Term
cause of secondary adrenal insufficiency 2, signs 3
Definition
any disorder that reduces ACTH (hypopituitary, prolonged steroids)

simillar to addisons
NO hyperpigmentation
normal ALD synthesis
Term
2 adrenal tumors
Definition
adrenocortical adenoma
adrenocortical carcinoma
Term
3 diseases of the adrenal medulla
Definition
pheochromocytoma
paraganglioma
neuroblastoma
Term
2 causes of cushings disease, which is most common
Definition
ACTH producing pituitary microadenoma - most common

corticotroph cell hyperplasia due to hypothalamic tumor releasing CRH
Term
who gets cushing disease
Definition
20-30yo women
Term
morphology of cushing disease 3
Definition
adrenal glands hyperplastic due to INCREASED ACTH STIMULATION
ONE GLAND LARGE, ONE ATROPHIC
Term
cause of ACTH independent cushing syndrome 3
Definition
adrenal adenoma and carcinoma in adults
adrenal carcinoma in kids
Term
pathogenesis of ACTH independent cushing syndrome
Definition
autonomous production of cortisol by adrenal glands increases cortisol despite DECREASED ACTH
Term
morphology of ACTH independent cushing syndrome 2
Definition
adrenal cortex OPPOSITE GLAND ATROPHY DUE TO DECREASED ACH

if adenoma gland atrophies due to decreased stimulation
Term
cause of iratogenic cushing syndrome
Definition
administration of exogenous steroids causes BOTH GLANDS TO ATROPHY due to supression of ACTH by therapy
Term
causes of paraneoplastic cushing syndrome 4, which is most common
Definition
small CC of lung - most common
carcinoid tumor
medullary thyroid carcinoma
pancreatic islet cell tumor
Term
6 ways cushings syndrome changes physiology and the symptoms each of these ways causes, 6 other symptoms
Definition
HTN: cortisol upregulates a1 on arterioles increasing effects of NE

high glucose releases insulin which results in fat storage (truncal obesity, moon facies, buffalo hump, weight gain (central adipose)

cortisol protein catabolism stops synthesis of collagen making vessels weak and rupture (striae on abdomen, osteoperosis, easy bruising)

cortisol raises blood sugar by breaking down muscles for gluconeogenesis (muscle weakness, thin extremities, hyperlgycemia, glucosuria, polydypsia, secondary DM, atrophy of fast twitch myofibrils, decreased muscle mass)

cortisol inhibits phospholipase A2 so cannot make arachadonic acid for inflammation, inhibits IL-2 so cannot make T cells, inhibits histamine from mast cells so no vasodilation or permeability (immunodeficiency)

mental disturbances, mood swings, depression, psychosis

histurism, menstural abnormalities
Term
labs of cushings syndrome 2
Definition
increased urine 24h cortisol showing loss of diumal cortisol secretion (cortisol increases in morning and decreases at night so blood cortisol is not as consistant as urine cortisol)

dexamethasone supression test helps determine cause
Term
explain how dexamethasone supression test works
Definition
pituitary adenoma increases ACTH
adrenal adenoma decreases ACTH
ectopic PNP increases ACTH

if ACTH is decreased it is adrenal adenoma

if cortisol decreases with DEX it is pituitary adenoma

if cortisol does not decrease with DEX it is small cell lung cancer

low DEX is designed to decrease normal cortisol so if they have disease it shouldnt return them to normal levels

high DEX will decrease cortisol in pituitary adenoma
Term
pneumonic for cushing syndrome
Definition
cushing
4C 2U 2S 4H 1I 1N 2G

central obesity
cervical fat pad
collagen fiber weakness
comedones - acne

urinary free cortisol and glucose

striae
supressed immunity

hypercorticolism
hypertension
hyperglycemia
hirsturism

iratogenic - increased steroid pills

noniratogenic - neoplasms

glucose intolerance
growth retardation
Term
labs in primary hyperALD 3
Definition
increased Na, blood volume
decreased renin
Term
labs in secondary hyperALD 3
Definition
increased renin
increased Na and blood volume
Term
cause of glucocorticoid remediable hyperALD and dx
Definition
fusion of 11-B-hydroxylase and ALD synthase causes chimeric gene that continues producing chimeric proteins that increase cortisol and ALD

supressed by exogenous dexamethasone because ACTH can supress ALD
chimeric gene is under infence of 11B-hydroxylase so steroids work
Term
signs of primary adernocortical hyperplasia 2
Definition
idiopathic bilateral nodular hyperplasia
most common in kids and young adults
Term
how can you diagnost adrenocortical neoplasms
Definition
conn syndrome: adrenocortical adenome that makes ALD in middle age women

does not supress ACTH secretion like cushings, no atrophy of normal glands
Term
signs of hyperALD 10
Definition
hypernatremia
hypokalemia
metabolic acidosis
HTN
EKG changes
cardiac decomposition
weakness
parasthesias
visual disturbances
tatany
Term
define adrenogenital syndrome
Definition
sex steroid release by adrenal cortex caused by autonomous production by cells in zona reticularis (neoplasms, congenital adrenal hyperplasia0 or as component of cushings disease (stimulation by increased ACTH)
Term
who gets congenital adrenal hyperplasia 2
Definition
hispanics
aschkenazi jews
Term
cause of congenital adrenal hyperplasia 3
Definition
90% 21-hydroxylase (CYP21B) recombination with neighboring non-functional pseudogene causing chymeric gene (deg non functioning pseudogene determines severity)

11-hydroxylase deficiency

17-hydroxylase deficiency
Term
morphology of congenital adrenal hyperplasia 2
Definition
adrenals hyperplastic bilateral

anerior pituitary corticotroph cell hyperplasia
Term
explain the pathogenesis and 3 forms of 21-hydroxylase deficiency, which form is most common
Definition
unable to make ALD and cortisol so increases production of sex steroids

severity is based on amount CYP21B replaced with pseudogene

salt wasting (Classic) androgenitalism: most severe

simple virilizing: less salt wasting

non-classic: most common, few symptoms
Term
signs of 21-hydroxylase deficiency 7
Definition
decreased cortisol and ALD...

soon after birth: salt wasting, hyponatremia, hyperkalemia

later: hypotension, CV collapse, possible death

virilization: clitoral enlargement in females (boys cant tell until salt wasting)

precocious puberty in males

decreased catecholamines causes hypotenion and circilatory collapse
Term
explain pathogenesis of 11-hydroxylase deficiency
Definition
cant make ALD and strong glucocorticoids so increased production of sex streoids

enough ALD for salt reabsorption but lowered cortisol fails to inhibit ACTH so precursors are shunted to androgen pathway
Term
signs of 11-hydroxylase deficiency 2
Definition
virilization
precocious puberty
Term
explain pathogenesis of 17-hydroxylase deficiency
Definition
unable to make glucocorticoids and sex steroids
increased production of ALD
Term
signs of 17-hydroxylase deficiency 4
Definition
asymptomatic
histurism
oligomenorrhea
delayed menarche
Term
causes of massive adrenal hemorrhage that leads to hypofunction 4
Definition
newborn delivery trauma and hypoxia

anticoagulant therapy

DIC with adrenal infarct

waterhouse friderichsen syndrome: bacterial infection and adrenal hemorrhage
Term
cause 4 and pathogenesis of waterhouse fridrichen syndrome
Definition
child with N. meningitis, pseudomonas, H. influenza, staph

infection causes DIC, hypotension, sock, purpura which causes bilateral necrosis of adrenal glands

acute decrease in cortisol exacerbates hypotension due to DIC/shock

adrenal glands look like sac of blood
Term
signs 5 and prognosis of adrenal crisis
Definition
intractable vomiting
abdominal pain
hypotension
coma
vascular collapse

die in days wo tx
Term
cause of autoimmune adrenalitis 3
Definition
auto-ab destruction of 21-hydroxylase

autoimmune polyendocrine syndrome 1: mutation in autoimmune regulator AIRE gene (causes death of self reactive T cells in thymus)

autoimmune polyendocrine syndrome 2: not single gene disorder, isolated immune addisons disease
Term
signs of autoimmune polyendocrine syndrome 1 (6)
Definition
chronic mucocutaneous candidiasis

ectodermal dystrophy: abnormalities of skin, dental enamel, nails

adrenalitis
hypoPTH
idiopathic hypogonadism
pernicious anemia
Term
signs of autoimmune polyendocrine syndrome 2 (4)
Definition
adrenal insufficiency
autoimmune thyroiditis
DM1
early adult presentation
Term
TB adrenalitis: epidemiology, cause
Definition
once 90% of addisons disease, less common now

infection at other sits (lung, GU)
Term
metastic neoplasm to adrenal gland primary locations 5, which is most common
Definition
lung - most
breast - most
GI
melanoma
hematopoietic
Term
genetic disorder of adrenal insufficiency
Definition
adrenoleukodystrophy
Term
signs of addisons
Definition
insidous 90% of gland must be destoried
progressive weakness
easy fragility
anorexia
N/V
weight loss
diarrhea
hyperpigmentation (esp oral mucosa)
hyperkalemia
hyponatremia
volume depletion
hypotension
hypoglycemia
Term
why is there hyperpigmentation in addisons
Definition
no cortisol means increased ACTH which comes from POMC which breaks into MSH
Term
pneumonic for addisons
Definition
autoimmune
DIC - meningococcus
Destruction by cancer, infection, vascular insfficiency
iratogenic
sarcoidosis, granulomatoous like TB, histomycosis
hypOtension/hypOnatremia
nelson syndrome: post adrenalectomy, increased ACTH
Term
signs of adrenocortical adnoma 2
Definition
USUALLY SILENT found on autopsy
if functional may atrophy neighboring cortex and opposite gland due to supression of ACTH by tumor products
Term
adrenocortical carcinoma: signs, 2 assciated diseases, prognosis
Definition
if virilizing (causes masculinization) more likley carcinoma

li-fraumeni, beckwith-weidmann

strong risk to invade adrenal vein, vena cava, lymphatics
Term
pheochromocytoma pathogenesis
Definition
neoplasm of chromaffin cells that makes catecholamines, hormones, or seroids (PNP cushings or endocrinopathies)
Term
pheochromocytoma rule of 10s (5)
Definition
10% familial
10% extra-adrenal
10% nonfamilial are bilateral
10% in kids (familial, male)
10% malignant
Term
familial mutations of pheochromocytomas 5
Definition
MEN2A
MEN2B
von recklinghausen (NF1)
von hippel-lindau: AD loss VHL tumor supressor
sturge-weber syndrome
Term
3 tumors of von-hippel-lindau
Definition
pheochromoytoma
hemangioblastoma of cerebellum
renal cell carcinoma
Term
most common age of pheochromocytome
Definition
female 40-60
Term
common place of pheochromocytoma malignancy and sign
Definition
bladder wall (episodic HTN on urination)

more chance of metastis when extra-adrenal
Term
morphology pheochromocytoma 3
Definition
small 1g to large 4kg
brown with yellow edge (cortex cholesterol)
lesion and L dichromatic dark brown (oxidation of catecholamines)
Term
how do we determine if pheochromocytoma is malignant
Definition
we dont we see if it metastasis
Term
signs of pheochromoctyoma
Definition
acute HTN <50%
chronic HTN 2/3
tachycardia
plapitations
headache
sweating
tremor
apprehension
abdominal pain
chest pain
nausea
vomiting
Term
what are pheochromocytoma symptoms percipitated by 4 and why, what symptoms does this cause 6
Definition
emotions, exercise, postural change, palpation of tumor

causes release of catecholamines causing catecholamine cardiomyopathy, myocardial instability, ventricle arrhythrmia, CHF, MI, CVA
Term
labs of pheochromocytoma
Definition
increased urinary catecholamines and metabolites (VMA, metanepherines)
Term
treatment pheochromocytoma
Definition
surgical excision AFTER phenobenzomine (irreversible a blocker so when they touch it catecholemines dont releease and kill pt due to massive HTN)
Term
what is a paraganglioma
Definition
extra renal pheochromocytoma
Term
what is the most common extracranial solid tumor of childhood (first 5y)
Definition
neuroblastoma
Term
pneumonic pheochromocytoma
Definition
PHE
palpitations
headache
episodic sweating (diaphoresis)
Term
features of MEN 5
Definition
occur at young age
arise in multiple endocrine organs (sometimes synchronized)
often multifocal tumors in single organ
tumors usually preceded by hyperplasia stage
tumors more agressive and recur
Term
men 1 mutation aka, pathogenesis
Definition
wener syndrome

germline mutation of menin tumor supressor that regulates cell cycle and transcription
Term
abnormalities in men 1 mutation
Definition
primary hyperparathyroid: 40-50yo, precered by hyperplasia

pancreas: agressive, metastasis, leading mortality, seen with hypersecretion

prolactinoma

enodenomal gastrinoma - frequent
Term
hypersecretion syndromes associated with men 1 (2)
Definition
zollinger ellison: gastrinoma, peptic ulcers

insulinoma: hypoglycemia, nueurologic signs
Term
men 2 pathogenesis
Definition
activating mutation iin RET protoncogene receptor TK transmits growth and differentiation signals
Term
men 2A mutation aka, 4 parts
Definition
sipple syndrome

medullary thyroid carcinoma 100%!: multifocal agressive PROPHYLACTIC THYROIDECTOMY

familial medillary thyroid cancer: varient with medulla carcinma risk

pehochromocytoma: bilateral, common extranodal

PARATHYROID HYPERPLASIA: hypercalcemia, nephrolithiasis
Term
men 2B mutation 4 signs
Definition
medullary thyroid carcinoma: more agressive than 2a

pheochromocytoma

neuroma: skin, oral mucosa, eye, respiratory tract, GI tract

marfanoid habitus: long axial skeleton, hyperextensible joints
Term
pneumonic for men
Definition
men 1: pituitary parathyroid pancreas

men 2: carcinoma of thyroid, catecholamines (pheochromocytoma)
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