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98 Cards in this Set

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Pituitary Adenoma
sporadic, may be associated w/ Multiple Endocrine Neoplasia Syndrome
May store/secrete any combination of hormones
Micro<1cm Macro>1cm
-->hyperfunction, visual changes, oculomotor palsy, hypothalamus invasion
pituitary adenoma
sporadic, may be associated w/ Multiple Endocrine Neoplasia Syndrome
May store/secrete any combination of hormones
Micro<1cm Macro>1cm
-->hyperfunction, visual changes, oculomotor palsy, hypothalamus invasion
Pituitary Adenoma
sporadic, may be associated w/ Multiple Endocrine Neoplasia Syndrome
May store/secrete any combination of hormones
Micro<1cm Macro>1cm
-->hyperfunction, visual changes, oculomotor palsy, hypothalamus invasion
hypopituitarism
compression by tumor
Sheehan Syndrome - post partum pituitary infarct
Pituitary Apoplexy - infarct of adenoma (presents w/ headache & visual changes)
Trauma
Inflammation
Iron Deposition (hemochromatosis)
Isolated Hormone Deficiencies
GH (Kallmann Syndrome)
End organ resistance (Laron Syndrome)
Empty Sella syndrome (herniation)
Lactotrope Adenoma (Prolactinoma)
Most common
may show amyloid and psammoma bodies (spherical calcified structures)
females: amenorrhea, galactorrhea, infertility
males: decreased libido, erectile dysfunction
TX: Bromocriptine (DA agonist) shrinks tumor
Somatotrope Adenoma (GH)
prior to epiphyseal closure - Gigantism
after epiphyseal closure - Acromegaly
Acromegaly: course facies & hair, enlarged hands, feet, head, & organs, headache, musculoskeletal symptoms, peripheral neuropathy, DM, hypercalciuria, hyperprolactinemia
TX: surgery, somatostatin (GH antagonist)
Corticotroph Adenoma (ACTH)
increase proopiomelanocortin increases ACTH, lipocortin, MSH, endorphins (all cleaved from precursor)
Cushing's Disease
Nelson's Syndrome - rapidly growing corticotroph adenoma following adrenalectomy for cushings
Gonadotrope Adenoma (FSH & LH)
more common in men, causes testosterone suppression and hypogonadism
Thyrotropin Adenoms (TSH)
Least common, causes hyperthyroidism, goiter and/or pit mass lesions
nonfunctional adenomas
null cell, oncocytoma, silent
Central Diabetes Insipidus
ADH deficiency --> polydypsia, polyuria
caused by: sporadic mutations, familial, tumors, inflammatory processes (sarcoid)
Craniopharyngioma
cystic supersellar mass
squamous cells
from Rafke's pouch
kids, men/women 50s-60s
compression symptoms or variety of hormonal symptoms
Craniopharyngioma
cystic supersellar mass
squamous cells
from Rafke's pouch
kids, men/women 50s-60s
compression symptoms or variety of hormonal symptoms
craniopharyngioma
cystic supersellar mass
squamous cells
from Rafke's pouch
kids, men/women 50s-60s
compression symptoms or variety of hormonal symptoms
acromegaly
result of a somatotrope adenoma after epiphyseal closure
coarse facies, enlargement of hands, feet, head, internal organs
headache, musculoskeletal symptoms, peripheral neuropathy, DM, hyperCa, Hyperprolactinemia
Tx: surgery, somatostatin
Posterior Pituitary
Neurohypophysis
from evagination of floor of the diencephalon
axon terminals from paraventricular & supraoptic nuclei
vasopressin & oxytocin
anterior pituitary
anterior pituitary
Thyroid
T4 - deiodinated in periphery
T3 - regulates gene transcription, increased thermogenesis, increased metabolic rate, increased gluconeogenesis, increased glycogenolysis
Goiter
Nontoxic goiter
enlargement not associated w/ functional, inflammatory, or neoplastic alterations. may progress to toxic multinodular goiter
TX: thyroid hormone, decrease TSH, radioactive iodine or surgery if compression
Nodular Goiter
more common >50y/o, female

toxic = functional, will cause hyperthyroid unresponsive to TH administration, presents similar to toxic adenoma, T3/T4 mildly elevated
Hypothyroid
defective synthesis of thyroid hormone (compensatory goiter), iatrogenic, inadequate TSH or TRH
effects: myxedema, easy bruise, lethargy, confusion, agitation, decrease CO, dilated cardiomyopathy, increase PVR, constipation, anovulatory (female), erectile dysfunction & oligospermia (male)
Nodular Goiter
more common >50y/o, female

toxic = functional, will cause hyperthyroid unresponsive to TH administration, presents similar to toxic adenoma, T3/T4 mildly elevated
Hypothyroidism (periorbital edema)
defective synthesis of thyroid hormone (compensatory goiter), iatrogenic, inadequate TSH or TRH
effects: myxedema, easy bruise, lethargy, confusion, agitation, decrease CO, dilated cardiomyopathy, increase PVR, constipation, anovulatory (female), erectile dysfunction & oligospermia (male)
hypothyroid (myxedema)
defective synthesis of thyroid hormone (compensatory goiter), iatrogenic, inadequate TSH or TRH
effects: myxedema, easy bruise, lethargy, confusion, agitation, decrease CO, dilated cardiomyopathy, increase PVR, constipation, anovulatory (female), erectile dysfunction & oligospermia (male)
hypothyroid (myxedema)
defective synthesis of thyroid hormone (compensatory goiter), iatrogenic, inadequate TSH or TRH
effects: myxedema, easy bruise, lethargy, confusion, agitation, decrease CO, dilated cardiomyopathy, increase PVR, constipation, anovulatory (female), erectile dysfunction & oligospermia (male)
Congenital Hypothyroidism (Cretinism)
endemic, sporadic, familial
within first weeks of life: apathy, lethargy, enlarged abdomen, decreased body temp, anemia, dilated heart, mental retardation, stunted growth
TX: TH replacement (early can prevent)
Primary Hypothyroidism
5th-6th decade, females, circulating thyroid antibodies
end stage autoimmune thyroiditis?
Hyperthyroidism
increased TSH (rare), abnormal stimulation (Graves), ectopic thyroid tissue (rare), thyroiditis before progression to hypo, Goitrous
Graves Disease (Lid Lag)
Hyperthyroidism, diffuse goiter, exophthalmos
anti-TSH receptor antibodies (Yersinia Enterocolitica?)
Histo: papillary projections of epithelium into follicles w/ scalloped borders, lymphoplasmacytic infiltrate
Symptoms: nervous, tremor, weak, weight loss, heat intolerance, palpitations, exophthalmos, Graves dermopathy
Dx: incresed Iodine uptake, elevated T3 & T4
Tx: antithyroid meds, radioactive I2, corticosteroids, adrenergic antagonists
Graves (diffusely enlarged thyroid)
Hyperthyroidism, diffuse goiter, exophthalmos
anti-TSH receptor antibodies (Yersinia Enterocolitica?)
Histo: papillary projections of epithelium into follicles w/ scalloped borders, lymphoplasmacytic infiltrate
Symptoms: nervous, tremor, weak, weight loss, heat intolerance, palpitations, exophthalmos, Graves dermopathy
Dx: incresed Iodine uptake, elevated T3 & T4
Tx: antithyroid meds, radioactive I2, corticosteroids, adrenergic antagonists
Graves (note scalloped borders of follicles)
Hyperthyroidism, diffuse goiter, exophthalmos
anti-TSH receptor antibodies (Yersinia Enterocolitica?)
Histo: papillary projections of epithelium into follicles w/ scalloped borders, lymphoplasmacytic infiltrate
Symptoms: nervous, tremor, weak, weight loss, heat intolerance, palpitations, exophthalmos, Graves dermopathy
Dx: incresed Iodine uptake, elevated T3 & T4
Tx: antithyroid meds, radioactive I2, corticosteroids, adrenergic antagonists
Graves
Hyperthyroidism, diffuse goiter, exophthalmos
anti-TSH receptor antibodies (Yersinia Enterocolitica?)
Histo: papillary projections of epithelium into follicles w/ scalloped borders, lymphoplasmacytic infiltrate
Symptoms: nervous, tremor, weak, weight loss, heat intolerance, palpitations, exophthalmos, Graves dermopathy
Dx: incresed Iodine uptake, elevated T3 & T4
Tx: antithyroid meds, radioactive I2, corticosteroids, adrenergic antagonists
Treated Graves
Toxic Adenoma
hyperfunctioning nodule
rare
Tx w/ radioactive iodine and/or surgery
Hypersecretion of TSH
thyrotropin adenoma (trophoblastic tumor)
Thyroiditis
Hashimoto's - cell mediated & humoral response against TSH receptors
Subacute (DeQuervian, Granulomatous, Giant Cell) - post URI
Silent - post partum, anti-thyroid peroxidase antibodies, resolves in months
Riedel - fibrosis
Toxic Adenoma
hyperfunctioning nodule
rare
Tx w/ radioactive iodine and/or surgery
Hypersecretion of TSH
thyrotropin adenoma (trophoblastic tumor)
Thyroiditis
Hashimoto's - cell mediated & humoral response against TSH receptors
Subacute (DeQuervian, Granulomatous, Giant Cell) - post URI
Silent - post partum
Riedel - fibrosis
Hashimoto's Thyroiditis
Cell mediated & humoral response against thyroid microsomal peroxidase, thyroglobulin, & TSH receptor, genetic predisposition
Gradual Goiter development
Hypothyroid
Hyperthyroid rare (Hashitoxicosis)
elevated TSH, circulating antibodies
Histo: lymphoplasmacytic infiltrate, destruction of follicles, Hurthle (Askanazy) cell metaplasia
Tx: Hormone Replacement
DeQuervain, Granulomatous, Giant Cell Thyroiditis (more names = more enjoyment garnered from studying)
following viral URI, granulomatous inflammation, transient hyperthyroidism w/ follicle destruction
Riedel Thyroiditis
Dense fibrosis of thyroid
associated w/ extrathyroid fibrosis (retroperitoneum, medisatinum)
histo: dense hylanized tissue w/ chronic inflammatory infiltrate
Follicular Adenoma
Most common thyroid tumor
Benign neoplasm, follicular differentiation, "cold", many histologic variants
Follicular Adenoma
Most common thyroid tumor
Benign neoplasm, follicular differentiation, "cold", many histologic variants
Thyroid Carcinoma
Most common malignant endocrine tumor
pathogenesis: Iodine excess, radiation
Papillary Carcinoma
most common variant
Histo: variable morphology, orphan annie nuclei, pseudonuclear inclusions, nuclear grooves, psammoma bodies
lymphatic spread
RET oncogene reasrrangement
Excellent prognosis
Thyroid Carcinoma
Most common malignant endocrine tumor
pathogenesis: Iodine excess, radiation
Papillary Carcinoma
most common variant
Histo: variable morphology, orphan annie nuclei, pseudonuclear inclusions, nuclear grooves, psammoma bodies
lymphatic spread
RET oncogene reasrrangement
Excellent prognosis
Follicular Carcinoma
purely follicular, resembles follicular adenoma but w/ CAPSULE INVASION
blood born mets
prognosis dependent on invasiveness
Follicular carcinoma (capsular invasion)
purely follicular, resembles follicular adenoma but w/ CAPSULE INVASION
blood born mets
prognosis dependent on invasiveness
Follicular Carcinoma (Capsular invasion)
purely follicular, resembles follicular adenoma but w/ CAPSULE INVASION
blood born mets
prognosis dependent on invasiveness
Follicular Carcinoma (bone metastasis)
purely follicular, resembles follicular adenoma but w/ CAPSULE INVASION
blood born mets
prognosis dependent on invasiveness
follicular carcinoma
purely follicular, resembles follicular adenoma but w/ CAPSULE INVASION
blood born mets
prognosis dependent on invasiveness
Follicular Carcinoma
Medullary Carcinoma
derived from C cells (hyperplasia is precursor lesion)
20% familial (MEN 2), RET oncogene
produces calcitonin (possible ACTH, glucagon, insulin, HCG, VIP, serotonin)
histo: solid sheets of polygonal granular cells w/ amyloid
Medullary Carcinoma
derived from C cells (hyperplasia is precursor lesion)
20% familial (MEN 2), RET oncogene
produces calcitonin (possible ACTH, glucagon, insulin, HCG, VIP, serotonin)
histo: solid sheets of polygonal granular cells w/ amyloid
Anaplastic Thyroid Carcinoma
Rapidly Fatal
50% have hx of longstanding goiter
many had prior lower grade neoplasms
histo: large pleomorphic cells not resembling thyroid
Lymphoma
95% B cell tumors
arise in setting of chronic thyroiditis
parathyroid
typically 4 glands
functionality shown by ratio of adipose to glandular tissue
Chief cells - PTH
clear cells - glycogen
oxyphil cells - mitochondria
Hypoparathyroidism
iatrogenic most common cause, idiopathic & familial forms
Hypocalcemia --> tingling, muscle cramps, convulsions
Tx: Ca & Vit D
PseudohypoPT - end organ resistance (Albright's Hereditary Osteodystrophy - short stature, obese, mental retardation, subcutaneous calcification, bone congenital abnormalities)
PseudopseudohypoPT - similar to Albright's w/o abnormal cAMP response to PTH
hyperparathyroidism
Increased serum Ca++, Osteitis fibrosa cystica, nephrocalcinosis (stones), polyuria, mental status change, muscle weak, peptic ulcer, pancreatitis, HTN
PT Hyperplasia, PT Adenoma, PT Carcinoma
Secondary (to renal failure) - increased PTH b/c Ca++ lost through kidneys
Tertiary - Secondary continuing after renal transplant
Osteitis Fibrosa Cystica
following hyperparathyroidism
Osteitis Fibrosa Cystica
following hyperparathyroidism
PT Hyperplasia (note decreased amount of adipose tissue)
1/3 MEN1 and 2A
1/3 show monoclonality
lack of cellular pleomorphism
symptoms: osteitis fibrosa cystica, neprocalcinosis, renal stones, polyuria, mental status changes, muscle weak, peptic ulcer, chronic pancreatitis, HTN
Parathyroid Adenoma
Sporadic or MEN1
histo: sheets of chief cells w/ rim of normal tissue
other glands atrophic (this one doing all the work)
Parathyroid Adenoma
Sporadic or MEN1
histo: sheets of chief cells w/ rim of normal tissue
other glands atrophic (this one doing all the work
Parathyroid Adenoma
Sporadic or MEN1
histo: sheets of chief cells w/ rim of normal tissue
other glands atrophic (this one doing all the work
Normal Adrenal Gland
Cortex (outershell)
Zona Glomerulosa (aldosterone)
Zona Fasiculata (cortisol)
Zona Reticularis (androgens)(dark band)
Medulla (neuroectoderm)(Epi, NE, DA)
normal adrenal
Cortex (outershell)
Zona Glomerulosa (aldosterone)
Zona Fasiculata (cortisol)
Zona Reticularis (androgens)(dark band)
Medulla (neuroectoderm)(Epi, NE, DA)
Normal Adrenal
Cortex (outershell)
Zona Glomerulosa (aldosterone)
Zona Fasiculata (cortisol)
Zona Reticularis (androgens)(dark band)
Medulla (neuroectoderm)(Epi, NE, DA)
Adrenal Hyperplasia
Congenital
21 Hydroxylase (90%) --> impaired cortisol production w/ excess androgens --> premature epiphyseal closure, virilization, salt wasting
11 beta Hydroxylase deficiency (5%) --> decreased cortisol w/ virilization, Na retention, HTN
Acute Adrenal Insufficiency
abrupt withdrawal of corticosteroid therapy
stress
Waterhouse-Fridrichsen
symptoms: HOTN, shock, abd & back pain --> pituitary damage, corticotropin deficiency (autoimmune), hyperpigmentation
Adrenal Hyperplasia
Congenital
21 Hydroxylase (90%) --> impaired cortisol production w/ excess androgens --> premature epiphyseal closure, virilization, salt wasting
11 beta Hydroxylase deficiency (5%) --> decreased cortisol w/ virilization, Na retention, HTN
Addison's Disease
Primary Adrenal Cortical Insufficiency (#1 in US, TB #1 in world)
symptoms: weakness, anorexia, skin pigmentation, HOTN, GI, personality changes, low Na, high K, lymphocytosis, elevated eosinophils
Waterhouse-Fridrichsen
meningococcal or pseudomonal septicemia w/ adrenal hemorrhage
Cushing Syndrome
Hypercorticolism
most commonly iatrogenic
Cushing Dz (pituitary adenoma realeasing ACTH)
Other paraneoplastic causes
symptoms: truncal obesity, atrophic skin, osteoposis, muscle weak, virilization, diabetes, personality changes, lymphopenia
Cushings
Hypercorticolism
most commonly iatrogenic
Cushing Dz (pituitary adenoma realeasing ACTH)
Other paraneoplastic causes
symptoms: truncal obesity, atrophic skin, osteoposis, muscle weak, virilization, diabetes, personality changes, lymphopenia
Cushings
Hypercorticolism
most commonly iatrogenic
Cushing Dz (pituitary adenoma realeasing ACTH)
Other paraneoplastic causes
symptoms: truncal obesity, atrophic skin, osteoposis, muscle weak, virilization, diabetes, personality changes, lymphopenia
Cushings
Hypercorticolism
most commonly iatrogenic
Cushing Dz (pituitary adenoma realeasing ACTH)
Other paraneoplastic causes
symptoms: truncal obesity, atrophic skin, osteoposis, muscle weak, virilization, diabetes, personality changes, lymphopenia
Adrenal Tumors
hyperplasia doesn't lead to adenoma
adenomas hormonally active
80% adrenal cortical carcinomas functions
poor prognosis
Adrenal Adenoma
adrenal adenoma
Adrenal Tumors
hyperplasia doesn't lead to adenoma
adenomas hormonally active
80% adrenal cortical carcinomas functions
poor prognosis
Adrenal Cortical Carcinoma
Adrenal Neuroblastoma
originates in adrenal medulla or SNS chain
10% childhood cancers, 15% childhood caner deaths
HSR on chromosome 2 amplifies N-myc
TRK, <2y/o, dissemination, extra adrenal origin = good prognosis
Primary Aldosteronism (Conn Syndrome)
Aldosteronomas (90%) or hyperplastic adrenal
symptoms: hypoK, HTN, weak, polyuria, polydispsia, decreased renin
Tx: surgery, Na restriction, spironolactone (aldosterone antagonist)
pheochromocytoma
sporadic or MEN2, NF1, vHL, etc --> need to rule out these etiologies in dx
histo: sheets, trabeculae, Zellballen pattern
symptoms: HTN, anxiety, hypervent, convulsions, heat intolerance, increased urinary catecholamine metabolites
Tx: surgery + adrenergic antagonists
Pheochromocytoma
sporadic or MEN2, NF1, vHL, etc --> need to rule out these etiologies in dx
histo: sheets, trabeculae, Zellballen pattern
symptoms: HTN, anxiety, hypervent, convulsions, heat intolerance, increased urinary catecholamine metabolites
Tx: surgery + adrenergic antagonists
Thymus
*next slide name the developmental abnormalities
developmental abnormalities --> immunodeficiency
Severe Combined Immunodeficiency (bubble boy) - B & T cell dysfunction
DiGeorge Syndrome - thymic, parathyroid agenesis + heart defects
Wiscott-Aldrich Syndrome - X recessive, thymic hypoplasia, eczema, thrombocytopenia
Ataxia Telangiectasia - AR, no Hassal's corpuscles or epithelial differentiation
Thymus
*next slide name the developmental abnormalities
developmental abnormalities --> immunodeficiency
Severe Combined Immunodeficiency (bubble boy) - B & T cell dysfunction
DiGeorge Syndrome - thymic, parathyroid agenesis + heart defects
Wiscott-Aldrich Syndrome - X recessive, thymic hypoplasia, eczema, thrombocytopenia
Ataxia Telangiectasia - AR, no Hassal's corpuscles or epithelial differentiation
Thymic Hyperplasia
Lymphoid follicles in thymus
myasthenia gravis in 2/3
associated w/ other autoimmune disorders
Thymoma
neoplasm of epithelial cells
10% of myasthenics
other autoimmune disorders associated
Pineal gland
melatonin major secretory product (secreted in darkness)
sleep & mating regulator?
serotonin & other peptides released
pineal
melatonin major secretory product (secreted in darkness)
sleep & mating regulator?
serotonin & other peptides released
pineocytoma
pineoblastoma
Multiple Endocrine Neoplasia
Autosomal Dominant
MEN 1 (Wermer's Syndrome) - pit adenoma, PT hyperplasia, pancreatic islet tumor
MEN 2A (Sipple's Syndrome) - 95% MEN 2, medullary thyroid carcinoma, pheochromocytoma, PT hyperplasia & adenoma, other neural crest
MEN 2B - similar to MEN2A except PT uncommon, ganglioneuromas
Genetics: RET mutation