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STEP 1 (2) > Endocrine Pathology > Flashcards

Endocrine Pathology Flashcards

1
Q

What causes Cushing syndrome?

A

increased cortisol levels due to:

  • exogenous corticosteroids that result in decreased ACTH and bilateral adrenal insufficiency (most common cause)
  • primary adrenal adenoma, hyperplasia, or carcinoma resulting in decreased ACTH, and atrophy of the uninvolved adrenal gland
  • ACTH secreting pituitary adenoma (aka Cushing disease) or paraneoplastic ACTH secretion from a cancer both resulting in increased ACTH and bilateral adrenal hyperplasia
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2
Q

What cancers most commonly cause paraneoplastic ACTH secretion?

A

small cell lung cancer

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3
Q

What are the symptoms of Cushing syndrome?

A
  • HTH, weight gain
  • moon facies
  • truncal obesity and striae
  • buffalo hump
  • osteoporosis
  • hyperglycemia
  • amenorrhea
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4
Q

How should Cushing syndrome be diagnosed?

A

Measure ACTH. If decreased (less than 5pg/mL), suspect an adrenal tumor. If elevated over 20 pg/mL then it is an ACTH-dependent Cushing syndrome. From there, do a high-dose dexamethasome suppression test (HDDST) and a CRH stimulation test.

If the HDDST does not cause suppression it is an ectopic ACTH secretion, and if it does, it is Cushing disease

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5
Q

What is adrenal insufficiency?

A

the inability of the adrenal glands to generate enough glucocorticoids +/- mineralocorticoids resulting in symptoms including weakness, fatigue, orthostatic hypotension, muscle aches, weight loss, GI disturbances, and sugar and/or salt cravings

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6
Q

What is a metyrapone stimulation test?

A

metyrapone blocks the conversion of 11-deoxycortisol to cortisol so the normal response is decreased cortisol and compensatory increase in ACTH. In adrenal insufficiency, ACTH remains depressed after the test

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7
Q

Describe primary adrenal insufficiency

A

this is a deficiency of aldosterone and cortisol production due to loss of gland function leading to hypotension, hyperkalemia, metabolic acidosis, and skin and mucosal hyperpigmentation due to MSH, a byproduct of increased ACTH production from POMC

the two main types are acute and chronic

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8
Q

What are the main causes of acute adrenal insufficiency?

A

hemorrhage

ketoconazole use

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9
Q

What are the main causes of chronic adrenal insufficiency (aka Addison disease)?

A
  • autoimmunity
  • TB
  • metastasis
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10
Q

Acute primary adrenal insufficiency due to adrenal hemorrhage associated with Neisseria meningitidis is known as _______

A

Waterhouse-Friderichsen syndrome

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11
Q

Describe secondary adrenal insufficiency

A
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12
Q

What is the most common pediatric adrenal tumor?

A

a neuroblastoma, of the adrenal medulla

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13
Q

What causes neuroblastomas?

A

tumor of the sympathetic chain in the adrenal medulla in children usually under 4 yo

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14
Q

How does a neuroblastoma present clinically?

A

abdominal distension and a firm, irregular mass

may also have opsoclonus-myoclonus syndrome (aka ‘dancing eyes-dancing feet’)

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15
Q

What are these?

A

Homer-Wright bodies seen in neuroblastomas

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16
Q

How are neuroblastomas diagnosed?

A

elevated urine HVA (breakdown product of dopamine) and VMA (breakdown produt of nor)

bombesin and neuron-specific enolase +

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17
Q

What mutation is common in neuroblastomas?

A

N-myc

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18
Q

What is the most common adrenal medullar tumor in adults?

A

pheochromocytoma

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19
Q

Where do pheos come from?

A

chromaffin cells, derived from the neural crest

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20
Q

What is the ‘Rule of 10s’ with pheos?

A

10% are

  • malignant
  • bilateral
  • extra-adrenal
  • calcify
  • are in kids
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21
Q

What are the symptoms of a pheo?

A

Episodic hyperadrenergic symptoms (5P’s):

Pressure (increased BP)

Pain (HA)

Perspiration

Palpitations

Pallor

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22
Q

What are some associations of pheos?

A
  • neurofibromatosis type I
  • VHL disease
  • MEN 2A and 2B
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23
Q

How are pheos tx?

A

irreversible a-antagonists (e.g. phenoxybenazine) followed by B-blockers prior to tumor resection

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24
Q

How does hypothyroidism present clinically?

A
  • cold intolerance (decreased heat production)
  • facial/periorbial myxedema
  • weight gain and decreased appetite (BMR decreased)
  • fatigue, weakness, lethargy
  • constipation
  • areflexia
  • bradycardia
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25
Q

How does hypothyroidism present in labs?

A

-increased TSH (in primary)

decreased free T3/T4

hypercholesterolemia due to decreased LDL receptor expression

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26
Q

How does hyperthyroidism present clinically?

A

heat intolerance

weight loss and increased appetite

hyperactivity

diarrhea

increased reflexes

pretibial myxedema

warm, moist skin

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27
Q

What are some main causes of hypothyroidism?

A
  • Hashimoto thyroidits
  • Congenital hypothyroidism (cretinism)
  • Subacute thyroiditis (de Quervain)
  • Riedel thyroiditis
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28
Q

Describe Hashimoto thyroiditis

A

this is the most common cause of hypothyroidism in iodine-sufficient regions and is an autoimmune disorder marked by Abs against thyroid peroxidase that may present as hyperthyroidism early in its course due to thyrotoxicosis following follicular rupture

and typically has an enlarged, nontender thyroid

associated with HLA-DR5 and increased risk of non-Hodgkin lymphoma

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29
Q

What are the histo findings of Hashimoto thyroiditis?

A

-Hurthle cells, lymphoid aggregates with germinal centers

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30
Q

How does cretinism present?

A

-pot-bellied, pale,

umbilical hernia

protuberant tongue

poor brain development

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31
Q

What is Subacute thyroiditis?

A

a self-limited disease following flu that may be hyperthyroidism early in its course but transitions to hypothyroidism and is associated with a tender thyroid and jaw pain

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32
Q

What is Riedel thyroiditis?

A

this occurs when the thyroid is replaced by fibrous tissue (hypothyroid), and fibrosis may extend to local strucures mimicking anaplastic carcinoma. Considered a manifestation of IgG4-related systemic disease

the thyroid is hard, rock like, and painless

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33
Q

What are the main causes of hyperthyroidism?

A
  • Graves disease
  • toxic multinodular goiter
  • thyroid storm
  • Jod-Basedow phenomenon
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34
Q

Describe Graves disease

A

This is the most common cause of hyperthyroidism caused by autoantibodies that stimulate TSH receptors on the thyroid and manifesting as protuberant eyes and pretibial myxedema

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35
Q

Toxic multinodular goiter is when focal patches of hyperfunctioning thyroid cells arise independent of TSH due to mutations in the TSH receptor (hot nodules are rarely malignant)

A
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36
Q

What causes a thyroid storm?

A

stress-induced catecholamine surge seen in chronic disease, presenting as agitation, confusion, fever, diarrhea, coma, and tachyarrhythmias

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37
Q

How is thyroid storm tx?

A

BBs (e.g. Propranolol), Propylthiouracial, and corticosteroids (eg. Prednisolone)

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38
Q

What is Jod-Basedow phenomenon?

A

thyrotoxicosis when a pt with iodine deficiency goiter is made iodine replete

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39
Q

What are some complications of thyroid removal?

A

hoarseness (recurrent laryngeal n. damage)

hypocalcemia (if the parathyroids are damaged)

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40
Q

What are the main types of thyroid cancer?

A
  • Papillary carcinoma
  • Follicular carcinoma
  • Medullary carcinoma
  • Anaplastic carcinoma
  • Lymphoma
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41
Q

Describe papillary carcinomas

A

these are the most common and have the best prognosis that spread via lymph and are marked histologically by:

  • empty appearing nuceli with central clearing (aka Orphan Annie eyes) (below)
  • psamomma bodies
  • nuclear grooves
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42
Q

What mutations are common in papillary carcinomas of the thyroid?

A

RET and BRAF

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43
Q

Describe medullary carcinomas

A

these arise from C cells and produce calcitonin, and sheets of cells in an amyloid stroma (below). These spread via heme

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44
Q

Medullary carcinoma of the thyroid is seen in _______

A

MEN 2A and 2B

45
Q

Thyroid lymphoma is a common sequelae of ______

A

Hashimoto thyroiditis

46
Q

What are the most common causes of hypoPTHism?

A
  • accidental removal surgical excisions,
  • autoimmune destruction
  • DiGeorge syndrome
47
Q

What is Chvostek sign?

A

tapping of the facial nerve (cheek) causes contraction of facial muscles in hypoPTHism

48
Q

What is Trousseau sign?

A

occlusion of the brachial a. with a BP causes carpal spasm

49
Q

Unresponsiveness of the kidney to PTN resulting in hypocalcemia, shorteneded 4th/5th digits, and shoft stature is known as ______

A

pseudo-hypoPTHism

50
Q

What causes familial hypocalciuric hypercalcemia?

A

a defective Ca2+ sensing receptor of parathyroid cells leading to elevated PTH even in the face of elevated calcium

51
Q

What are the main causes of primary hyperPTHism?

A

-parathyroid adenoma or hyperplasia

52
Q

What are the lab findings of primary hyperPTHism?

A

hypercalcemia, hypercalciuria (renal stones), hypophosphatemia, elevated PTH

elevated ALP, and cAMP in urine

53
Q

How does hyperPTHism present?

A

most often asymptomatic but can present with:

bones (bone pain), stones (kidney stones), groans (constipation), and moans (depression)

54
Q

What is osteitis fibrosa cystica?

A

the formation of cystic bone sapces filled with brown fibrous tissue that causes bone pain

55
Q

What are the main causes of secondary hyperPTHism?

A

decreased calcium or vitD, or elevated phosphate, most often occurring in chronic kidney disease

56
Q

What is the most common pituitary adenoma?

A

prolactinoma (benign)

57
Q

How can pituitary adenomas present?

A

they may be fucntional (hormone producing) or nonfunctional (silent). Nonfunctional tumors present with mass effect including bitemporal hemianopia, hypopituitarism, and HA

a functional prolactinoma would present with amenorrhea, galactorrhea, low libido, inferility

58
Q

What is the tx of a prolactinoma?

A

-dopamine agonists like bromocriptine or cabergoline

59
Q

Acromegaly increases the risk of what?

A

colorectal polyps and cancer

60
Q

What is the most common cause of death with gigantism?

A

HF

61
Q

How is acromegaly diagnosed?

A

increased IGF-1

failure to suppress serum GH following an oral glucose tolerance test

62
Q

What is diabetes insipidus?

A

failure to concentrate urine due to failure of secretion (central) or binding of ADH (nephrogenic)

63
Q

What are the main causes of central DI?

A

-pituitary tumor, trauma, surgery, idiopathic

64
Q

What are the lab findings of central DI?

A
  • decreased ADH
  • urine SG less than 1.006

serum osmolarity over 290 mOsm/kg

65
Q

What are the main causes of nephrogenic DI?

A

-hereditary (ADH receptor mutation)

secondary to hypercalcemia, lithium, demeclocycline (ADH antagonist)

66
Q

How can central and nephrogenic DI be differentiated?

A

central will show a 50+% increase urine osmolarity after administration of an ADH analog (desmopressin acetate) while nephrogenic will not

67
Q

How is central DI tx?

A

intranasal desmopressin acetate (ADH analog)

Hydration

68
Q

How is nephrogenic DI tx?

A

HCTZ, indomethacin, amiloride

Hydration

69
Q

Describe SIADH

A

syndrome of:

  • excessive free water retention
  • Euvolemic hyponatremia with continued urinary na+ excretion
  • urine osmolarity > serum osmolarity
70
Q

What are the complications of SIADH?

A

the body responds to water retention by decreasing aldosterone from the adrenal glands. Thre resultant hyponatremia can lead to cerebral edema and seizures

Must correct slowly to prevent osmotic demyelination syndrome

71
Q

What are the main causes of SIADH?

A
  • Ectopic ADH (e.g. small cell lung cancer)
  • CNS disorders/head trauma
  • pulmonary disease
  • drugs (cyclophosphamide)
72
Q

How is SIADH tx?

A

-demeclocycline

-vaptans

  • fluid restriction
  • IV hypertonic saline
73
Q

What are the main causes of hypopituitarism?

A
  • nonsecreting pituitary adenoma, craniopharyngioma
  • Sheehan syndrome
  • Empty sella syndrome
  • Pituitary apoplexy
  • brain radiation, injury
74
Q

What is Sheehan syndrome?

A

ischemic infract of the pituitary following postpartum bleeding presenting with the classic triad of:

-failure to lactate

-absent menstruation

-loss of pubic hair

and cold intolerance

75
Q

What is a major cause of empty sella syndrome?

A

obesity compressing the pituitary

76
Q

What is pituitary apoplexy?

A

sudden hemorrhage, often in the presence of an existing adenoma

77
Q

What is a craniopharyngioma?

A

a type of brain tumor derived from pituitary gland embryonic tissue, that occurs most commonly in children but also in men and women in their 50s and 60s. People may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopsia, as the tumor may compress the optic chiasm.

78
Q

Describe the pathophysi of diabetes mellitus

A

insulin deficiency or resistance (and/or glucagon excess) leads to decreased glucose uptake and increased proteolysis and lipolysis all promoting hyperglycemia leading to dehyration and acidosis

79
Q

What are the symptoms of DM?

A

-polydipsia, polyuria, polyphagia

DNA (type I)

hyperosmolar coma (type II)

80
Q

What are some chronic complications of diabetes?

A

nonezymatic glycation leading to:

  • small vessel diffuse thickening of BM leading to retinopathy (hemorrhage, exudates, microaneurysms)
  • nephropathy (Kimmelstiel-WIlson nodules)
  • large vesse; atherosclerosis, CAD, gangrene, occlusive disease
81
Q

What is the most common cause of death in DM?

A

MI

82
Q

Seen in DM nephropathy

A
83
Q

Omsotic damage in DM can also cause neuropahty and/or cataracts. How does this occur?

A

sorbitol accumulation in organs with aldose reductase and decreased or absent sorbitol dehydrogenase

84
Q

Describe type I Diabetes

A

cause: autoimmune destruction of B cells

insulin is always needed for tx

severe glucose intolerance

insulin resistance less common

85
Q

What are the HLA associations of type I DM?

A

HLA-DR3/4 (less genetic predispostion than type II)

86
Q

Islet leukocytic infiltrate (below) of IOL is a characteristic finding of what _____

A

Type I DM

87
Q

What are the HLA associations of Type II DM?

A

None but more genetic disposition than type I

88
Q

What is the classic histo finding in the IOL in Type II diabetes?

A

amyloid deposits

89
Q

What causes DKA?

A

Commonly seen only in Type I DM due to excessive fat breakbown (lipolysis) and ketogenesis from increased circulating free fatty acids (B-hydroxybutyrate > acetoacetate)

NOTE: Only seen in type I becuase insulin prevents lipolysis

90
Q

What are the signs and symptoms of DKA?

A
  • Kussmaul respirations (rapid/deep breathing)
  • fruity breath

N/V

abdominal pain

dehydration

delirium

91
Q

What are the lab findings of DKA?

A

hyperglycemia

acidosis and decreased HCO3- (increased anion gap metabolic acidosis)

leukocytosis

-kyperkalemia, but depleted total body K+ (due to extracellular shift)

92
Q

What are the major complications of DKA?

A
  • Rhizopus mucormycosis
  • cerebral edema
  • cardiac arryhthmia or HF
93
Q

How does a glucagonoma present?

A

Tumor of pancreatic a cells causing overproduction of glucagon presenting as the four D’s:

dermatitis, diabetes, DVT, and depression

94
Q

Describe insulinomas

A

tumor of B cells causing overproduction of insulin leading to hypoglycemia

95
Q

How might insulinomas present?

A

Whipple triad:

hypoglycemia

lethary, syncope, diplopia (symptoms of hypoglycemia)

resolution of symptoms after normalization of BG

96
Q

What is carcinoid syndrome?

A

syndrome seen in metastatic carcinoid tumors caused by secretion of serotonin that results in bouts of diarrhea, cutaneous flushing, wheezing, HTN, and right-sided valvular disease

97
Q

What are the lab findings of Carcinoid syndrome?

A

increased urine 5-HIAA

niacin deficiency (pellagra)

98
Q

What is Pellagra?

A

A vitamin deficiency disease most frequently caused by a chronic lack of niacin (vitamin B3 or vitamin PP, from pellagra-preventing factor) in the diet. It can be caused by decreased intake of niacin or tryptophan, and possibly by excessive intake of leucine. It may also result from alterations in protein metabolism in disorders such as carcinoid syndrome or Hartnup disease. A deficiency of the amino acid lysine can lead to a deficiency of niacin, as well

99
Q

What is the Rule of 1/3rds in Carcinoid syndrome?

A

1/3:

metastastize

present with a 2nd malignancy

are multiple

100
Q

What is Zollinger-Ellison Syndrome?

A

Gastrinoma of the pancreas or duodenum causing acid hypersecretion leading to recurrent ulcers, abdominal pain, and diarrhea

101
Q

How is ZES diagnosed?

A

paradoxical increase in gastrin levels following adminstration of secretin, which normally inhibits gastrin

102
Q

All MEN syndromes have _____ MOI

A

AD (All MEN are dominant)

103
Q

What are the components of MEN1?

A
  • Parathyroid tumor
  • Pituitary tumors (proalctin or GH)
  • Pancreatic endocrine tumor (ZES, insulinomas, VIPomas, glucagonomas)
104
Q

What is the cause of MEN 1?

A

mutation of MEN1 gene (menin, a tumor suppressor)

105
Q

What causes MEN2A AND MEN2B?

A

RET gene

106
Q

What are the components of MEN2A?

A
  • Parathyroid hyperplasia
  • Pheochromocytoma

Medullary thyroid carcinoma (Secretes calcitonin)

107
Q

What are the components of MEN 2B?

A
  • Pheochromocytoma
  • Medullary thyroid carcinoma (secretes calcitonin)
  • Oral/intestinal ganglioneuromatosis (mucosal neuromas) (below)
108
Q

MEN 2A and 2B are associated with _______

A

marfanoid habitus

STEP 1 (2) (16 decks)

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