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Endo/Repro 2 Final > 5. Robbins: Adrenal Glands > Flashcards

5. Robbins: Adrenal Glands Flashcards

1
Q

Adrenal glands are made up of the [adrenal cortex] and the [adrenal medulla].

What are the zones of the adrenal cortex.

A

Capsule

  1. Zona glomerulosa => mineralcorticoids (aldosterone); SALTY
  2. Zona fasiculata => glucocorticoids (cortisol); SWEET
  3. Zona reticularis => sex steroids (estrogens and androgens); SEX

Medulla

GO FIND REX; MAKE GOOD SEX

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2
Q

Adrenal glands are made up of the [adrenal cortex] and the [adrenal medulla].

What cells are in the adrenal medulla.

A

Chromaffin cells => catecholamines (mainly EPI)

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3
Q

Syndromes of Adrenal Hyperfunction

A
  • 1. Cushings Syndrome
  • 2. Hyperaldosteronism
  • 3. Adrenogenital or Virulizing Syndromes
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4
Q

Vast majority of Cushings syndrome is due to what?

A

Iatrogenic Cushings Syndrome = exogenous glucocorticoid administration

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5
Q

MCC of endogenous hypercorticolism?

A

ACTH-secreting pituitary adenomas (Cushings Disease)

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6
Q

What are the causes of Hypercorticolism (Cushings Syndrome)?

A
  • Primary Hyperadrenalism (ACTH-independent)
      1. Adrenal adenoma
      1. Adrenal carcinoma
  • Secondary Hyperadrenalism (ACTH-dependent)
      1. ACTH pituitary adenoma
      1. Ectopic ACTH-secreting tumor
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7
Q

What are ectopic ACTH-secreting tumors that cause Cushings syndrome?

A
  1. Small cell lung cancer *
  2. Pancreatic cancer
  3. Neural tumors
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8
Q

Cushings Syndrome => hypercortisolism

What is the effect on the adrenal glands in ACTH-dependent causes of Cushings Syndrome (Cushings disease or ectopic ACTH-producing tumor)?

A

[Bilateral Cortical Hyperplasia]

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9
Q

Bilateral atrophic adrenal glands would be expected in which variant of Cushing Syndrome?

A

“Iatrogenic” Cushing Syndrome = administration of exogenous glucocorticoids

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10
Q

What are the serum levels of cortisol and ACTH like in adrenal adenomas/carcinomas?

A

- ↑↑↑ serum cortisol

- ↓↓↓ serum ACTH

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11
Q

What are the serum levels of cortisol and ACTH like in ACTH-dependent causes of hypercortisolism?

A
  • - ↑↑↑ serum cortisol
  • - ↑↑↑ serum ACTH
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12
Q

Morphological Changes in the Pituitary Gland in Hypercorticolism

A
  • Pituitary change occur no matter the cause.
    • 1. Crook hyaline change = high levels of endogenous or exogenous glucocorticoids cause the basophilic cytoplasm of ACTH-producing cells => homogenous and pale due to accumulation of intermediate keratin filaments in cytoplasm.
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13
Q

Morphological Changes in the Adrenal Glands in Hypercortisolism

A
  • Changes depend on cause;
    • 1. Cortical atrophy
    • 2. Diffuse hyperplasia
    • 3. Macronodular or micronodular hyperplasia
    • 4. Adenoma / carcinoma
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14
Q

What causes bilateral cortical atrophy of the adrenal glands?

A

Exogenous glucocorticoids;

  • Zona fasiculata and reticularis => atrophy because they are not stimulated
  • Zona glomerulosa => NL thickness because does not respond to ACTH
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15
Q

What is macronodular hyperplasia of the adrenal glands?

A

Adrenal glands are replaced with nodules (< 3 cm) with lipid-poor + lipid-rich cells; areas between nodules have micronodular hyperplasia

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16
Q

What is micronodular hyperplasia of the adrenal glands?

A

1-3mm darkly pigmented (brown/black) micronodules made up of lipofuscin with atrophic intervening areas.

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17
Q

Both benign and malignant adrenocortical adenomas are more common in which sex and age range?

A

Women in their 30s - 50s.

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18
Q

What is a major morphological difference between adrenocortical adenomas and adrenocortical carcinomas?

A
  • Adenomas: yellow, smaller (less than 30 gm), / thin- or well-developed capsules
  • Carcinomas: MUCH large ( >300 gm), UNencapsulated masses
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19
Q

How can we determine the cause of Cushings Disease?

A

Measure:

  • 1. Serum ACTH
    1. Measure urinary steroid excretion after administration of dexamethasone.
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20
Q

Results of dexamethasone suppresion test for a patient with Cushings Disease (pituitary microadenoma)?

A
  • ACTH is elevated
  • Low dose dexamethasone test = ACTH not supressed
    • ​No reduction in urinary excretion of 17-hydroxycorticosteroids
  • High dose dexamethasone test = ACTH suppressed
    • ​Reduction in urinary excretion of 17-hydroxycorticosteroids
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21
Q

Which cause of Cushing Syndrome will have elevated levels of ACTH which is completely insensitive to low or high doses of exogenous dexamethasone?

A

Ectopic ACTH-producing tumors

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22
Q

Results of dexamethasone suppresion test for a patient with Cushing Syndrome caused by an adrenal tumor?

A

ACTH is low

  • Low dose dexamethasone test = ACTH not supressed
  • High dose dexamethasone test = ACTH not suppressed
    *
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23
Q

Hypercortisolism causes selective atrophy of fast-twitch myofibers resulting in what clinical manifestations?

A

- Decreased muscle mass

- Proximal limb weakness

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24
Q

Results of dexamethasone suppresion test for a patient with Ectopic ACTH secretion?

A

ACTH is high

  • Low dose dexamethasone test = ACTH not supressed
  • High dose dexamethasone test = ACTH not suppressed
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25
Q

What does aldosterone do?

A

Retain Na+ => retain BV/BP.

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26
Q

Causes of Primary Hyperaldosteronism

How does it affect RAAS system?

A
  1. Primary Hyperaldosteronism: ↑ aldosterone => supress RAAS (+)
    1. Bilateral Idiopathic hyperaldosteronism *** MC
    2. Neoplasm
      • Aldosterone- secreting adenoma (Conn Syndrome)
      • Adrenocorticocarcinoma
    3. Glucocorticordiod-remediable hyperaldosteronism
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27
Q

Causes of Secondary Hyperaldosteronism

A

Things that (+) RAAS system => ↑ plasma renin => ↑ aldosterone

  1. Diuretic use
  2. ↓ renal perfusion
  3. Arterial hypovolemia and edema
  4. Pregnancy (estrogen ↑ plasma renin)
  5. Renin-secreting tumors
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28
Q

MC manifestation of primary hyperaldosteronism is __________.

A

High BP

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29
Q

Primary Hyperaldosteronism due to Conn’s syndrome causes what:

A
  1. Adrenal mass (solitary aldosterone-secreting adenoma) + HTN
  2. Severe HTN (>160/100 mmHg)
  3. HTN at a young age (30-40 YO)
  4. Refractory HTN
  5. Hypokalemia and hypomagnesia
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30
Q

What is the most common underlying cause of primary hyperaldosteronism and what is seen morhphologically in the adrenal glands?

A
  • Bilateral idiopathic hyperaldosteornism (IHA)
  • Characterized by bilateral nodular hyperplasia of adrenal glands
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31
Q

Germline and somatic mutations of which gene are present in familial idiopathic hyperaldosteronism and some aldosterone-secreting adenomas?

A

KCNJ5 encoding a K+ channel

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32
Q

What is glucocorticoid-remediable hyperaldosteronism?

A

Rare cause of primary familial hyperaldosteronism and due to:

  • [ACTH-responsive CYP11B1 gene promoter] controlling [CYP11B2 (gene encoding aldosterone synthase) on Chr 8]
    • ACTH => (+) production of aldosterone synthase => ↑ aldosterone
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33
Q

In glucocorticoid-remediable hyperaldosteronism what is the function of ACTH?

A

ACTH => (+) production of aldosterone synthase => ↑ aldosterone

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34
Q

In glucocorticoid-remediable hyperaldosteronism, bc aldosterone production is controlled by ACTH, how can this production be suppressed?

A

Suppressible by dexamethasone

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35
Q

What can cause decreased renal perfusion => 2º Hyperaldosteronism?

A
  1. Renal artery stenosis
  2. Arteriolar nephrosclerosis

↓↓↓ BF kidney => think entire body needs to ↑↑↑ BF => + RAAS, even though BP is fine.

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36
Q

What can cause arteriolar hypovolemia and edema => 2º hyperaldosteronism?

A
  1. CHF
  2. Cirrhosis
  3. Nephrotic Syndrome
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37
Q

How does activity of the RAAS and levels of renin differ between primary and secondary hyperaldosteronism?

A
  • Primary= suppression of RAAS and ↓ renin
  • Secondary = activation of RAAS and ↑↑↑ renin
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38
Q

Morphology of Bilateral Idiopathic Hyperplasia

A

Diffuse and focal hyperplasia of cells in zona granulosa

- Wedge shaped: extend from [periphery => center].

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39
Q

Gross Morphology of Alderone-producing Adenomas (Conns Syndrome)

A
  • Well-cicumscribed, solitary, small (<2 cm) and buried within the gland (not visibly enlarged)
  • Bright yellow and resemble lipid-laden fasciculata cells (more than glomerulosa)
  • More often on L adrenal gland.
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40
Q

What is a characteristic histological feature of aldosterone-secreting adenomas?

A

Spironolactone bodies=> Eosinophilic, laminated cytoplasmic inclusions

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41
Q

Patients with aldosterone-secreting adenoma have a HIGH incidence of __________

A

Ischemic Heart Disease

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42
Q

Pt presents with HTN: we want to evaluate for hyperaldosteronism.

How do we diagnose?

A

1. Stop taking meds that affect plasma renin and aldosterone.

2. Measure plasma renin and aldosterone.

  • ↑↑↑ ratios of plasma aldosterone: renin activity (↑ ALD; ↓ renin) => 1º Hyperaldosteronism
    • => If +: confirm with a [aldosterone supression test]
  • ↑↑↑ plasma aldosterone AND renin (↑ ALD; ↑ renin) => 2º Hyperaldosteronism
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43
Q

Tx for primary hyperaldosteronism caused by an adenoma vs. bilateral hyperplasia?

A
    • Adenomas => surgically resect
  • - Bilateral hyperplasia => Aldosterone ANT (Spironolactone)
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44
Q

Causes of Adrenogenital Syndromes (Adrenal Virulization)

A

Pituitary causes:

  • ACTH stimulation of androgens (Cushings disease)

Adrenal gland causes:

  • Primary adrenal neoplasms (adenoma or carinoma)
  • Congenital adrenal hyperplasia (CAH)
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45
Q

What is the most common cause of adrenal virulization due to a primary adrenal neoplasm?

A

Carcinoma

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46
Q

Androgen-secreting adrenal carcinomas often secrete what other hormone?

A

Cortisol and are known as “mixed syndromes”

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47
Q

What is CAH (Congenital Adrenal Hyperplasia)?

A
  • Group of AR inherited errors of metabolism that cause a deficiency/total lack of enzymes involved in making cortical steroids, particulary cortisol.
    1. Steroid precursors build-up behind the defective step =>
    2. Channeled into other pathways =>
    3. ↓ production of gluco/mineralcorticoids & ↑ production of androgens => virulization.
    4. Impaired feedback to hypothalamus/pituitary => hyperplasia.
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48
Q

What enzyme is most commonly deficient in CAH (congenital adrenal hyperplasia)?

A

21-hydroxylase (dt mutation in CYP21A2)

  • Thus, only thing adrenal gland can produce are the sex steroids. ↓ of cortisol means that the pituitary continues to produce ACTH, resulting in ↑ sex steroids. This cycle is responsible for the virilization seen in this syndrome.
  • 17-hydroxyprogesterone (17-OHP) will spike however.
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49
Q

21-hydroxylase deficiency can cause what syndromes?

Most common***?

A
  • 1. Classic salt-wasting syndrome (complete lack)
  • 2. Simple virilizing syndrome (partial lack)
  • 3. Non-classic/late-onset adrenal virilism (partial lack) ****
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50
Q

Salt-Wasting Syndrome

  • Enzyme activity
  • Levels of glucocorticoids/mineralcorticoids/androgens
  • Symptoms and M/W
A

Salt-Wasting Syndrome

  • Complete lack of 21-hydroxylase
  • No mineralcorticoids and glucocortiods (cortisol); ↑ androgens
  • Symptoms in M/W
    • No mineralcorticoids:
        1. Salt-wasting (=> hyponatremia), hyperkalemia (↑ K+)
        1. Acidosis, Hypotension => seize, CV defects, death
    • In F => virulization is present at birth.
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51
Q

How does the presentation of salt wasting syndrome differ in males vs. females at birth?

A

- Females present EARLIER: d/t easily recognizable virilization at birth

  • Males dx 5-15 days after birth due to some salt-losing crisis
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52
Q

Patients w/ severe salt-wasting 21-hydroxylase deficiency what can happen?

A

↓ cortisol & adrenomedullary dysplasia (developmental defects of medulla) =>

  • ↓ catecholamine secretion =>
    • HYPOtension & circulatory collapse
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53
Q

Simple Virilizing Adrenogenital Syndrome (without salt wasting)

  • Enzyme activity
  • Levels of glucocorticoids/mineralcorticoids/androgens
  • Symptoms and M/W
A
  • Partial lack of 21-hydroxylase
  • Some mineralcorticoids and small cortisol, but not enough to prevent ACTH overproduction; ↑ androgens (testosterone)
  • Genital ambiguity (virilization) at a later age.
54
Q

Most common form = Nonclassic/late-onset adrenal virilism

  • Enzyme activity
  • Levels of glucocorticoids/mineralcorticoids/androgens
  • Symptoms and M/W
A
  • Partial lack of 21-hydroxylase
  • Some gluco/mineral; increase androgens
  • Symptoms in M/W
    • Virtually asymptomatic
    • Mild manifestations (Precocious puberty, acne and hirsutism @ puberty)
55
Q

Morphological Change in all cases of Congenital Adrenal Hyperplasia (CAH)

A

BILATERAL hyperplastic; some times ↑↑↑ 10-15x normal weights

56
Q
A
57
Q

Pt’s with CAH are treated with what?

A
  1. Exogenous glucocorticoids: replenishes levels AND ↓ ACTH levels via negative feedback
  2. Mineralocorticoid supplementation given in salt-wasting variants
58
Q

2 ways of diagnosis of CAH

A
  1. ↑↑↑ Serum 17-hydroxyprogesterone
  2. ACTH-stimulation test: no ↑ in glucorticoids/cortisol, but ↑ in 17-OHP.
59
Q

In what case is Serum 17-hydroxyprogesterone the highest? Lowest?

A
  • Highest = classic congenital adrenal hyperplasia
60
Q

What is mandated thoughout the US and EXTREMELY important for diagnosing bbs with CAH, who were not ID’d through family history?

A

Heel stick test

61
Q

What is Adrenocortical Insufficiency?

A

Decrease in the ability of the adrenal cortex (hypofunction) to produce steroids, especially cortisol. Can be primary or secondary causes,.

62
Q

Amyloidosis, sarcoidosis, and hemochromatosis may all lead to what type of adrenal dysfunction?

A

Primary adrenocortical insufficiency

63
Q

Primary Adrenocortical Insufficiency

A
  1. Loss of adrenal cortical tissue (or steroid producing cells)
  2. Problem making hormones
64
Q

Secondary Adrenocortical Insufficiency

A
  1. Hypothalamic - pituitary disease
  2. Exogenous steroids suppress HPA axis
65
Q

Primary acute adrenocortical insufficiency occurs in what clinical settings?

A

Causes adrenal crisis in:

    • Pt with chronic adrenocortical insufficiency, who encounter stress that causes rapid ↑ in steroid production, but adrenals cant.
    • In patients on exogenous steroids, where acute stress causes rapid withdrawl of steroids or failure to ↑ dose & atrophied adrenals can’t respond well.
    • Massive adrenal hemorrhage, occurring in newborns after long/difficult delivery; pt’s on anticoagulant therapy who develop DIC; and in Waterhouse-Friderichsen Syndrome
66
Q

If patient is taking exogenous corticosteroids, what happens to adrenals?

A

Atrophy

67
Q

What is Waterhouse-Friderichsen Syndrome characterized by?

A
  1. Bacterial infection (often due to Neisseria meningidits) => sepsis (rapidly progressing hypotension => shock) =>
  2. DIC + purpuric rash=>
  3. Massive bilateral adrenal hemorrhagic necrosis and primary acute adrenocortical insufficiency
  4. => adrenals become “sacs of clotted blood”
68
Q

Which 5 bacterial species may be associated with Waterhouse-Friderichsen Syndrome?

A
  1. - N. meningitidis
    • H. influenzae
    • Pseudomonas
    • Pneumococci
    • Staphylococci
69
Q

Histological examination of the adrenals in Waterhouse-Friderichsen syndrome show hemorrhage that starts where and then travels how?

A

Starts within medulla near thin-walled venous sinusoids => peripherally into cortex, often leaving islands of recognizable cortical cells

70
Q

What are 6 signs/sx’s that should raise suspicion of acute adrenal insufficiency?

A
  1. - HYPOtension (refractory to volume repletion)
    • HYPOnatremia/ hyperkalemia
    • HYPOglycemia
    • Abdominal pain
    • Fever, N/V
71
Q

90% of all cases of primary chronic adrenocortical insufficiency (Addison’s Disease) can be attributed to one of what 4 disorders?

A
  1. - Autoimmune adrenalitis
    • Tuberculosis
    • AIDS
    • Metastatic cancers
72
Q

Most common cause of primary chronic adrenocortical insufficiency in developed countries?

A

Autoimmune adrenalitis (APS1 or APS2)

73
Q

Most common cause of primary chronic adrenocortical insufficiency worldwide?

A

Tuberculosis —> Autoimmune

74
Q

Primary Chronic Adrenocortical Insufficiency (Addison’s Disease) Symptoms

A
  1. Joint pain
  2. No appetite (weight loss)
  3. Malaise / fatigue
  4. Hyperpigmentation (due to ACTH → αMSH)
75
Q

Autoimmune polyendocrine syndrome type 1 (APS1)

  • Mutations:
  • Affects what organs:
  • Causes:
A

Mutation in the AIRE on Chr 21q22 that affects the [adrenals, parathyroid, gonad and blood] causing

  1. Adrenalitis
  2. Parathyroiditis
  3. Hypogonadism
  4. Pernicious anemia
76
Q

What is APECED?

A

Autoimmune PolyEndocrinopathy, Candidiasis and Ectodermal Dystrophy (APECED):

When ASP1 + mucocutaneous candidiasis (Ab against IL-17/22) and ectodermal dystropgy

77
Q

What is Ectodermal dystrophy?

A

Abnormalities of skin, dental enamel, and nails

78
Q

Autoantibodies against which 2 cytokines seen in autoimmune polyendocrinopathy syndrome type 1 (APS1) are the reason for chronic mucocuntaneous infections?

A

IL-17 and IL-22

79
Q

When does autoimmune polyendocrine syndrome type 2 (APS2) usually develop and what are the characteristic findings?

A
  • Early adulthood
  • [Adrenal insufficiency (adrenalitis) + AI thyroiditis OR Type 1 DM]
80
Q

Which 3 infectious agents may cause primary chronic adrenocortical insufficiency?

A
  1. TB used to be most common cause
  2. Histoplasma capsulatum
  3. Coccidioides immitis
81
Q

AIDS patients are at ↑ risk for developing adrenal insufficiency from what?

A
  • CMV and Mycobacterium avium-intracellulare
  • Noninfectious (Kaposi Sarcoma)
82
Q

Metastatic neoplasms that affect the adrenals and causing adrenocortical insufficiency most often arise from which 2 sites; what are some other sites which may be implicated?

A
  • Lungs and breast cancer = most common
  • May also be from [GI cancers, malignant melanoma, and hematopoietic neoplasms]
83
Q

What are 2 genetic causes of adrenal insufficiency?

A
  1. - Congenital adrenal hypoplasia (CAH)
  2. - Adrenoleukodystrophy (X-linked recessive)
84
Q

When does typically Addisons Dz begin?

Initial signs and symptoms?

A
  • Insidiously and not until levels of glucocorticoids/mineralcorticoids are VERY low.
  • Progressive weakness and easy fatigability
85
Q

What are the signs/sx’s of primary chronic adrenocortical insufficiency as a result of corticosteroid and mineralocroticoid deficiency?

A
  • ↓ corticosteroids –> vague malaise, N/V, hypoglycemia, and refractory hypotension
  • ↓ mineralocorticoids –> hyperkalemia and hyponatremia
86
Q

What is the gross morphology and histology of the adrenal glands in primary autoimmune adrenalitis?

A
    • Irregularly SHRUNKEN glands
    • Scattered residual cortical cells in collapsed network of CT w/ a variable lymphoid infiltrate of the cortex
87
Q

Which type of inflammatory reaction will be seen in the adrenal glands affected by tuberculosis and fungal disease?

A

Granulomatous inflammation

88
Q

Which type of adrenocortical insufficiency is associated with hyperpigmentation of the skin and which is not?

A
  • Primary chroinc adrenal disease (Addisons) = hyperpigmentation
  • Adrenocortical insufficiency caused by primary pituitary or hypothalamic disease will not
89
Q

Dx Adrenocortical Insufficiency

A
  • 1. Random cortisol test
    • ​2º: deficient cortisol and androgens; normal or near-normal synthesis of aldosterone
  • 2. ACTH stimulation test
    • 1º: ↓ response to exogenous ACTH
    • 2º: prompt ↑ in plasma cortisol levels
90
Q

How does the presentation of adrenal carcinomas differ from that of adenoma?

A

SIZE tells us.

  • Adenoma = solid and yellow; vaculoated neoplastic cells d/t intracytoplasmic lipids
  • Carcinomas = heterogenous and red; larger (> 200 gm) and produce mass

effect causing compression/invasion of adjacent structures; more likely to be virulizing

91
Q

How do are adrenal carcinomas and adenomas SIMILAR??

A
  • Both are often incidental finding on radiograpgy or autopsy
  • Both are more often functional
92
Q

When are adrenal adenoma and carcioma more common in?

A

Equally common in adults.

Carcinoma more likely in kids.

93
Q

Functional and nonfunctional adrenocortical neoplasms CANNOT be distinguished from one another based on ________.

A

Morphology

94
Q

Adrenocortical carcinomas have a strong tendency to invade which structures?

A
  1. - Adrenal vein
  2. - Vena cava
    • Lymphatics —> regional and periaortic nodes = common
95
Q

Distant hematogenous spread by adrenocortical carcinomas to where is common; what is the prognosis of these neoplasms?

A
  • Lungs and other viscera
  • Median pt survival = about 2 years
96
Q

Which is more common: primary adrenocortical carcinomas or metastases to the adrenal cortex?

A

Metastases (esp bronchogenic origins)

97
Q

ADRENAL ADENOMA or ADRENOCORTICAL CARINOMA?

A

Adrenocortical carcinoma:

  1. Well differented cells like in adenomas => bizarre giant cell
  2. Marked anaplasia
98
Q

ADRENAL ADENOMA or ADENOCORTICAL CARCINOMA

A
  1. Vaculoated and eosinphilic neoplastic cells d/t intracytoplasmic lipids
  2. Mild nuclear pleomorphism
99
Q

Which 2 familial cancer syndromes are associated with a predisposition for developing adrenocortical carcinomas?

A
  1. - Li-Fraumeni syndrome, due to germline TP53 mutations
  2. - Beckwith-Wiedemann syndrome, a disorder of epigenetic imprinting
100
Q

If cortical and medullary neoplasms undergo necrosis and cystic degeneration => may present as ___________.

A

“Nonfunctional cysts”

101
Q

Adrenal myelolipomas are unusual benign/malignant lesions composed of what?

  • Size?
  • How do they preent?
A

benign: fat + bone marrow/HSC

  • Vary
  • With hemorrhage
102
Q

What cells make up the adrenal medulla?

A

Neural crest (neuroendocrine) cells callled Chromaffin cells + sustacular (supporting) cells.

103
Q

Adrenal Medulla

  • Under ______ control
  • Most important diseases of the adrenal medulla:
A
  • Sympathetic
  • Pheochromacytomas (neoplasm of chromaffin cells) + neuroblastic tumors
104
Q

What are Pheochromocytomas?

A

Rare neoplasms made up of chromaffin cells (which make and release catecholamines NE and EPI) & sometimes peptide hormones, often presenting with surgically correctable HTN.

105
Q

What is the dominant clinical manifestation of pheochromocytomas and what is the classic triad?

A
  • - HTN (>90%) which can be either chronic or paroxysmal
    • Triad = HA + Palpitations + Diaphoresis
106
Q

Acute and Chronic complications with Pheochromocytomas

A

Acute: CHF, MI, CVA, Pulmonary edema, Ventricular fibrillation

Chronic: Catecholamine cardiomyopathy

107
Q

What is the 10% rule associated with Pheochromocytomas (5 of them)?

A
    • 10% are extra-adrenal (paraganglioma)
    • 10% are bilateral
    • 10% in kids
    • 10% are malignant
    • 10% are NOT associated with HTN
108
Q

_____% of pheochromocytomas have germline mutations and are familial.

A

25%

109
Q

3 groups of extra-adrenal paraganglia based on anatomic distribution

A
  • 1. Branchiomeric
  • 2. Intravagal
  • 3. Aorticosympathetic
110
Q

4 familial syndromes associated with pheochromocytomas and extra-adrenal paragangliomas

A
  1. MEN-2A
  2. MEN-2B
  3. NF-1
  4. Von Hippel-Lindau (VHL)
111
Q

How does the patient presentation and location of pheochromocytomas in pt’s with familial pheochromcytoma (d/t germline mutations) differ from that of sporadic types?

A

Pts with familial mutations are:

  1. Younger
  2. Bilateral disease
112
Q

Histological pattern in pheochromocytomas

A
  1. Nests (zellballen) of polygonal to spindle-shaped chromaffin or chief cells surrounded by sustentacular cells, supplied by rich vascular network
  2. Granular cytoplasm that contains catecholamines
113
Q

What stains can we use to view cytoplasm and sustenacular cells in a pheochromacytoma?

A
  • Cytoplasm (catecholamines) = silver stain
  • Sustentacular cells = antibodies against S-100
114
Q
  • Which histological feature of pheochromocytomas reliably predicts clinical behavior?
  • What is the definitive diagnosis of malignancy in pheochromocytomas based on?
A
  • Nothing histologically predicts clinical behavior.
  • ONLY on presence of metastasis
115
Q

What is the laboratory diagosis of pheochromocytomas based on?

A

↑ urinary excretion and plasma free catecholamines + their metabolites i.e., vanillylmandelic acid and metanephrines

116
Q

What 3 features of adrenal incidentalomas impact the appropriate managment of the mass?

A
    • SIZE: >4cm = more likely to be carcinoma
  2. - Positive functional assays: i.e., dexamethasone suppression test for hypercortisolism or urinary metanephrines for pheochromo.
  3. - CT enhancement characteristics
117
Q

What is MEN syndrome?

A

Group of inherited diseases that results in [hyperplasia, adenoma and carcinomas] of multiple endocrine organs

118
Q

How do endocrine tumors due MEN syndromes differ from their sporadic counterparts in terms of age, organs involved, behavior, and foci?

A
    • Tend to occur at younger age
    • Arise in multiple endocrine organs, either synchronously or metachronously
    • Typically preceded by asymptomatic stage of hyperplasia pre-cursor lesions
    • Even in one organ, tumors are often multifocal and bilateral
    • Are usually more aggressive and recur
119
Q

_MEN1 (aka _____)_

  • Mutation
  • Causes what? (in order of MC)
A
  • Werner Syndrome
  • Germline mutation in MEN1 (Menin) tumor supressor
  • 3 Ps:
    1. ​Primary _hyper_parathyroidism (parathyroid adenoma or primary parathyroid hyperplasia)
    2. Pancreatic endocrine tumors (Insulinoma, Gastrinoma, Somatostatinoma, Glucagonoma, VIPoma, also DUODENAL gastrinomas, as well as pancreatic gastrinomas)
    3. Pituitary adenomas (prolactinoma = MC)
120
Q

What is the initial manifestation of MEN 1 in most patients, appearing in almost all patients by 40-50 YO?

A

Primary hyperparathyroidism

121
Q

Which tumors of MEN-1 are th_e leading cause of morbidity_ and mortality due to their aggressiveness and metastases?

A

Pancreatic endocrine tumors

122
Q

What is the most frequent pituitary adenoma encountered in MEN-1?

A

Prolactinoma (lactotroph); may also see somatotroph adenoma (GH) => acromegaly

123
Q

What is the most common site of gastrinomas present in pt’s with MEN-1?

A

Duodenum; may also have synchronous duodenal + pancreatic tumors

124
Q

MEN-2A (aka)

  • Mutation:
  • Triad:
A
  • Sipple Syndrome
  • Germline GOF in the RET proto-oncogene on _Cr. 10_q11.2
  • Triad (3 C’s)
    1. Medullary thyroid cancer => ↑ calcitonin
    2. ​Pheochromocytoma => ↑ catecholamines
    3. Parathyroid hyperplasia => ↑ calcium (hypercalcemia) and kidney stones
125
Q
  1. Which tumor is seen in almost 100% of patients with MEN-2A?
  2. Medullary thyroid carcinoma in MEN-2A are usually ____ and are virtually always associated with what?
A
  1. Medullary thyroid cancer
    1. Multifocal; C-cell hyperplasia in the adjacent thyroid
126
Q

MEN-2B

  • Mutation
  • Causes
A

Point mutation in RET gene

Causes

  1. Pheochromocytomas
  2. Medullary thyroid cancer (multifocal and more aggressive)
  3. Mucosal neuromas (in mouth, eyes, respiratory/GI tract)
  4. Marfainoid habitus
127
Q

Familial medullary thyroid cancers are a variant of MEN-2A; how are they different?

A
    • Develop at an older age
  • - No other clinical manifestation assoc. w/ MEN-2A
    • Typically follow a more indolent course
128
Q

About 1/3 of sporadic medullary thyroid carcinomas have the identical point mutation of RET encountered in MEN-2B and follow what type of course?

A

More aggressive disease and adverse prognosis

129
Q

Diagnosis via screening of at-risk family members for which MEN syndrome is important?

A

MEN-2A due to medullary thyroid carcinoma being life-threatening, and can be prevented by prophylactic thyroidectomy

130
Q

The pineocytes of the pineal gland are epithelial cells with what 2 functions?

A

Photosensory and neuroendocrine functions

131
Q

Majority of tumors arising in the pineal gland are of what type?

A
  • Germ cell tumors i.e., germinomas, embryonal carcinomas; choriocarcinomas and mixtures of the 2

Endo/Repro 2 Final (11 decks)

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