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Endo Repro Exam I > Adrenal Pathology > Flashcards

Adrenal Pathology Flashcards

1
Q

What gross reactive changes would be expected in the case of iatrogenic Cushing syndrome from corticosteroid use?

A

Bilateral adrenal atrophy

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2
Q

What gross reactive changes would be expected in the case of ACTH dependent Cushing syndrome?

A

Bilateral cortical hyperplasia

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3
Q

Describe the further diagnostic workup of Cushing’s syndrome in the setting of low ACTH

A

In confirmed hypercortisolism with low ACTH levels, you know it is ACTH-independent aka adrenal Cushing’s

Next step is CT/MRI of abdomen to determine whether there is an adrenal adenoma/carcinoma OR bilateral adrenal hyperplasia

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4
Q

Presentation of primary hyperaldosteronism (Conn’s syndrome)

A

Typically presents as severe refractory HTN at a young age, confirmed by presence of adrenal mass

Also see hypokalemia and hypomagnesemia

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5
Q

What are some things that can cause secondary hyperaldosteronism?

A 
Diuretic use
Decreased renal perfusion
Arterial hypovolemia
Pregnancy
Renin-secreting tumors

[all are things that increase RAAS activity]

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6
Q

Size, age of onset, characteristic histologic feature, and major complication associated with aldosterone-secreting adenoma

A

May be very small (<2cm)

Young patients (age 30-40)

Histologically may see spironolactone bodies (exist in pts previously treated with this drug)

High incidence of ischemic heart disease!

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7
Q

How do you determine (based on lab workup) whether pt has primary or secondary hyperaldosterone activity?

A

Check plasma renin activity! — if it is high in the setting of high aldosterone, it is most likely secondary hyperaldosteronism

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8
Q

Pituitary vs. adrenal adrenogenital syndromes

A

Pituitary: ACTH stimulation of androgens (Cushing disease)

Adrenal: primary adrenal neoplasm (adneoma or carcinoma), congenital adrenal hyperplasia (CAH)

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9
Q

Inheritance pattern of congenital adrenal hyperplasia

A

Inherited error of metabolism — autosomal recessive!

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10
Q

Congenital Adrenal Hyperplasia (CAH) involves a defective enzyme responsible for steroidogenesis resulting in impaired feedback to the hypothalamus/pituitary, with resultant hyperplasia. 90-95% of CAH cases are caused by a deficiency in what enzyme?

A

21-hydroxylase

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11
Q

Describe syndrome caused by 21-hydroxylase deficiency

A

Salt wasting syndrome — complete lack of 21-hydroxylase results in NO mineralocorticoids and NO cortisol

Presents in males and females with hyponatremia, hyperkalemia, and hypotension. Females also show virilization at birth

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12
Q

Long term consequence of adrenomedullary dysplasia (i.e, in the setting of CAH)

A

Hypotension

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13
Q

Describe pt presentation in the case of a partial lack of of 21-hydroxylase

A

Some mineralocorticoids and some amount of cortisol, but not enough to prevent ACTH overproduction, so virilization still occurs

Most commonly presents with precocious puberty, and acne and hirsutism at the time of puberty

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14
Q

How is CAH diagnosed?

A

2 options:
Direct measurement of serum 17-hydroxyprogesterone

ACTH-stimulation test (normally would see response in glucocorticoid production with minimal effect on androgen production; in CAH the opposite occurs)

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15
Q

Tx options for CAH

A

Glucocorticoids! — replenishes cortisol AND provides negative feedback for ACTH suppression (no further overstimulation of androgen production)

Can give mineralocorticoids as necessary

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16
Q

Primary vs. secondary adrenocortical insufficiency

A

Primary: loss of cortical cells or defect in hormonogenesis

Secondary: hypothalamic-pituitary disease or HPA suppression by extra-adrenal steroid source

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17
Q

Major causes of primary acute adrenocortical insufficiency

A

Relative adrenal insufficiency (adrenal crisis)

Rapid withdrawal of steroids

Massive adrenal hemorrhage (waterhouse friderichsen syndrome)

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18
Q

Pathophysiology of exogenous steroid withdrawal

A

Steroid administration results in suppression of ACTH production by the pituitary through negative feedback

If prolonged, adrenal hypofunction or even atrophy can result

Rapid withdrawal of exogenous steroids results in adrenal insufficiency

19
Q

Causes of adrenal hemorrhage

A

Sepsis (Waterhouse Friderichsen syndrome — usually d/t Neisseria meningiditis)

Neonatal period

Trauma

Postsurgical pts

Coagulopathy

20
Q

Signs/symptoms important in recognizing ACUTE adrenal insufficiency

A

Hypotension (refractory to volume repletion)

Abdominal pain

Fever

N/V

Hyponatremia

Hypoglycemia

21
Q

Signs/symptoms of CHRONIC adrenocortical insufficiency

A

Long duration of malaise, fatigue

Anorexia and weight loss

Joint pain

Hyperpigmentation of skin

22
Q

What is Addison’s disease and what is the most common cause?

A

Primary chronic adrenocortical insufficiency

Most common cause used to be tuberculosis, but in developed countries has now shifted to autoimmune etiology

23
Q

What condition comprises >70% of all cases of primary hypoadrenalism in the western world?

A

Autoimmune adrenalitis

24
Q

Gene mutation and clinical features of autoimmune polyendocrine syndrome type 1

A

Mutation in AIRE gene

Adrenalitis
Parathyroiditis
Hypogonadism
Pernicious anemia
Mucocutaneous candidiasis
Ectodermal dystrophy
25
Q

Manifestations of autoimmune polyendocrine syndrome type 2

A

Adrenalitis

Thyroiditis

Type 1 diabetes mellitus

26
Q

Presentation of adrenocortical insufficiency

A

Lack of corticosteroids —> vague malaise, N/V, hypoglycemia, and refractory hypotension

Lack of mineralocorticoids —> hyperkalemia and hyponatremia

27
Q

Diagnosis of adrenocortical insufficiency

A

Random cortisol test

ACTH-stimulation test

28
Q

T/F: the adrenal glands are a common point of metastasis for many types of carcinoma

A

True

29
Q

Difference in presentation of adrenal cortical adenomas vs. adrenal cortical carcinomas

A

Adenomas = incidental on radiography; functional

Carcinoma = incidental on radiography; functional; ALSO show compression/invasion of adjacent structures and are virilizing

30
Q

The adrenal medulla is comprised of _____ cells deriving from the neural crest and is responsible for ________ secretion under sympathetic control

A

Chromaffin

Catecholamine (epi/norepi)

31
Q

Presentation of pheochromocytoma

A

Pts can present with profound hypertension resulting from catecholamine secretion from the tumor

25% are familial

32
Q

What is the 10% rule of pheochromocytoma?

A

10% are extra-adrenal (paraganglioma — zellballen!)

10% are bilateral

10% are in kids

10% are malignant (can only tell by metastasis)

10% are not associated with HTN

33
Q

Chronic or paroxysmal hypertension is present in >90% of cases of pheochromocytoma. What is the classic triad that also characterizes pheochromocytoma?

A

Headache
Palpitations
Diaphoresis

34
Q

Acute vs. chronic complications of pheochromocytoma

A

Acute = related to catecholamine surges

Chronic = cardiomyopathy

35
Q

How to diagnose a pheochromocytoma

A

Urine and plasma metanephrines

36
Q

Benign tumor affecting the adrenals that is made up of fat and bone marrow that varies in size and can present with hemorrhage

A

Myelolipoma

37
Q

Positive functional assays for adrenal incidentalomas

A

Dexamethasone suppression test for hypercortisolism

Pheochromocytoma

[also show CT enhancement characteristics]

38
Q

MEN type 1 is caused by a germline mutation in the MEN1 tumor suppressor gene (codes for menin). What are the clinical manifestations of this syndrome?

A

Primary hyperparathyroidism

Pancreatic endocrine tumors

Pituitary adenomas (lactotroph, somatotroph)

[3 P’s]

note, you can also see duodenal gastrinomas

39
Q

Mutation and clinical manifestations of MEN type 2A

A

Germline gain of function mutation in RET proto-oncogene

Clinically:
Pheochromocytoma
Medullary thyroid carcinoma
Parathyroid hyperplasia

40
Q

Mutation and clinical manifestations of MEN type 2B

A

Germline gain of function mutation in RET proto-oncogene (specific point mutation)

Clinically:
Medullary thyroid carcinoma
Pheochromocytoma
Mucosal neuromas

41
Q

Mutation associated with familial medullary thyroid carcinoma

A

Germline gain of function mutation in RET protooncogene (specific mutations)

42
Q

Marfan syndrome may be a clue for what type of MEN syndrome?

A

MEN2B

43
Q

Describe the pineal gland

A

Comprised of photoreceptor-containing neural tissue

Responsible for melatonin secretion

44
Q

Tumors associated with pineal gland

A

Germ cell tumors

Pineocytoma

Pineoblastoma

Endo Repro Exam I (10 decks)

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