Adrenal Gland Pathology Flashcards
1
Q
What does the cortical zone look like?
A
- Yellow color
2
Q
What are the components of the cortical zone (from out to in)?
A
- Zona glomerulosa
- Zona fasciculata
- Zona reticularis
3
Q
What does the zona glomerulosa secrete?
A
- Aldosterone
4
Q
What does the zona fasciculata secrete?
A
- Cortisol
5
Q
What does the zona reticularis secrete?
A
- Weak androgens
6
Q
What does the medulla secrete?
A
- Catecholamines
7
Q
What does the medulla look like?
A
- Dark brown
8
Q
What do the adrenals look like in ACTH dependent cushing syndrome?
A
- Bilateral cortical hyperplasia
9
Q
What are the symptoms of primary hyperaldosteronism (Conn’s syndrome)?
A
- Hypertension
- Hypokalemia
- Hypomagnesemia
10
Q
What is seen in the hypertension with primary hyperaldosteronism?
A
- Refractory hypertension
- Adrenal mass and hypertension
- Hypertension at a young age
- Severe hypertension
11
Q
What are some things that disrupt the RAAS causing secondary hyperaldosteronism?
A
- Diuretic use
- Decreased renal perfusion
- Arterial hypovolemia
- Pregnancy
- Renin-secreting tumors
12
Q
What can be used to treat hypertension in an aldosterone-secreting adenoma?
A
- Spironolactone
13
Q
What is congenital adrenal hyperplasia?
A
- Inherited error of metabolism (AR)
- Defective enzyme responsible for steroidogenesis
- Impaired feedback to hypothalamus/pituitary, with resultant hyperplasia
14
Q
What enzyme is deficient in a majority of cases of congenital adrenal hyperplasia?
A
- 21-hydroxylase
15
Q
What is salt wasting syndrome?
A
- Complete lack of 21-hydroxylase enzyme
- No mineralocorticoids
- No cortisol
16
Q
What is the presentation that is shared in both males and females for salt wasting syndrome?
A
- Hyponatremia
- Hypokalemia
- Hypotension
17
Q
What is a presentation that is seen in only females with salt wasting syndrome?
A
- Virilization (seen at birth)
18
Q
What is a long term consequence of adrenomedullary dysplasia?
A
- Hypotension
19
Q
What is simple virilizing syndrome?
A
- Partial lack of 21-hydroxylase enzyme
- Some mineralocorticoids
- Small amounts of cortisol, not enough to prevent ACTH overproduction
20
Q
What is seen in simple virilizing syndrome?
A
- Virilization
21
Q
What is nonclassic/late onset adrenal virilism?
A
- Partial lack of 21-hydroxylase (more than simple virilizing syndrome)
22
Q
What is seen in nonclassic/late onset adrenal virilism?
A
- Precocious puberty
- Acne and hirsutism at time of puberty
23
Q
How is the diagnosis of CAH made?
A
- Look at serum 17-hydroxyprogesterone
24
Q
What is the treatment for CAH?
A
- Give glucocorticoids
- It replenishes cortisol
- Provides negative feedback for ACTH suppression
25
What are some causes of primary adrenocortical insufficiency?
- Loss of cortical cells
| - Defect in hormonogenesis
26
What are some causes of secondary adrenocortical insufficiency?
- Hypothalamic-pituitary disease
| - HPA suppression by extra-adrenal steroid source
27
What are some causes of primary acute adrenocortical insufficiency?
- Adrenal crisis
- Rapid withdrawal of steroids
- Massive adrenal hemorrhage (Waterhouse-Friderichsen syndrome)
- Stress, infection, trauma, burns
28
What happens to the adrenal glands on corticosteroids?
- Atrophy
29
What are the clinical symptoms of acute adrenal insuffiency?
- Hypotension
- Abdominal pain
- Fever
- N/V
- Hyponatremia
- Hypoglycemia
30
What are some symptoms of primary chronic adrenocortical insuffiency?
- Long duration of malaise, fatigue
- Anorexia and weight loss
- Joint pain
- Hyperpigmentation of skin
31
What was the most common cause of Addison's disease when it was initially found?
- TB
32
What is the most common cause of Addison's disease in developed countries and worldwide?
- Autoimmune
33
What causes autoimmune polyendocrine syndrome type 1?
- Mutation in AIRE gene
34
What is seen in autoimmune polyendocrine syndrome type 1?
- Adrenalitis
- Parathyroiditis
- Hypogonadism
- Pernicious anemia
35
What is seen in autoimmune polyendocrine syndrome type 2?
- Adrenalitis
- Thyroiditis
- Type 1 diabetes mellitus
36
What is apeced?
- Has same symptoms of autoimmune polyendocrine syndrome type 1
- Also has misshapen teeth and nail beds
37
What are individuals with apeced more likely to have?
- Mucocutaneous candidiasis
| - Ectodermal dystrophy
38
What are some ways to check for adrenocortical insufficiency?
- Random cortisol
| - ACTH-stimulation test
39
What is the size that signifies it is a carcinoma?
- >200 grams
40
What are some features that are more commonly seen with carcinoma?
- Invasion of adjacent structures
| - Virilization
41
What is the adrenal medulla derived from?
- Comprised of chromaffin cells deriving from the neural crest
42
What is the adrenal medulla responsible for?
- Catecholamine secretion
| - Epi and Norepi
43
What is a pheochromocytoma?
- Patients can present with profound hypertension
| - Results from catecholamine secretion from the tumor
44
What is the 10% rule with pheochromocytoma?
- 10% are extra-adrenal
- 10% are bilateral
- 10% are in kids
- 10% are malignant
- 10% are not associated with hypertension
45
What is the 25% rule with pheochromocytoma?
- 25% are familial
46
What is the presentation of a pheochromocytoma?
- Hypertension is present in >90% of cases
- Headache
- Palpitations
- Diaphoresis
47
What are some complications with pheochromocytomas?
- Acute: related to catecholamine surges
| - Chronic: cardiomyopathy
48
How is a pheochromocytoma diagnosed?
- Urine and plasma metanephrines
49
What is a myelolipoma?
- Benign (fat or bone marrow)
- Vary in size
- Can present with hemorrhage
50
What is an adrenal incidentaloma?
- Incidentally discovered adrenal nodule
51
What is done for an adrenal incidentaloma?
- CT enhancement characteristics
- Positive functional assays ( Dexa suppression test or pheochromocytoma)
- Look at size
52
What comprises MEN type 1?
- Primary hyperparathyroidism
- Pancreatic endocrine tumors
- Pituitary adenomas (lactotrophs and somatotrophs)
53
What is the germline mutation in MEN type 1?
- MEN tumor suppressor gene (menin)
54
What comprises MEN type 2A?
- Pheochromocytoma
- Medullary thyroid carcinoma
- Parathyroid hyperplasia
55
What germline gain of function is seen in MEN type 2A?
- RET proto-oncogene
56
What comprises MEN type 2B?
- Medullary thyroid carcinoma
- Pheochromocytoma
- Mucosal neuromas
57
What germline gain of function is seen in MEN type 2B?
- RET proto-oncogene
58
What are some generalities of MEN syndromes?
- Patients develop tumors at younger ages
- Tumors are more likely to be bilateral/multiple
- Preceding hyperplasia is often seen
- Tumors tend to be aggressive and reccurent
59
What are some tumors of the pineal gland?
- Germ cell tumors
- Pineocytoma
- Pineoblastoma
60
What is the pineal gland responsible for?
- Melatonin secretion
