Adrenal Gland Pathology Dr. Singh

Question 1

Cortex of adrenal gland has wha layers ad secretes what

Answer 1

1. Zona Glomerulosa (Aldosterone) salt outer
2. Zone Fasciculata (Cortisol) sweet middle
3. Zona Reticularis (androgens) sex inner

Question 2

Medulla of adrenal gland has wha layers ad secretes what

Answer 2

Chromaffin Cells = NE and Epi (catacholamines)

Question 3

Adrenal hypercortisolism ACTH dependent + independent

Answer 3

1. ACTH dependent = secondary cause, primary AP adenoma or ectopic
2. ACTH independent = primary adrenal tumor or problem secreting too much Cortisol

Question 4

ACTH dependent Cushing syndrome cause what to happen to the adrenal glands

Answer 4

Bilateral Cortical Hyperplasia

Question 5

Primary hyperadrenalism ACTH independent causes what to happen to the gland

Answer 5

Cortical adenoma , yellow round mass

Question 6

Giving high dose dexamethasone will suppress cortisol when and when will it not

Answer 6

1. Will suppress it : AP microadenoma (cushings disease)

2. Will not suppress it : Adrenal primary tumor or hyperplasia, + Ectopic ACTH (corticotropin)

Question 7

Conn’s Syndrome :

1. From what
2. SX
3. Most common cause + 2 other causes

Answer 7

1. High Aldosterone
2. = HTN (severe over 160/100, refractory, young age)**
   = Adrenal mass
   = hypokalemia + HypoMg (not always seen)
3. Idiopathic : glomerular cells secrete too much aldosterone (others : high ACTH driven, adenoma)

Question 8

Secondary Hyperaldosteronism :

1. Caused by what and causes what

Answer 8

1. Anything activating renin-angiotensin - aldosterone system
   (Diuretics, low renal perfusion, arterial hypovolemia, Pregnancy, Renin secreting tumors)
   Low renal perfusion (like sclerosis = makes BV through renal capsules low) = makes body think you’re Hypovolemic

Question 9

Hyperaldosteonism with low renin and high aldosterone what do you do

Answer 9

1. Adrenal CT for any adrenal tumors (usually see in young pts 20-30yo)
2. Tx HTN with aldosterone specific antagonists : Spironolactone (histology will show spironolactone bodies if pt was on this)

Question 10

Adrenal Virilization

1. what makes this happen

Answer 10

1. high ACTH increases androgens
2. Primary Adrenal Reticularis tumor adenoma or CARCINOMA ** more commonly
3. Congenital Adrenal Hyperplasia (CAH)

Question 11

Congenital Adrenal Hyperplasia

1. Is caused by what
2. Causes what to happen worst thing that can happen

Answer 11

1. AR X 21-Hydroxylase ( Xaldosterone + Xcortisol + HIGH Adrongens)

Low cortisol will never block ACTH from being secreted
2. Salt wasting syndrome

Question 12

21-Hydroxylase deficiency causes what and what does that cause and what syndrome is this from

Answer 12

Congenital Adrenal Hyperplasia = Salt wasting syndrome

1. no cortisol + No mineralocorticoids (most severe form)
2. HYPONATRIEMIA , hypokalemia, hypotension
3. Virulizaion in females (Cliteromegaly + fused labia)

Question 13

Simple Virulization syndrome is what

Answer 13

Partial loss of 21-Hydroxylase ( NO salt wasting syndrome)

= just virulization

Question 14

Nonclassic / Late onset adrenal virilism is what

Answer 14

Partial loss of 21-Hydroxylase
= precocious puberty
= acne + hirsutism during puberty

Question 15

Congenital adrenal hyperplasia is dx how

Answer 15

Measure 17-Hydroxyprogesterone levels OR give 17-Hydroxyprogesterone and see if cortisol increases
(17-Hydroxyprogesterone + 21-Hydroxylase ——-> Cortisol)

+ HEEL STICKS IN INFANTS ARE USED NOW

Question 16

CAH tx

Answer 16

GLUCOCORTICOIDS (cortisol) and minerocorticoids as necessary

Question 17

Primary Acute Adrenocortical insufficiency

1. what happens and
2. Can happen from

Answer 17

1. Low cortisol released from cortical cells (cortical atrophy)
2. Rapid withdrawal from steroids
   Massive adrenal hemorrhage (Waterhouse’s Friderichsen syndrome)
   Stress, infections, trauma, burns

Question 18

What does an adrenal crisis looks like **

Answer 18

ACUTE ABD pain + FEVER + HYPOTENSION (not solved with fluids) = low glucose, K+ , Na+

= GIVE STEROID REPLACEMENT

Question 19

Waterhouse Friderichen syndrome is usually seen when

Answer 19

Sepsis form Neisseria Meningitis (whippes out adrenal glans)

Question 20

Primary Chronic Adrenocortical Insufficiency :

1. Is what
2. SX

Answer 20

1. ADDISONS disease (low chronic cortisol)

2. Malaise, fatigue, WL, Joint pain, HYPERPIGMENTATION

Question 21

Addison dz hyperpigmentation comes from

Answer 21

Elevated ACTH (as seen in Nelson’s syndrome also when you remove adrenal glands) = increases MSH (melanocyte stimulating syndrome)

Question 22

Addisons in 1800 and developing countries and Addisons now and in US and all other countries

Answer 22

TB

No is Autoimmune

Question 23

Primary Chronic Adrenocortical Insufficiency : WHAT DO I DO NOW

Answer 23

ADDISONS DZ :

You need to figure out if it is Autoimmune Polyendocrine TYPE 1 or Autoimmune Polyendocrine TYPE 2

Question 24

Autoimmune Polyendocrine TYPE 1 of Addisons

Answer 24

1. AIRE mutation
2. Adrenalitis, Parathyroiditis,
3. Hypogonadism
4. Pernicious anemia
5. Mucocutaneous candidiasis chronic
6. .Ectodermal dystrophy
   ==== APECED (Autoimmune Polyendocrine candidiasis Ectodermal dystrophy)

Question 25

Autoimmune Polyendocrine TYPE 2 of Addisons

Answer 25

1. Adrenalitis 2. Thyroiditis 3. T1D

Question 26

Ectodermal dystrophy

Answer 26

Malformed fingernails + malformed teeth some missing

Question 27

Adrenal insufficiency DX

Answer 27

Random free cortisol test + ACTH stimulation test (should increase cortisol) = tx give cortisol (can cause Cushings )

Question 28

Adrenal metastasis can cause

Answer 28

Adrenal insufficiency from tx

Question 29

Autoimmune adrenalitis

Answer 29

Dark blue nuclei = infiltrating lymphocytes

Question 30

Adrenal carcinoma vs adenoma

Answer 30

Carcioma = get really big and invade (compress adjacent structures)+ more likely to VIRILIZE Adenoma = stay small and round and dont invade = both are incidental xray findings

Question 31

Adrenal medulla is controlled by

Answer 31

Sympathetic Neves

Question 32

Phenochromocytoma is what + Sx + rule to 10

Answer 32

1. Hypersecreting NE + Epi 2. profound HTN, cyclical, HA, insomnia 3. 10% extraderenal (paraganglioma), 10% bilateral, 10% kids, 10% malignant, 10% no HTN ++++ 25% Familial****

Question 33

Adrenal medualla tumor like phenochromocytoma look like

Answer 33

Brown

Question 34

Phenochromocytoma TRIAD

Answer 34

1. Palpitations 2. HA 3. Diaphoresis = HTN (chronic or paroxysmal)

Question 35

Phenochromocytoma chronic complications

Answer 35

Cardiomyopathy

Question 36

Phenochromocytoma histology + DX

Answer 36

1. Zellballen = ball of cells | 2. Urine + plasma elevated metanephrines

Question 37

Myelolipoma is what 1. Consists of 2. Presents with

Answer 37

Benign tumor 1. BM + Fat 2. Hemorrhage (into itself) = pain inretroperitnium

Question 38

Adrenal incidentaloma is what and what do I do

Answer 38

1. Incidentally finding adrenal tumor on imaging 2. Get it out of its bigger then 4cm + level of cortisol, aldosterone, metanephines + looks at of CT shows (if there is cysts , fat (myelolipoma) you dont have to do anything) if it’s malignant looking or functionally increases Hs then remove it

Question 39

MEN syndromes are what and types

Answer 39

Familial syndromes that give you Endocrine tumors of multiple sites (aggressive, recurrent, bilateral multiple) 1. MEN 1 = Pituitary adenoma, Pancreas, parathyroid adenoma 2. MEN 2A = Parathyroid adenoma, Phenochromocytoma, medullary thyroid carcinoma 3. MEN 2B = Mucosal neuromas, Marfanoid body habitus + Phenochromocytoma, medullary thyroid carcinoma

Question 40

MEN 1 (Multiple Endocrine Neoplasia) 1. Mutation 2. What you get 3. Another thing you can get that is associated

Answer 40

1. Menin lost tumor suppression 2. Primary hyperparathyroidism, Pancreatic Endocrine Tumor, Pituitary adenoma (Lactotroph + Somatotroph) 3 Ps 3. Gastrinomas (Duodenal)

Question 41

MEN 2A 1. Mutation 2. What you get

Answer 41

1. Gained oncogene RET | 2. Phenochromocytoma + Medullary Thyroid carcinoma + Parathyroid hyperplasia

Question 42

MEN 2B : 1. Mutation 2. What happens

Answer 42

1. Gain of oncogene RET 2. Medullary thyroid carcinoma + Phenochromocytoma + Mucosal neuromas (tongue) 3. You also see Marfan syndrome characteristics

Question 43

Medullary Thyroid carcinoma means what and what do you need to do

Answer 43

Very deadly , need to remove thyroid by age 10yo (medically C-cell hyperplasia is precursor to see) = will lead to Medually Thyroid Carcinoma

Question 44

Familial Medullary Thyroid carcinoma

Answer 44

RET oncogene and in familial

Question 45

Pineal Gland 1. Tissue it has + secretes what 2. 3 tumors it has

Answer 45

1. Photoreceptor with neuro tissue ( Melatonin) | 2. Germ cell tumor (can happen any place midline) , Pineocytoma, Pineoblastoma