Pathology: Adrenal Glands

Question 1

The healthy adrenal gland is approximately how big ?

Answer 1

2.5-3 cm

Question 2

What are the two main subdivisions of the Adrenal Gland ?

Answer 2

Cortex

Medulla

Question 3

What are the three layers of the Cortex ?

Answer 3

Zona Glomerulosa
Zona Fasciculata
Zona Reticulata

Question 4

What does the Zona Glomerulosa produce ?

Answer 4

Mineralocorticoids (Aldosterone)

Question 5

What does the Zona Fasciculata produce ?

Answer 5

Glucocorticoids

Question 6

What does the Zona Reticulata produce ?

Answer 6

Sex hormones

Question 7

What is Cushing Disease ?

Answer 7

Excess ACTH production in the anterior pituitary that will lead to increased cortisol production

Question 8

Is Cushing Disease a primary or secondary endocrine dysfunction ?

Answer 8

Secondary

Question 9

What is Cushing Syndrome ?

Answer 9

Anything that can lead to increased glucocorticoid levels (Cushing Syndrome is Hypercotrisolism and can be exogenous or endogenous.)

( In fact, the Syndrome includes Cushing Disease, which only involves hypercortisolism caused by pituitary hyper production of ACTH)

Question 10

Who are affected more by Cushing Disease: Men or Women ?

Answer 10

Women

Question 11

Cushing Disease is caused by adenoma of the pituitary gland leading to increased release of ACTH. Are they micro or macro adenomas ?

Answer 11

Microadenoma.

Question 12

What is seen in the Adrenal glands of those with Cushing Disease (pituitary ACTH secreting micro adenoma) ?

Answer 12

Varying degrees of Nodular cortical hyperplasia.

Question 13

What percent of Cushing SYNDROME patients have a ACTH secreting tumor that is NOT found within the adrenal gland ? (ectopic secretion)

Answer 13

10%

Question 14

Where is the main site of Non-renal ACTH secreting tumor ? (ectopic secretion)

Answer 14

Lung (Small Cell Carcinoma of the Lung)

Other ectopic sites include: Carcinoids, medullary carcinomas of the thyroid, and islet cell tumors

Leads to bilateral cortical hyperplasia of the adrenals

Question 15

Who is most likely to have an ectopic ACTH producing tumo ?

Answer 15

MEN ! (40-50 yo)

Question 16

What is the morphological ‘change’ called in the pituitary which produces homogeneous and paler cells in the course of Cushing Syndrome ?

Answer 16

“Crooke Hyaline Change”

Question 17

What are the two classification of ‘endogenous’ causes of Cushing syndrome ?

Answer 17

ACTH Dependent (Production of Corticosteroids is reliant on ACTH, typically caused by an ACTH secreting adenoma of the pituitary)

ACTH Independent (Tumor of the adrenals which produces Corticosteroids w/o need for ACTH stimulation)

Endogenous: occurs within the axis of the Hypothalamus/Pituitary/Adrenals. (anything outside of this is considered exogenous: drugs, ectopic tumors etc.)

Question 18

What is the MOST COMMON cause of Cushing Syndrome ?

Answer 18

According to Pathoma and the Cleveland Clinic, it is Exongenous Corticosteroid Drug use. (iatrogenic)

Question 19

Is Primary pigmented nodular adrenocortical disease malignant ? How can you tell

Answer 19

No, just looks nasty with lipofuscin rich granules

you can tell because most malignancies have a ‘desmoplastic’ look, meaning they have an outer ring that looks blue/ purple due to NEW collagen being placed down to wall off tumor

Question 20

How large do adrenal glands get in Cushing Syndrome (secondary causes) ?

Answer 20

Usually up to 10-12 g (have been shown to get up to 30 but very unlikely)

Question 21

What does the adrenal cortex often look like when examined in a patient with cushing syndrome ?

Answer 21

Diffusely thickened and variably nodular

Question 22

How does the zona reticularis look with Cushing Syndrome ?

Answer 22

LIPID POOR

Question 23

Cushings often shows compact eosinophilic cells surrounded by an outer zone of what kind of cells ?

Answer 23

vacuolated lipid rich cells

Question 24

Primary adrenocortical adenomas

are more prevalent in which demographic ?

Answer 24

More common in women in their 30s to 50s

Question 25

What color are primary adrenocortical adenomas ?

Answer 25

yellow

Question 26

Do primary adrenocortical adenomas have a capsule ?

Answer 26

Yes !! Differentiate between them and carcinoma

Question 27

How much Do primary adrenocortical adenomas weigh ?

Answer 27

Less than 30 g

Question 28

primary adrenocortical adenomas resemble cells from which cortex 'zone' ?

Answer 28

Zone Fasciculata

Question 29

Do adrenocortical carcinomas have a capsule ?

Answer 29

Nope !

Question 30

How much do adrenocortical carcinomas weigh ?

Answer 30

200-300 g!!

Question 31

Where does the weight gain seen in Cushings distribute ?

Answer 31

Trunk , Back (buffalo hump) and Face (moon facies)

Question 32

Are patients with Cushings hypo or hypertensive ?

Answer 32

Hyper

Question 33

Primary Hyperaldosteronism is considered Conn Syndrome when ....

Answer 33

It involves a primary adenoma that secretes Aldosterone

Question 34

What is the Most common manifestation of Primary Hyperaldosteronism ?

Answer 34

HTN

Question 35

What is the most common cause of Primary Hyperaldosteronism ?

Answer 35

Aldosterone-producing adenoma (Conn Syndrome)

Question 36

On what side are Aldosterone-producing adenomas most prevalent ?

Answer 36

Left (still occur on the right)

Question 37

Who is most at risk for getting a Aldosterone-producing adenoma ?

Answer 37

Women in 30-40s

Question 38

What color are Aldosterone-producing adenomas ?

Answer 38

YELLOW

Question 39

What kinds of cells compose the majority of Aldosterone-producing adenomas

Answer 39

lipid-laden cortical cells (LOTS OF FAT) Typically uniform in size and shape.

Question 40

What are spironolactone bodies ?

Answer 40

Characteristic finding in Aldosterone-producing adenomas Eosinophilic, laminated cytoplasmic inclusions Following treatment with spironolactone (for HTN but unknowing of underlying disease) Resembles a psamomma body.

Question 41

One cause of Primary Hyperaldosteronism is Bilateral idiopathic hyperplasia. What kind of hyperplasia is seen in this ?

Answer 41

Wedge shaped towards the middle.

Question 42

What is the most common form of Adrenogenital Neoplasm ?

Answer 42

Adrenogenital CARCINOMA

Question 43

Congenital adrenal hyperplasia (CAH) is a autosomal-recessive, inherited metabolic defect in which enzyme ?

Answer 43

21-Hydroxylase Deficiency

Question 44

What will be absent in this disorder ?

Answer 44

CORTISOL and ALDOSTERONE

Question 45

Is Congenital adrenal hyperplasia (CAH) a primary or secondary hypoadrenal disease ?

Answer 45

PRIMARY

Question 46

Primary acute adrenocortical insufficiency is also known as ...

Answer 46

Adrenal Crisis

Question 47

Primary chronic adrenocortical insufficiency is also known as

Answer 47

Addison Disease.

Question 48

Who typically gets Primary acute adrenocortical insufficiency

Answer 48

Patients who are maintained of corticosteroid therapy. When the drugs are withdrawn their cortiol levels plummet.

Question 49

What is the disease of newborns who suffer adrenal infarction following an infection with N. meningitides that leads to sepsis, hypotension and DIC called ?

Answer 49

Waterhouse-Friderichsen Syndrome

Question 50

What kind of disease is Waterhouse-Friderichsen Syndrome classified as ?

Answer 50

Primary acute adrenocortical insufficiency

Question 51

What is seen on microscopy of adrenal with Waterhouse-Friderichsen Syndrome

Answer 51

adrenals were grossly hemorrhagic and shrunken; microscopically, little residual cortical architecture is discernible. Fibrin deposition.

Question 52

What is the primary cause of Addison disease ?

Answer 52

Progressive destruction of the adrenal cortex Do not appear until at least 90% of the adrenal cortex has been compromised

Question 53

What are the four major causes of cortex destruction in Addisons disease ?

Answer 53

Autoimmune adrenalitis Tuberculosis AIDS Metastatic cancers

Question 54

Autoimmune polyendocrine syndrome type 2 (APS2) which can cause Addisons, presents as ..

Answer 54

a combination of adrenal insufficiency and autoimmune thyroiditis or type 1 diabetes

Question 55

Addison Disease due to Primary autoimmune adrenalitis presents as irregularly shrunken glands Scattered residual cortical cells in connective tissue and infiltrate with what kind of cells in the cortex (possible medulla) ?

Answer 55

Lymphocytes.

Question 56

What occurs to the architecture of the Adrenals with Tuberculous and fungal disease (leading to Addisons) ?

Answer 56

COMPLETE DESTRUCTION

Question 57

What occurs to the architecture of the Adrenals with Metastatic carcinoma (leading to Addisons ) ?

Answer 57

obscures it

Question 58

Secondary Adrenocortical Insufficiency is due to a problem in the hypothalamus or pituitary. How will this differ from primary (addisons ) ?

Answer 58

``` LOW ACTH levels (high in addisons) No hyperpigmentation (lots of hyper pigmentation in addisons) ```

Question 59

What occurs to the overall size of the adrenals in Secondary Adrenocortical Insufficiency ?

Answer 59

Decrease in size ! (No stimulation) but retain their color

Question 60

Secondary Adrenocortical Insufficiency causes the cortex to be reduced to a 'ribbon size'. Which cells make up the majority of this ribbon ?

Answer 60

zona glomerulosa

Question 61

Is the medulla affected in Secondary Adrenocortical Insufficiency ?

Answer 61

Nope....

Question 62

What kind of neoplasm is more prominent in the adrenals of children ?

Answer 62

Carcinoma

Question 63

What is the defect associated wit the adrenal cancer caused by Li-Fraumeni syndrome

Answer 63

Li-Fraumeni syndrome is caused by Germline p53 mutations.

Question 64

Beckwith-Wiedemann syndrome is associated with adrenal neoplasm and is an Imprinting disorder that is characterized by what finding ?

Answer 64

Large tongue

Question 65

how are most Adrenocortical adenomas found ?

Answer 65

Incidentally..

Question 66

What size are most Adrenocortical adenomas ?

Answer 66

2.5 cm in diameter

Question 67

Describe the morphological presentation of Adrenocortical neoplasms cells ?

Answer 67

Composed of cells similar to normal adrenal cortex Nuclei tend to be small Some degree of pleomorphism may be encountered Cytoplasm ranges from eosinophilic to vacuolated.

Question 68

Virulism is associated with which kind of Adrenocortical neoplasms ?

Answer 68

Carcinoma

Question 69

What size are most Adrenocortical Carcinomas ?

Answer 69

Many exceed 20 cm in diameter | WEIGHT: 200-300 g. (HUGE)

Question 70

What is a common site from which metastatic Adrenocortical Carcinomas migrate ?

Answer 70

THE LUNGS (bronchogenic)

Question 71

Due to their strong tendency to invade local tissue (Adrenal vein, vena cava, and lymphatics) and spread hematogenously (lungs, bone) what is the overall survival for patients with Adrenocortical Carcinomas .

Answer 71

2 years.

Question 72

Unusual benign lesions Composed of mature fat and hematopoietic cells seen in the Adrenal gland...

Answer 72

Adrenal myelolipoma

Question 73

What kind of cells make up the Adrenal Medulla ?

Answer 73

Composed of specialized neural crest (neuroendocrine) cells (Chromaffin and Supporting cells)

Question 74

What do chromaffin cells produce ?

Answer 74

catecholamines. (epi, nor epi)

Question 75

Pheochromocytomas are composed almost entirely of what cells ?

Answer 75

chromaffin cells

Question 76

What are the rules of 10 ?

Answer 76

10% of pheochromocytomas are extra-adrenal 10% of sporadic adrenal pheochromocytomas are bilateral (Majority are unilateral) 10% of adrenal pheochromocytomas are biologically malignant (most are benign) 10% of adrenal pheochromocytomas are not associated with hypertension (HEAVY HYPERTENSION IN MOST)

Question 77

Although the size of Pheo's varies, what is the average size ?

Answer 77

100 g (more than Adenoma but less than carcinoma)

Question 78

Are Pheos' well demarcated ?

Answer 78

yess

Question 79

are pheos richly supplied by blood ?

Answer 79

yupp

Question 80

What color are Pheo's ?

Answer 80

Smaller pheochromocytomas--yellow-tan | Larger lesions--hemorrhagic, necrotic, and cystic

Question 81

What stain will turn Pheo tissue brown ?

Answer 81

Potassium dichromate solution | causes oxidation of catecholamines

Question 82

What shape will chromaffin cells take in Pheo ?

Answer 82

Polygonal to spindle-shaped

Question 83

The nuclei in chromaffin cells of Pheo's will have what kind of chromatin appearance ?

Answer 83

Stippled "salt and pepper" chromatin

Question 84

Zellbalin effect in pheo's

Answer 84

fibrous network clumping it into balls

Question 85

What will lead a physician to believe a pheo is malignant ?

Answer 85

Based exclusively on the presence of metastases but also.... Numbers of mitoses, confluent tumor necrosis, and spindle cell morphology