Pathology - S2B3 - Endocrine - Adrenal

Front 1

What is the Adrenal Cortex derived from?

What is the Adrenal Medulla derived from?

Back 1

Cortex = Mesoderm

Medulla = Neural Crest

Front 2

Label the layers of the Adrenal Glands

Back 2

-

Front 3

List the hormone secreted from each layer of the Adrenal Gland

Back 3

Glomerulosa = Aldosterone = increases Na+ reabsorption; increase K+ & H+ secretion

Fasciculata = Cortisol

Reticularis = Sex Hormones = Testosterone, DHEA, Androstenedione

Medulla = Catecholamines

Front 4

What are the stimulators & inhibitors of release of each layer of the Adrenal Glands?

Back 4

Glomerulosa
-Stimulators = Angiotensin II; hyperkalemia; ACTH

Fasciculata
-Stimulator = ACTH
-Inhibitor = Cortisol

Reticularis
-Stimulator = ACTH
-Inhibitor = Cortisol

Medulla
-Stimulator = ACh from SNS

Front 5

Another name for "Acute Bilateral Hemorrhagic Adrenal Infarction"

Back 5

Waterhouse-Friderichsen Syndrome

Front 6

What is Waterhouse-Friderichsen Syndrome usually associated with?

Back 6

Septicemia from Neisseria Meningitidis

Front 7

What bacteria can cause Waterhouse-Friderichsen Syndrome?

Back 7

1. N. menigitidis
2. Pneumococcus
3. Staph
4. Strep
5. Hemophilus

Front 8

What virus can cause Waterhouse-Friderichsen Syndrome?

Back 8

Herpes

Front 9

Other than infections, what are other causes of Acute Adrenocortical Insufficiency?

Back 9

1. abrupt withdrawal of Corticosteroids
2. Stress
3. Surgery

Front 10

What is the mechanism of Waterhouse-Friderichsen Syndrome?

Back 10

release of tissue Thromboplastin due to Endotoxin causes DIC

Front 11

Waterhouse-Friderichsen Syndrome due to N. menigitidis

Back 11

What is this called?

Front 12

Waterhouse-Friderichsen Syndrome = Acute bilateral adrenal hemorrhage

Back 12

What is the name of this condition?

Front 13

Waterhouse-Friderichson Syndrome = DIC

Back 13

This is a Glomerulus. What adrenal condition is associated with this?

Front 14

Chronic Adrenocortical insufficiency = ?

Back 14

Addison Disease

Front 15

What are the clinical features of Addison's Disease?

Back 15

Insidious onset

Signs of decreased Glucocorticoids
-weakness & fatigue
-GI disturbances
-HYPOGLYCEMIA
-SKIN HYPERPIGMENTATION = due to MSH, a by-product of increased ACTH production

Decreased Alodsterone
-HYPOTENSION
-Hyperkalemia
-Hyponatremia

Front 16

Addison's disease = primary adrenocortical insufficiency

Low Cortisol does not produce feedback inhibition on ACTH production = high ACTH yields MSH as a by-product of ACTH production from POMC

Back 16

What adrenal condition would cause this? What is the mechanism?

Front 17

What are the causes of Addison's disease?
1. 60% = ?
2. 25% = ?
3. 15% = ?

Back 17

1. Autoimmune destruction of Adrenal Gland = Autoimmune Adrenalitis

2. Granulomatous Adrenalitis due to TB or Histoplasmosis

3. Other = trauma, metastatic tumor, amyloidosis

Front 18

Tuberculous granulomas

Back 18

What would be the cause of this Addison's diseased adrenal gland?

Front 19

Autoimmune Adrenalitis

Back 19

What would be the cause of this Addison's diseased adrenal gland?

Front 20

Addison's Disease due to Autoimmune Adrenalitis
-atrophied cortex with lymphocytic infiltration

Back 20

What are these pictures showing?

Front 21

What are the clinical features of Cushing Syndrome

Back 21

Weight gain = due to hyperinsulinism fom hyperglycemia (due to Cortisol)
-truncal obesity, moon facies, buffalo hump

Hypertension = due to increase in weak mineralcorticoids & glucocorticoids = Na+ retention

Osteoporosis = due to high cortisol

Lymphopenia & Eosinopenia

Front 22

What are the lab findings in Cushing Syndrome?

Back 22

1. increased Cortisol levels

2. increased 17-hydroxycorticoids & Cortisol in urine

Front 23

Cushing Syndrome

Back 23

What syndrome are these clinical findings associated with?

Front 24

List the causes of Cushing Syndrome

Back 24

Iatrogenic = Corticosteroid therapy

Cushing Disease = ACTH-secreting Pituitary adenoma

Adrenal Cortical Adenoma/Carcinoma = decreased ACTH; increased Cortisol

Ectopic ACTH = Small Cell Carcinoma of Lung = markedly increased ACTH & Cortisol

Front 25

A 45 yoa woman presents complaining of increased urination over the past several months. She has also gained 35 lbs. over the past year, bruises easily, & has grown hair on her chin. While speaking with her, you note that she has moon facies & increased fat pads on the back of her neck. Blood tests reveal hyperglycemia, increased Cortisol levels, & increased ACTH levels. Dexamethasone suppression test causes decreased Cortisol levels

Back 25

Cushing Syndrome caused by Pituitary Adenoma secreting ACTH

Front 26

How do you differentiate b/w the different causes of Cushing Syndrome?

Back 26

Pituitary Adenoma
-Cortisol suppression with Dexamethasone suppression test
-high ACTH & Cortisol

Paraneoplastic Syndrome
-markedly high ACTH; high cortisol
-no cortisol suppression by Dexamethasone

Adrenocortical Adenoma
-high Cortisol; low ACTH
-No cortisol suppression by Dexamethasone

Front 27

What would 17-alpha-hydroxylase deficiency cause?

Back 27

low Sex hormones & Cortisol
high Mineralcorticosteroids

Hypertension, Hypokalemia
Phenotypically female but no maturation

Front 28

What would 21-beta-hydroxylase deficiency cause?

Back 28

low Cortisol & Mineralcorticoids
high Sex Hormones

Masculinization
Hypotension
Hyperkalemia
Salt wasting can lead to Hypovolemia
Ambiguous genitalia in females
Precocious puberty in males

Front 29

What would 11-beta-hydroxylase deficiency cause?

Back 29

low Cortisol & Aldosterone
high Sex hormones & 11-deoxycorticosterone

Hypertension = due to increased 11-deoxycorticosterone
Masculinization

Front 30

Congenital Adrenal Hyperplasia due to 21-hydroxylase deficiency
-Clitoral enlargement

Back 30

What is the cause of this?

Front 31

Congenital Adrenal Hyperplasia (21-hydroxylase deficiency)
-Adrenal gland is under constant stimulation by ACTH but only the Reticularis can responds b/c it contains the only cells that do not need 21-hydroxylase to make their product = Sex hormones

Back 31

What is the cause of this?

Front 32

Primary Hyperaldosteronism due to a benign adenoma in the Zona Glomerulosa

Back 32

Conn's Syndrome

Front 33

What are the clinical features of Conn's Syndrome?

Back 33

-Hypertension = due to Aldosterone causing Na+ retention
-Metabolic Alkalosis & Hypokalemia = due to increase K+ & H+ secretion
-Decreased Renin levels
-decreased Serum Potassium

Front 34

List the causes of Conn's Syndrome
1. 90%
2. 10&
3. very rare

Back 34

1. Cortical Adenoma = Aldosteronoma

2. Nodular Cortical Hyperplasia

3. Adrenocortical Carcinoma

Front 35

Adrenal Cortical Adenoma as seen in Conn's Syndrome = Primary Hyperaldosteronism

Back 35

What is seen here?

Front 36

Lipid-filled Adrenal Cortical cells in a Benign Adrenal Adenoma = Conn's Syndrome

Back 36

What is seen here?

Front 37

Describe a Neuroblastoma

Back 37

Small cell malignant neoplasm arising in the Adrenal Medulla

Front 38

Neuroblastoma:
1. Most common presenting age?
2. How does it present?
3. What are the frequent metastasis sites?
4. Who has a better prognosis?
5. What do the tumor cells secrete?
6. What is increased in the urine?

Back 38

1. 1-10 years of age
2. large abdominal mass
3. lungs & marrow
4. pts under 1 year of age
5. Epi or NE
6. VMA

Front 39

Neuroblastoma = malignant tumor of Adrenal Medulla

Back 39

What is seen here?

Front 40

Neuroblastoma
-small, hyperchromatic malignant cells
-"rosettes"

Neural Ectoderm

Back 40

What is this? What is it derived from?

Front 41

Ganglioneuroma = Neuroblastoma that has reverted to a benign state

Back 41

What is seen here?

Front 42

What gene is Neuroblastoma associated with?

Back 42

N-myc oncogene

Front 43

Define Pheochromocytoma

Back 43

Neoplasm of Chromaffin cells of the Adrenal Medulla that produces Catecholamines

Front 44

Pheochromocytoma
1. peak incidence?
2. what do tumor cells secrete?
3. unilateral or bilateral
4. benign or malignant?

Back 44

1. 30-50
2. Epi &/or NE = cause surgically-correctable HTN
3. Unilateral
4. Benign

Front 45

What conditions are Pheochromocytomas associated with?

Back 45

1. Neurofibromatosis
2. MEN II = Medullary Thyroid CA + Pheochromocytoma + Parathyroid Hyperplasia or Adenoma
3. von Hippel-Lindau disease

Front 46

What are the clinical manifestations of Pheochromocytoma?

Back 46

Intermittent attacks of HTN, headache, palpitations

Anxiety

Drenching sweats

Front 47

What are the laboratory findings in Pheochromocytomas?

Back 47

increased 24-hour urinary VMA & Metanephrine levels

increased urinary & plasma Catecholamines

Front 48

Pheochromocytoma
-expanded Medullary space in the Adrenal gland

Back 48

What is seen here?

Front 49

Pheochromocytoma
-nests of polygonal chromaffin cells containing catecholamine-rich granules

Back 49

What is this from?

Front 50

Pheochromocytoma
-secretory granules staining brownish with the chromate stain

Back 50

What is seen here?

Front 51

List the "Rule of 10's" associated with Pheochromocytomas

Back 51

1. 10% occur in children
2. 10% are bilateral
3. 10% occur outside the Adrenal Gland = Paraganglioma
4. 10% are malignant
5. 10% are familial (MEN II & III)