Path 11 Schneider

Question 1

Cushing disease

Answer 1

Only pituitary adenoma/microadenomas -> ACTH

Question 2

Most common ectopic ACTH production

Answer 2

Small cell lung CA

Question 3

Dexamethasone suppression test

Answer 3

Won’t suppress ectopic ACTH or Hypercorticism Autonomous

Question 4

Cushing disease morphologic change

Answer 4

Bilateral hyperplasia of adrenal Zona Fasciculata

-Atrophy in C syndrome

Question 5

Cushing syndrome clinical appearance

Answer 5

• Moon facies
• Central obesity
• Abdominal striae (skin atrophy)
• Osteoporosis
• Muscle wasting
• Emotional disturbance

Question 6

Conn syndrome

Answer 6

Primary aldosteronism

Question 7

Primary aldosteronism causes

Answer 7

Adrenocortical adenoma (aldosteronoma)

• Zona Glomerulosa hyperplasia (less)
• Adrenocortical carcinoma (least)

Question 8

Primary aldosteronism clinical (4)

Answer 8

• HTN
• Hypokalemia
• Sodium and water retention
• Decreased serum Renin

Question 9

Secondary aldosteronism causes

Answer 9

• Renal ischemia/tumor

- Profound edema (cirrhosis, CHF, neprhotic syndrome)

Question 10

Secondary aldosteronism mechanism

Answer 10

Renin-angiotensin stimulation

-Serum renin increased (opposite of primary aldosteronism)

Question 11

Primary v secondary aldosteronism

Answer 11

• Unilateral v bilateral ZG hyperplasia

- Decreased v increased serum Renin

Question 12

Adrenal virilism

Answer 12

Congenital cortisol enzyme defect -> ACTH compensation -> adrenal hyperplasia -> adrogenic steroid production

Question 13

Adrenal virilism enzyme

Answer 13

21-hydroxylase deficiency (salt wasting form -> hypotension)

11-hydroxylase deficiency (less common, salt retention -> HTN)

Question 14

21-hydroxylase function

Answer 14

17-hydroxyprogesterone -> 11-deoxycortisol

Question 15

21-hydroxylase deficiency

Answer 15

17-hydroxyprogesterone -> androstenedione

Question 16

Adrenal virilism morphology

Answer 16

Zona Reticularis adenoma, carcinoma, hyperplasia

Question 17

Pituitary deficiency

Answer 17

Secondary adrenal failure - No ACTH

-No hyperpigmentation - No B-MSH

Question 18

Addison disease

Answer 18

Primary adrenocortical deficiency

-Autoimmune or tuberculosis

Question 19

Addison clinical

Answer 19

• Hypotention
• Hyperpigmentation
• Decreased electrolytes except potassium

Question 20

Tuberculosis histo

Answer 20

Caseating granuloma

Langhans giant cell

Question 21

Waterhouse-Friderichsen syndrome (3)

Answer 21

Catastrophic adrenal insufficiency & vascular collapse

• Meningococcal meningitis
• Hemorrhagic necrosis of adrenal cortex due to DIC

Question 22

Adrenal cortical adenoma

Answer 22

Loss of function?

Question 23

Chromaffin cell tumors

Answer 23

Adrenal - Pheochromocytoma

Extra-adrenal - Paraganglioma

Question 24

Pheochromocytoma

Answer 24

Surgically correctable HTN

• due to catecholamine hyperproduction
• usually benign

Question 25

Pheochromocytoma test

Answer 25

Urine vanillylmandelic acid

• metanephrine
• normetanephrine

Question 26

Pheochromocytoma EM

Answer 26

Membrane-bound secretory granules (black)

Question 27

Neuroblastoma

Answer 27

Malignant childhood catecholamine tumor

-“Small blue cell” tumor

Question 28

Neuroblastoma origin

Answer 28

Adrenal medulla

Question 29

Neuroblastoma presentation

Answer 29

Large abdominal mass

-HTN

Question 30

Neuroblastoma cause

Answer 30

N-myc oncogene amplification

• Copy number related to aggressiveness
• Occasionally converts into benign ganglioneuroma