Path: Adrenal

Question 1

Two causes of ACTH dependent Cushings Syndrome.

Answer 1

1. Cushings Disease: ant. pit. adenoma secreting ACTH

2. Ectopic Tumor somewhere in the body secreting ACTH: small cell lung carcinoma, Carcinoids

Question 2

What kind of change occurs in the pituitary in a patient with ACTH independent Cushing Syndrome

Answer 2

Crooke Hyaline Change
-increased levels of cortisol lead to decreased ACTH synthesis in the pituitary. This causes atrophy of the corticotrophs and lightens the cytoplasm because no ACTH production is necessary.

Question 3

Normal mass of adrenal glands

Answer 3

11g (combined)

Question 4

Mass of adrenal glands in Cushing Syndrome caused by adrenocortical adenomas.

Answer 4

About 30g

Question 5

Mass of adrenal glands in Cushing Syndrome caused by adrenocortical carcinomas.

Answer 5

200-300g

Question 6

Characteristic finding on microscopy of the adrenal gland adenomas in patients with Conn Syndrome.

Answer 6

Spironolactone Bodies

-eosinophilic laminated cytoplasmic inclusions, occurs following treatment with sprionolactone

Question 7

Mutation that can lead to Congenital Adrenal Hyperplasia resulting in ambiguous genitalia and precocious puberty.

Answer 7

21-hydrolyase deficiency

Question 8

Mutation that can lead to Congenital Adrenal Hyperplasia resulting in hypokalemia, alkalosis, hypernatremia, and hypertension.

Answer 8

17alpha-hydroxylase

Question 9

Most common manifestation of Acute Adrenal Insufficiency.

Answer 9

Waterhouse-Friderichsen Syndrome (WFS)

Question 10

Main cause of WFS.

Answer 10

N. meningitides infection in newborns

• bleeding on adrenal glands results in DIC w/ widespread purpura
• hypotension leading to shock

Question 11

Why do patients with Primary Chronic Adrenal Insufficiency (Addisons Disease) often have an irreversible condition?

Answer 11

It doesn’t show symptoms until 90% of the adrenal cortex is destroyed.

Question 12

What are the 4 main causes of Addisons Disease?

Answer 12

1. Autoimmune (in developed countries)
2. TB (in developing countries)
3. AIDS (usually fungal or bacterial)
4. Metastatic Cancer

Question 13

How can the cause of Addisons be confirmed with microscopy?

Answer 13

1. Autoimmune: lymphoid infiltrate
2. TB or AIDS (fungus): granulomas, architecture destroyed
3. Cancer: adrenals enlarged

Question 14

What is an immediate physical exam characteristic differentiating a secondary adrenal insufficiency from a primary?

Answer 14

Secondary will have NO hyperpigmentation

• no ACTH production because no POMC production
• means no MSH or excess melanin production

Question 15

What type of adrenocortical neoplasms are most common in adults?

Answer 15

Adenomas and Carcinomas are equal

carcinomas more common in children

Question 16

Most common inherited causes of adrenocortical neoplasms.

Answer 16

1. Li-Fraumeni Syndrome: p53 mutation

2. Beckwith-Wiedemann Syndrome: imprinting disorder

Question 17

In adults, how can adenomas be differentiated from carcinomas in the adrenal cortex using microscopy?

Answer 17

Adenomas will have an outer capsule layer

Carcinomas won’t

Question 18

What are the Rule of 10s with Chromaffin Cell Neoplasms (Pheochromocytomas)

Answer 18

10% are:

• extra adrenal
• bilateral
• biologically malignant
• NOT associated with HTN

Question 19

Mass of Pheochromocytomas.

Answer 19

100g

Question 20

How can pheochromocytoma be diagnosed on biopsy of an adrenal medulla?

Answer 20

Tissue is placed in Potassium Dichromate and the stored catecholamines are oxidized. They turn dark brown.

Question 21

How does pheochromocytoma appear on microscopy?

Answer 21

Salt and Pepper chromatin