Adrenal Pathology

Question 1

What’s the most important cause of ectopic ACTH secretion?

Answer 1

Small cell lung cancer.

Question 2

When you have an adrenal adenoma (esp. corticotroph), what does the rest of the gland often look like?

Answer 2

Rest of gland looks atrophic, because ACTH etc. will be suppressed by the hormones the adenoma is producing.

Question 3

You guys all know what malignant tumors look like, right?

Answer 3

Yeah. Hemorrhage, necrosis, atypical nuclei, all that stuff.

Question 4

What makes adrenal cortical carcinomas nasty pieces of shit?

Answer 4

They have a strong propensity for vascular invasion - often invading the IVC and right atrium. Assholes.

Question 5

What hormones do adrenal cortical carcinomas make?

Answer 5

It varies. They can make all, or none. Cortisol-producing is most common.

Question 6

Factors contributing to worse prognosis for adrenal cortical carcinoma?

Answer 6

Large size.
Lots of mitosis.
Loss of p53.
High Ki-67.
Venous invasion.

Question 7

What are the criteria for rating malignancy of adrenal cortical carcinoma (ACC)?

Answer 7

The Weiss Criteria.

Question 8

4 genetic syndromes associated with ACC? (I don’t want to memorize them either, but she said it’s good to know…)

Answer 8

Li-Fraumeni syndrome (loss of p53)
Beckwith-Wiedemann syndrome
Familial Adenomatous Polyposis coli - loss of APC
Multiple Endocrine Neoplasia 1 - loss of Menin

Question 9

How can ACC be a cause of fever of unknown origin?

Answer 9

Necrosis -> inflammatory reaction.

Question 10

What are adrenal tumors-UMP?

Answer 10

Tumors of Uncertain Malignant Potential: Weiss criteria puts them between adenoma and ACC. Most are benign, but some progress.

Question 11

3 types of hyperplasia of the adrenal cortex? Sex bias for each? Degree of weight change of gland relative to normal?

Answer 11

Simple diffuse (more in women) - 2x normal weight.
Bilateral Nodular (more in women) - about 3x normal weight.
Ectopic (more in men) - about 4x normal weight.

Question 12

How do the causes of hypercortisolism vary between adults and children?

Answer 12

Adults more likely to have hyperplasia.

Children more likely to have ACC (if they have hypercortisolism).

Question 13

What do aldosteronomas look like grossly? Histologically?

Answer 13

Benign, egg-yolky things.

Histologically, the cells have lots of space in the cytoplasm as is seen in the zona glomerulosa.

Question 14

How much of each adrenal cortex must be destroyed before Addison’s happens?

Answer 14

About 90%.

Question 15

What’s the clinical triad of pheochromocytoma?

Answer 15

Paroxysmal hypertension
Headaches
Diaphoresis

Question 16

What’s the important thing to remember about the pheo’s that occur in syndromes associated with pheo’s?

Answer 16

They can be in multiple organs, and are more likely to be malignant.

Question 17

What’s the rule of 10% for pheo’s?

Answer 17

10% are bilateral
10% are extra-adrenal.
10% are malignant.
But…. then she emphasized to us that it’s not a good rule.

Question 18

How is malignancy of a pheo determined? (2 ways) (Also, what’s weird about grading of pheo’s)

Answer 18

Presence of metastases.
PASS score.
(Pheo’s can be clearly invasive, necrotic, mitotic without being considered malignant)

Question 19

Genetic syndromes associated with pheo’s? (name 3)

Answer 19

Multiple Endocrine Neoplasia (MEN) 2A and 2B
Von Hipple Lindau Syndrome (mutation in VHL, tumor suppressor)
Neurofibromatosis

Question 20

How many people with pheo’s have a germline predisposing mutation?

Answer 20

25-30%

Question 21

3 multiple endocrine neoplasia (MEN) syndromes?

Answer 21

MEN 1
MEN 2A
MEN 2B

Question 22

Where to MEN 1 neoplasms occur? Which is most serious?

Answer 22

The 3 P’s:
Pituitary
Parathyroid
Pancreatic Islets - most serious.

Question 23

Mutated gene in MEN1?

Answer 23

Menin

Question 24

Mutation in MEN 2A and 2B?

Answer 24

Ret proto-oncogene.

Question 25

Worst part of MEN 2A and 2B?

Answer 25

Medullary thyroid carcinoma.

Question 26

3 areas of neoplams in MEN 2A?

Answer 26

Thyroid C-cells
Adrenal Medulla (pheo’s)
Parathyroids

Question 27

Where does MEN 2B affect?

Answer 27

All the stuff of MEN 2A, plus:
Neural tissue of oral and GI
Causes skeletal and eye lens abnormalities…

Question 28

How much does she miss her cat?

Answer 28

A lot.