Lecture 17: Adrenal Pathology

Question 1

What is the important cause of hypercortisolism?

Answer 1

Lung cancers

Ectopic ACTH

Question 2

What are the causes of hypercortisolism?

Answer 2

1. Bilateral hyperplasia (80%)
   
   • pituitary ACTH
   • ectopic ACTH
   • primary
   • idiopathic
2. Adenoma
3. Carcinoma

Question 3

What are the gross characteristics of adrenal cortex carcinoma?

Answer 3

Gross:

- large size
- necrosis
- hemorrhage
- invasion

Question 4

What are the histologic characteristics of adrenal cortex carcinoma?

Answer 4

1. cellular pleomorphisim
2. capsular invasion
3. vascular invasion
4. invasion of nearby organs
5. metastases
   MITOSIS
   NECRSOSIS

Question 5

What is Li fraumeni syndrome?

Answer 5

Mutation in TP53

Patient is predisposed to all different types of cancer

Question 6

What are the characteristics of adrenocortical carcinoma?

Answer 6

Extremely rare (0.5-2 cases per million)
Highly necrotic
Thromboemboli
Only definitive criteria for malignancy are distance metastases

Question 7

What is Tumors-UMP?

Answer 7

Tumors of uncertain malignant potential
-weight is between 200-500g
Size: 4-6 cm
Confined to adrenal

Question 8

What are poor prognostic factors for adrenal carcinoma?

Answer 8

Mitotic activity, p53, venous invasion, weight of 50g

Question 9

What does the lung cancer make?

Answer 9

It makes ACTH INDIVIDUALLY!!!, it doesn’t matter where the lung cancer is, it always make ACTH

Question 10

What characterizes HYPOfunction of adrenal cortex?

Answer 10

1. autoimmune destruction
2. replaced by
   
   • TB
   • fungi
   • amyloid
   • tumor

Question 11

What are the rule of 10s for pheochromocytoma?

Answer 11

10% bilateral
10% of extraadrenal
10% are malignant

Question 12

What is the clinical triad of pheochromocytoma?

Answer 12

1. Paroxysmal hypertension
2. headaches
3. diaphoresis

Question 13

What is the PASS score?

Answer 13

Phochromocytoma of the Adrenal Gland Scaled Score

Gave patients score for presence of metastases

Question 14

What is MEN syndrome?

Answer 14

Multiple Endocrine Neoplasia
Genetically FAMILIAL syndromes
Signs and symptoms depends on organs

Question 15

What is the only definitve criteria for malignancy?

Answer 15

Distant metastasis or local invasion

Question 16

What are the three MEN syndromes?

Answer 16

MEN-1 (Werner)
MEN-2a (Sipple)
MEN-2b

Question 17

What are the characteristics of MEN1?

Answer 17

Hyperplasia and neoplasia of
	i. pituitary
	ii. parathyroid
	iii. pancreatic islets
	iv. adrenal cortex
	v. lung
	vi. thymus
Different family members may manifest different symptoms due to organ involved
Gene = MENIN
Clinical prognosis related to pancreatic lesions

Question 18

What are the characteristics of MEN 2A and 2B?

Answer 18

Medullary thyroid carcinoma is most prognostically important lesion
RET protooncogene
MEN2A = thyroid C cells and parathyroids neoplasia
MEN2B = thyroid C cells and neural tissue of oral/GI systems

Question 19

The clinical prognosis of MEN-1 is related to which of the following? EXAM QUESTION

Answer 19

Pancreatic lesion

Question 20

Which of the following define a malignant pheochromocytoma? EXAM QUESTION

Answer 20

1. most reliable is presence of metastases (lymph nodes, liver, lung, bone)
2. malignant cases have MIB-1 positive cell rate of 2-3%
   PASS score greater than 4 (nesting, mitotic figures)

Question 21

You diagnose hyperparathyroidism, thyroidism, Ganglioneuroma and pheocytochroma. Which gene has a mutation? EXAM QUESTION

Answer 21

Gene = RET
Multiple Endocrine Neoplasia type 2
MEN-2

Question 22

You diagnose hemangioblastoma, renal, pancreatic tumors and pheochromocytoma. Which gene has a mutation?

Answer 22

VHL (vonHippel disease)

Question 23

What happens if you have multiple adrenal tumors?

Answer 23

That means you have FAMILIAL TUMORS

RET, SDHB, VHL, NF1