Adrenal Medulla

Question 1

What is a pheochromocytoma?

Answer 1

Neoplasm of chromaffin cells that produces catecholamines

Question 2

What is the 5 ten percent rule for pheochromocytoma?

Answer 2

10% are...
Extra adrenal
Bilateral
In kids
Malignant
Not associated with HTN

Question 3

What is the 25% rule?

Answer 3

25% are familial

Question 4

2 groups of mutations that fall under the familial cause?

Answer 4

Growth factor pathway signaling like RET

Transcription factor HIF

Question 5

Knowing pheochromocytoma can be caused by mutations in HIF, what syndrome can pheochromocytoma be part of?

Answer 5

VHL syndrome

Question 6

What is the classic histo feature of a pheo?

Answer 6

Zellballen

Question 7

Definitive diagnosis of malignancy in pheochromocytoma is based exclusively on what?

Answer 7

Presence of metastasis

Question 8

What is the hallmark clinical symptom of a pheochromocytoma?

Answer 8

HTN

Question 9

Besides HTN, what is the clinical triad of a pheo?

Answer 9

Headache, palpitations and sweating

Question 10

Big time complication of pheochromocytoma and why?

Answer 10

Cardiomyopathy because of the increased catecholamines

Question 11

What is laboratory diagnosis of pheochromocytoma?

Answer 11

Increased unitary excretion of free catecholamines and their metabolites, metanephrines