Adrenal Medulla and Paraganglia

Question 1

What are the primary cells of the adrenal medulla?

Answer 1

The chromaffin cells

Question 2

What hormones are secreted by the medulla?

Answer 2

Epinephrine and Norepinephrine

Question 3

What is pheochromocytoma?

Answer 3

Catecholamine-secreting tumors of chromaffin cells of the adrenal medulla

Question 4

What is the key symptom of a person with pheochromocytoma?

Answer 4

Hypertension

Question 5

Photomicrograof the tumor shows polyhedral tumor cells with ample finely granular cytoplasm. Dx?

Answer 5

Pheochromocytoma

Question 6

Pheochromocytoma is shown. What IHC markers are used to identify this tumor?

Answer 6

Chromogranin and synaptophysin

Question 7

What can episodic catechol release associated with pheochromocytoma precipitate?

Answer 7

Paroxysms/Crises of headache, sweating, palpitations, tachycardia, abdominal pain, and vomiting

Question 8

What is catecholamie cardiomyopathy?

Answer 8

Myocardial necrosis caused by elevated catecholamine levels

Question 9

What is Dx of pheochromocytoma

Answer 9

Elevated urinary levels of catechol metabolites (vanillylmandelic acid, metanephrine, unconjugated catecholamines)

Question 10

What are paragangliomas?

Answer 10

Pheochromocytomas arising at extra-adrenal sites

Question 11

What are carotid body tumors?

Answer 11

Paragangliomas arising at the carotid bifurcation

Question 12

What gene is associated with Paragangliomas?

Answer 12

SDHD gene

Question 13

What is Multiple Endocrine Addenomatosis?

Answer 13

Inherited disorders in which multiple endocrine organs are affected by diverese cellular proliferations

Question 14

What is another name for MEN type 1? What complications are typically seen?

Answer 14

Wermer Syndrome

1. Pituitary Adenoma
2. Parathhyroid Hyperplasia/Adenoma
3. Islet cell tumor of the pancreas

Question 15

What mutation causes Wermer Syndrome?

Answer 15

Mutation in the MEN1 tumor suppressor gene that encodes protein termed menin

Question 16

What condition is observed in almost all patients with MEN1? MEN2?

Answer 16

Primary Hyperparathyroidism; Medullary Thryroid Carcinoma

Question 17

What gene is responsible for MET2 syndromes?

Answer 17

RET protooncogene

Question 18

What mutation is responsible for most patients with MEN 2B?

Answer 18

Point mutation at codon 918 of the tyrosine kinase domain of RET

Question 19

What mutation has been identified in most patients with MEN2A?

Answer 19

Mutations in the cysteine-rich extracellular domain of RET

Question 20

What is used to Dx MEN2?

Answer 20

ID RET mutations

Question 21

What is a neuroblastoma?

Answer 21

An embryonal malignancy of neural crest origin composed of neoplastic neuroblasts

Question 22

What is the most common tumor of childhood?

Answer 22

Neuroblastoma

Question 23

What are the four categories of neuroblastomas?

Answer 23

1. Neuroblastoma
2. Ganglioneuroblastoma, intermixed
3. Ganglioneuroma
4. Ganglioneuroblastoma, nodular

Question 24

Neuroblastoma is shown. What morphologies are characteristic of this tumor?

Answer 24

Homer Wright Rosettes

Question 25

What metabolites are found in the urine of a person with Neuroblastoma?

Answer 25

Norepinephrine, VMA, homovanillic acid, and dopamine

Question 26

What is a ganglioneuroma?

Answer 26

Mature variant of neuroblastic tumors

Question 27

Histology shows well-differentiated, amture ganglion cells, associated with spindle cells in a loose, abundant fibrillar stroma. Dx?

Answer 27

Ganglioneuroma