Hypothalamus Disorders

Question 1

what is a pheochromocytoma?

Answer 1

catecholamine secreting tumor that arises from chromaffin cells of the adrenal medulla and sympathetic ganglia

Question 2

what is a paraganglioma?

Answer 2

a extraadrenal catecholamine secreting tumor

Question 3

what is the most common population that has pheochromocytomas?

Answer 3

may occur at any age, most common in 4th-5th decade

Question 4

where are most PCC found in body?

Answer 4

benign, unilateral, in adrenal medulla

some are intra-abdominal

Question 5

what types of germ-line mutations might you find with them?

Answer 5

MEN (multiple endocrine neoplasia)

von Hippel-Lindau Dz

Neruofibromatosis Type 1

Familial Paraganglioma

Question 6

what are the clinical features of a PCC?

Answer 6

triad: Headahce, palpitations, sweating that may be episdic (catecholamine release is paroxysmal)

Question 7

how do you test for a PCC?

Answer 7

• plasma free metanephrines
• 24 hour urine (catecholamines, metanephries)
• MRI of abdomen (or non-contrast CT)
• whole body of nuclear imaging when CT or MRI failu to locate tumor

Question 8

tx of PCC?

Answer 8

surgical resection of the tumor

*undx PCC can be lethal (HTN crisis!)

Question 9

what drug can be given while a pt is awaiting to have surgery for a PCC?

Answer 9

phenoxybenzamine!

• long acting alpha adrenergic blocker
• increases bloodflow
• lowers BP

Question 10

what are some ADRs of phenoxybenzamine?

Answer 10

hypotension, tachycardia, miosis, nasal congestion, ED