Adrenal

Question 1

Catecholamine-producing tumors derived from the sympathetic or parasympathetic nervous system.

Answer 1

pheochromocytoma

Question 2

These may arise sporadically or be inherited as features of

Answer 2

• multiple endocrine neoplasia type 2
• von Hippel–Lindau disease
• pheochromocytoma-associated syndromes

Question 3

The clinical presentation is variable, ranging from an .

Answer 3

adrenal incidentaloma to a hypertensive crisis with associated cerebrovascular or cardiac complications

Question 4

The mean age at diagnosis of pheochromocytoma is

Answer 4

40 y.o

Question 5

The classic “rule of tens” for pheochromocytomas states that

Answer 5

• 10% are bilateral,
• 10% are extraadrenal,
• 10% are malignant.

Question 6

well-vascularized tumors that arise from cells derived from the
- sympathetic (e.g., adrenal medulla)
- parasympathetic (e.g., carotid body, glomus vagale) paraganglia

Answer 6

Pheochromocytomas and paragangliomas

Question 7

The name pheochromocytoma reflects the black-colored staining caused by___; although a variety of terms have been used to describe these tumors, most clinicians use this designation to describe symptomatic catecholamine-producing tumors, including those in extra-adrenal retroperitoneal, pelvic, and thoracic sites.

Answer 7

chromaffin oxidation of catecholamines

Question 8

The term paraganglioma is used to describe ___ in the skull base and neck; these tumors may secrete little or no catecholamine.

Answer 8

catecholamine-producing tumors

Question 9

• inactivation has been demonstrated for the VHL
• activate receptor tyrosine kinase activity.

Answer 9

• Biallelic gene
• RET mutations

Question 10

reduce protein degradation, resulting in upregulation of components involved in cell cycle progression, glucose metabolism, and oxygen sensing.

Answer 10

VHL mutations

Question 11

Its clinical presentation is so variable that pheochromocytoma has been termed

Answer 11

“the great masquerader”

Question 12

typical clinical features and these manifestations constitute a classic triad of pheochromocytoma.

Answer 12

episodes of palpitation, headache, and profuse sweating

Question 13

The presence of all three manifestations in association with ___ makes pheochromocytoma a likely diagnosis.

Answer 13

hypertension

Question 14

can lead to heart failure, pulmonary edema, arrhythmias, and intracranial hemorrhage.

Answer 14

Catecholamine crises

Question 15

The diagnosis is based on documentation of ___ by biochemical testing and localization of the tumor by imaging.

Answer 15

catecholamine excess

Question 16

traditionally the first step in diagnosis of pheochromocytoma

Answer 16

measurement of catecholamines or metanephrines (their methylated metabolites)

Question 17

Catecholamines and metanephrines can be measured by different methods

Answer 17

• high-performance liquid chromatography,
• enzyme-linked immunosorbent assay,
• liquid chromatography/ mass spectrometry.

Question 18

re more convenient and include measurements of catecholamines and metanephrines.

Answer 18

Plasma tests

Question 19

re the most sensitive and are less susceptible to false-positive elevations from stress, including venipuncture.

Answer 19

Measurements of plasma metanephrine

Question 20

In this circumstance, it is important to exclude dietary or drug-related factors (withdrawal of levodopa or use of sympathomimetics, diuretics, tricyclic antidepressants, alpha and beta blockers) that might cause false-positive results and then to repeat testing or perform a

Answer 20

clonidine suppression test

Question 21

Test that are of relatively low sensitivity and are not recommended.

Answer 21

phentolamine test and the glucagon provocation test,

Question 22

similar in sensitivity and should be performed with contrast

Answer 22

CT and MRI

Question 23

is optimal for detecting pheochromocytomas and is somewhat better than CT for imaging extraadrenal pheochromocytomas and paragangliomas.

Answer 23

T2-weighted MRI with gadolinium contrast

Question 24

The primary treatment for a pheochromocytoma is

Answer 24

surgery to remove the tumor

Question 25

• Before you have surgery, your health care provider will likely prescribe ___.
• These medications block high-adrenaline hormones to lower the risk of developing dangerously high blood pressure during surgery

Answer 25

specific blood pressure medications