Deck 32: Epidemiology of Adrenal Cancer

Question 1

The adrenal gland consists of the adrenal cortex and the adrenal medulla.The adrenal cortex secretes steroid hormones, cortisol that regulates metabolism of protein, fat, and carbohydrates and aldosterone that regulates salt and water balance.

Answer 1

True

Question 2

The adrenal medulla contains neuroendocrine cells called chromaffin cells that communicate with the sympathetic nervous system by synapses with splanchnic nerves of the CNS.These cells synthesize and secrete catecholamines, epinephrine (adrenaline) and norepinephrine (noradrenaline) that regulate blood pressure and modulate response to stress.

Answer 2

True

Question 3

Adrenal tumors are often found incidentally during imaging studies for other conditions.

Answer 3

True

Question 4

Malignant tumors that arise from the adrenal cortex are called adrenal cortical carcinomas.Adrenal tumors that secrete adrenal steroids are classified as functional, whereas nonsecreting tumors are classified as nonfunctional.Most (90%) of adrenal cortical carcinomas secrete steroids and are therefore functional.

Question 5

Adrenal cortical carcinomas are rare (the annual incidence is 1 case per million in the USA).

Question 6

Cases of adrenal cortical carcinoma show a small early peak during childhood and a larger peak after age 55 years.

Question 7

A specific germline mutation in the p53 tumor suppressor gene is responsible for more than 90% of Brazilian cases of adrenal cortical carcinoma in children.

Question 8

Adrenal cortical carcinoma is not associated with tobacco smoking.

Question 9

The two principal tumors of the adrenal medulla are neuroblastoma and pheochromocytoma.

Question 10

Tumors of the adrenal medulla are associated with reduced blood pressure.

Question 11

Neuroblastomas are diagnosed primarily in children, whereas their counterpart, pheochromocytomas, are diagnosed in adults.

Question 12

Certain gestational exposures (opiates, folate deficiency, gestational diabetes) increase the risk of neuroblastoma in children.

Question 13

Carcinomas that secrete cortisol evolve from what tissue?

A) Adrenal cortex
B) Adrenal medulla
C) Kidney
D) Ovary

Question 14

Carcinomas that secrete catecholamines usually evolve from what tissue?

A) Adrenal cortex
B) Adrenal medulla
C) Kidney
D) Ovary

Question 15

Characteristic features of neuroblastomas include all of the following except:

A) they occur primarily in children.
B) they secrete catecholamines that elevate blood pressure.
C) they often spontaneously regress in infants under 1 year of age.
D) they are always associated with poor survival.

Question 16

Characteristic features of pheochromocytomas include all of the following except:

A) they occur primarily in adults.
B) they secrete catecholamines that elevate blood pressure.
C) approximately 90% are curable by local excision.
D) they are always associated with poor survival.

Question 17

Neuroblastomas diagnosed in children over the age of 1 year have which of the following characteristics?

A) They hypersecrete catecholamines.
B) They readily metastasize to the lungs, brain, and liver.
C) They cause 15% of cancer deaths in children.
D) The genetic aberration most consistently associated with poor outcome is amplification of the N-myc proto-oncogene.
E) a, b, c only are correct.
F) All answers are correct.

Question 18

What germline mutation was found to cause 90% of adrenal cortical carcinomas in children living in Brazil?

A) p53 (R337 H mutation)
B) Rb (Retinoblastoma gene)
C) APC (Adenomatous Polyposis Coli gene)
D) TERT (Telomerase gene)

Question 19

Describe basic clinical features of adrenal cancer.

Question 20

Briefly describe germline mutations and other risk factors associated with the development of adrenal cortical carcinomas.

Question 21

Describe characteristic features of neuroblastomas.

Question 22

Discuss the survival of neuroblastoma.

Question 23

Describe characteristic features of pheochromocytomas.