Adrenocortical carcinoma

Question 1

Most common adrenocortical tumor?

Answer 1

Unilateral, benign, adrenocortical adenoma

Question 2

Peak age of incidence for adrenocortical carcinoma

Answer 2

Bimodal – early childhood + fifth decade of life

Question 3

presentation

Answer 3

symptoms of excess steroid hormone production

Question 4

therapy for localized ACC

Answer 4

Surgery – open adrenalectomy
Consider adjuvant RT (improves local control but unclear effect on PFS and OS)
Aduvant mitotane (unclear benefits)

Question 5

therapy for metastatic ACC

Answer 5

IF indolent → consider observation
Options:
Mitotane monotherapy + replacement dose hydrocortisone
cisplatin OR carboplatin + etoposide + doxorubicin +/- mitotane
IF hormone-producing → antihormonal therapy (aldactone, mitotane, ketoconazole, metyrapone)

Question 6

Mechanism of action of mitotane

Answer 6

• drug that inhibits steroid production
  (steroidogenesis inhibitor and cytostatic antineoplastic medication which is used in the treatment of adrenocortical carcinoma and Cushing’s syndrome.[1][2][3][4] It is a derivative of the early insecticide DDT )