Adrenocortical carcinoma

Question 1

Potential clinical presentation of ACC from question stem

Answer 1

• virilization (deep voice, bulk)
• cushing’s (HTN, easy bruising, facial weight gain, thick dark hair, amenorrhea, muscle weakness)

Question 2

Imaging features suggestive of ACC rather than benign

Answer 2

• irregular borders
• calcified
• invading surrounding structures
• large (greater than 4 cm)
  ***higher attenuation (hounsfield units, 20-50% washout at 10 minutes)

Question 3

Workup to determine if ACC is functional

Answer 3

***Rule out pheo and cortisol-secreting prior to surgery (So order, since endo refer can delay things)
plasma metanephrines or urinary metanephrines and catecholamines
AM cortisol, corticotropin (ACTH), 24 hr urinary free cortisol, fasting serum cortisol at 8 AM following a 1 mg dose of dexamethasone at bedtime
Refer to endocrine for remainder of workup
Review CMP (Fasting glucose, potassium)
Serum estradiol in men and postmenopausal women,
IF HTN or spontaneous or diuretic-induced hypokalemia, work up for hyperaldo
IF signs of virilization, androstenedione + testosterone + adrenal androgens (dehydroepiandrosterone sulfate [DHEAS], 17-hydroxyprogesterone) (more sense to me to work up if virilization)

Question 4

Management of early stage ACC

Answer 4

surgery if resectable w/ adjuvant mitotane depending on risk factors

Question 5

Critical component of workup prior to surgery for localized ACC

Answer 5

• hormonal evaluation - rule out pheo or cortisol secreting

Question 6

Indications for adjuvant for localized ACC

Answer 6

• Ki67>10
• R1
• intraoperative tumor spillage
• large low grade with vascular or capsular invasion

Question 7

1) Drug used for adjuvant 2) duration

Answer 7

mitotane 3-5 years

Question 8

Very high risk localized disease criteria

Answer 8

1) Ki-67>20
2) extensive vascular invasion/vena cava thrombus

Question 9

Adjuvant for very high risk localized disease

Answer 9

cisplatin + mitotane

Question 10

Management of metastatic ACC

Answer 10

• surgical debulking
• Given low grade disease OR symptoms of hypercortisolism, mitotane (prolonged remission in the case series limited to low grade disease + useful for palliation)

Question 11

mitotane goal level

Answer 11

14-20

Question 12

mitotane SE to know

Answer 12

adrenal insufficiency
aldosterone deficiency
- hypothyroidism (increases serum thyroid hormone-binding globulin (TBG).)
- hypogonadism (commonly require replacement testosterone)

Question 13

when XRT is indicated adjuvantly

Answer 13

R1 resection