Adrenal Cortical Tumors

Question 1

What percentage of cortical neoplasms are hormonally active in kids?

Answer 1

95% (50% in adults)

Question 2

What is the most common benign cause of Cushing in children?

Answer 2

McCune-Albright syndrome (fibrous bone dysphasia, cafe au lait spots, and precocious puberty) - bilaterally macronodular adrenalcortical disease producing cortisol.

Question 3

What is the most common cause of Cushings in children less than 6?

Answer 3

ACC - 60-80 % of all cases of Cushing

Question 4

DDx for Cushing in children?

Answer 4

ACC
McCune-Albright 
Pituitary adenoma 
Nodular adrenal hyperplasia 
Primary pigmented nodular adrenocortical disease

Question 5

Symptoms os sex hormone producing tumors?

Answer 5

Virilizing- most common presentation of cortical tumors in kids.

• Boys: precocious puberty
• Girls: clitoral hypertrophy, hirsutism and Acne.

Feminizing- rare estrogen producing tumors.

Girls: precocious isosexual development
Boys: gynocomastia, accelerated growth, delayed puberty.

Question 6

Primary vs. secondary causes of hyperaldosterone?

Answer 6

Primary: Aldosterone secreting tumor or bilateral adrenal hyperplasia (more common)

Secondary: renin overproduction - cirrhosis, CHF, renovascular disease or renin producing renal tumor.