Adrenocortical carcinoma in a pediatric patient is rare but often virilizing.
The presence of a pediatric adrenocortical carcinoma raises the possibility of genetic cancer predisposition syndrome such as:
(1) Li Fraumeni syndrome
(2) Beckwith-Wiedemann syndrome
In a virilizing tumor the patient may present with virilization if female or precocious puberty if male.
Laboratory tesitng may show:
(1) elevated serum dehydroepiandrosterone sulfate (DHEAS)
(2) elevated serum testosterone
(3) elevated urinary 17-ketosteroid
Poor prognostic factors:
(1) high mitotic rate
(2) tumor necrosis
(3) atypical mitotic figures
(4) high Ki67 staining
(5) mutated p53
Follow-up is required to:
(1) identify local recurrence or distal metastases
(2) central precocious puberty (due to hypothalamic-pituitary activation by the hormonal changes)
