Clinical management of adrenocortical carcinoma
A thorough pre-operative hormonal workup is advised following the recommendations of the European Network for the Study of Adrenal Tumors. Demonstration of endocrine activity may serve to the following purposes: i) prove adrenocortical origin of the mass; ii) suggest malignancy, in the event of androgen hypersecretion; iii) provide tumor markers whose assessment may be exploited during follow-up to detect tumor residual or recurrence after surgery; iv) avoid life-threatening post-operative adrenal insufficiency. Hypercortisolism may indeed escape clinical detection, thus preventing use of post-operative steroid replacement. Assessment of tumor dignity and visualization of potential metastases needs an adequate imaging study. Computerized tomography (CT) is the technique of choice but magnetic resonance imaging may be equally effective. CT characteristics suggesting malignancy are: a) large mass size, usually >4 cm; b) elevated mass density (>10 Hounsfield Units) on unenhanced scan; c) rapid washout of contrast medium on enhanced scan. Recent data demonstrate that 18F-FDG PET helps to differentiate suspicious CT scan lesions.
As to treatment, recent studies challenged the dogma that there is no role for laparoscopy, although data on the comparison between open and laparoscopic adrenalectomy remain conflicting. The crucial issue is to select an experienced surgeon. Adiuvant therapy with mitotane has recently received increased interest after publication of a multicentric study on a large series of ACC patients that showed a significant reduction in the risk of recurrence and death for the mitotane treated patients. However, controversy still exists on the value of post-operative mitotane and a randomized controlled trial is currently ongoing. Results of medical treatment of advanced disease with the available regimens are overall disappointing and also the first experiences with targeted therapies are not encouraging, but new trials are ongoing and hopefully will allow definition of a more personalized treatment.
Declaration of interest: The author declares that there is a conflict of interest.
Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.