Adrenocortical carcinoma:

A. May be suspected in a patient with rapidly progressive Cushing's syndrome and virilizing features or in asymptomatic patients with adrenal tumors larger than 6 cm on CT.

E. Carries a poor prognosis: overall 5-year survival less than 25%.

DISCUSSION: Patients with adrenocortical carcinoma often present with rapidly progressive syndromes of combined adrenocortical hormone excess. However, approximately half do not have syndromes of hormone excess but present with abdominal pain, increased abdominal girth, weight loss, and anorexia. Furthermore, incidentally discovered adrenal masses larger than 6 cm. harbor carcinoma in as many as 92% of cases. Adrenocortical carcinoma is differentiated from adenoma by pathologic demonstration of either local invasion or distant metastases. Most patients with adrenocortical carcinoma present with locally advanced (stage III) or metastatic (stage IV) disease. Prognosis for these patients is poor: 5-year survival is less than 25% in most series. Patients with adrenocortical carcinoma should undergo primary surgical resection or palliative surgical debulking of locally advanced, metastatic, or even recurrent disease since response of these tumors to medical therapy, including mitotane, is poor.