Staging Surgical Indications and Preoperative Treatment

Adrenocortical carcinomas are classified according to stages described by MacFarlane and modified by Sullivan (Table 69-3).

This classification has one major drawback (i.e., malignancy in stage I is based on histologic criteria only). Whether all of these tumors are malignant is unknown, and the assumption that all are malignant may lead to an overly optimistic affirmation of the results of surgery.

All tumors at stage I, II, or III, whether diagnosed preop-eratively or intraoperatively, should be resected. The need to

1 TABLE 69-

-3 Staging of Adrenocortical Carcinomas


Size (cm)

Weight (g)

Local Extension

Lymph Node Extension Distant Metastasis

n in


<5 and >5 or

<50 >50

None None + or + and

None None None None + None

+ None — +

Adapted from MacFarlane D. Cancer of the adrenal cortex: The natural history, prognosis, and treatment in a study of 55 cases, Ann R Coll Surg Engl 1958:23:155.

operate on patients with stage IV disease and distant metastases is controversial because these patients have an average postoperative survival of 3 months and a 1-year actuarial survival of 10%. Widespread metastases in elderly patients should dissuade against surgical treatment. Conversely, in young patients, a solitary metastasis should not be a contraindication to surgery, and in rare cases pre- and postoperative adjunctive chemotherapy has provided long-lasting survival with complete remission.

Preoperative treatment with mitotane (8 to 12 g/day) is indicated in two situations: metastatic disease and severe hyper-cortisolism. Mitotane successfully treats Cushing's syndrome in up to 75% of patients24 and sometimes causes partial or dramatic shrinkage of the primary tumor and the metastases. Cortisol replacement therapy is essential because hypocorti-solism results in some patients. Unfortunately, many patients cannot tolerate the nausea and other side effects of mitotane, which limits its successful application. We recommend using mitotane for 3 or 4 weeks before surgery, and patients who respond to mitotane have a more favorable prognosis.

Mitotane has a long half-life, and monitoring of serum levels can allow a lower maintenance dose for better tolerance. Alternatively, ketoconazole (400 mg/day) can be used to control the hypercortisolism.

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