Adrenocortical carcinoma is a rare malignant disease with a dismal prognosis and an estimated incidence of 0.5 cases per 1 million individuals per year.45 Patients with nonfunctioning tumors have manifestations attributable to a large abdominal mass. Forty percent to 70% of adrenocortical carcinomas are secretory,4546 and these patients usually present with clinical features of hormone excess. The clinical features depend on the predominant excess steroid production: glucocorticoid-secreting tumors cause Cushing's syndrome; androgen-secreting tumors lead to virilization; mineralocorticoid-secreting tumors cause hypertension and hypokalemia; and estrogen-secreting tumors result in gynecomastia and testicular atrophy in men and menstrual irregularities and precocious puberty in girls.45,46 Diagnosis is confirmed by elevated levels of urinary steroids. Eighty percent of patients demonstrate a suprarenal mass on CT scan.45-46 Pooled data from several institutions confirm that more than 60% of adrenal tumors are stage IV with local invasion, positive lymph nodes, or distant metastasis at initial diagnosis.47
Complete surgical excision, which may include a nephrectomy, is the only therapy that offers an opportunity of cure, but recurrence rates are up to 73% and 5-year survival may reach only 37%.48 Symptoms are largely proportional to tumor bulk; therefore, resection of hepatic metastases and excision of peritoneal and omental implants may be indicated for symptom control.
Mitotane (o,//-DDD) is an insecticide that causes necrosis of the adrenal cortex and has been used in the therapy of adrenocortical carcinomas. Response to therapy can be measured by objective decreases in tumor size or by changes in the levels of circulating hormones. Biochemical response rates are high but do not necessarily correlate with objective decreases in tumor size.45 Treatment toxicity includes gastrointestinal and neuromuscular symptoms.
Experience with other chemotherapeutic agents is limited. Antitumor responses have been reported in patients treated with etoposide and cisplatin. One of 11 patients showed a complete response and 2 of 11 showed partial responses with 1- and 2-year survival rates of 18% and 9%, respectively.49 A subsequent phase II trial was unable to improve on these results, with only a 13% response rate.50 The combination of doxorubicin, vincristine, and etoposide with daily mitotane yielded a response rate of 22%, but the authors noted that it is uncertain whether this is superior to mitotane alone.51
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