Clinical Features of Adrenocortical Carcinoma, Prognostic Factors, and the Effect of Mitotane Therapy

Abstract

Adrenocortical carcinoma is a rare tumor, and only limited information is available about its natural history and the effects of therapy. We studied 105 patients (75 female and 30 male; mean age, 46 years) with adrenocortical carcinoma who were referred to us between 1963 and 1987. The average duration of symptoms before diagnosis was 8.7 months. At the time of diagnosis, 68 percent of the patients had endocrine symptoms, and 30 percent had distant metastases. Hormonal studies showed that 79 percent of the tumors were functional. Eighty patients underwent surgery, and 59 also received the adrenal cytotoxic agent mitotane. The median disease-free interval after surgery was 12.1 months (range, 1 to 175). Tumor dissemination occurred in 82 percent of the patients, most commonly to the lung, liver, and adjacent organs. The median survival time was 14.5 months (range, less than 1 to 175), and the five-year survival was 22 percent. Age over 40 years and the presence of metastases at the time of diagnosis were the only factors recognized as indicating a poor prognosis. Mitotane controlled hormonal secretion in 75 percent of the patients. Eight mitotane-treated patients had partial tumor regression, but the drug did not have a significant effect on survival. We conclude that adrenocortical carcinoma carries a poor prognosis. Mitotane therapy may offer transient benefits, particularly in controlling endocrine symptoms.

Cytotoxic therapy with etoposide and cisplatin in advanced adrenocortical carcinoma

“…Adrenocortical carcinoma (ACC) is a rare malignant tumour with a poor prognosis (Hutter and Kayhoe, 1966;Bertagna and Orth, 1981;Didolkar et al 1981;Luton et al, 1990). An aggressive surgical approach provides disease control for the minority of patients presenting with localized disease.…”

“…An aggressive surgical approach provides disease control for the minority of patients presenting with localized disease. In patients with advanced disease, survival is < 50% at I year and < 10% at 5 years (Luton et al, 1990;Jensen et al, 1991;Icard et al, 1992;Pommier and Brennan, 1992).…”

“…Measurement of serum mitotane level has been available since 1996; serum mitotane level was above 14 mg 1-1 in six of the nine patients in whom it was measured. Mitotane (Pharmacie Centrale de l'Assistance Publique-Hopitaux de Paris) was given by mouth as capsules containing 0.5 g of micronized mitotane mixed with cellulose acetylphthalate (Luton et al, 1990). This provides a good digestive tolerance but a low bioavailability.…”

Characterization of a newly established cell line derived from human adrenocortical carcinoma

“…12 Feller et al reported that treatment with a combination of multiple-resistance drugs plus mitotane yielded no significant effects in vivo. 1 Therefore, we must analyze the oncogenesis of adrenocortical carcinoma more deeply and develop a good model to help us establish an effective new treatment.…”

Adrenocortical carcinoma presenting as varicocele and renal vein thrombosis: a case report

“…Approximately 60% of ACCs are functional tumors [2]. Patients can present with Cushing's syndrome alone (45%), a mixed Cushing's and virilization syndrome (25%), or virilization alone (< 10%) [3].…”