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

• Published in: The New England journal of medicine Vol. 322; no. 17; pp. 1195 - 1201
• Main Authors: Luton, Jean-Pierre, Cerdas, Sonia, Billaud, Line, Thomas, Guy, Guilhaume, Brigitte, Bertagna, Xavier, Laudat, Marie-Hélène, Louvel, Albert, Chapuis, Yves, Blondeau, Philippe, Bonnin, André, Bricaire, Henri
• Format: Journal Article
• Language: English
• Published: Boston, MA Massachusetts Medical Society 26-04-1990
• Subjects: Adrenal Cortex Hormones - secretion; Adrenal Cortex Neoplasms - drug therapy; Adrenal Cortex Neoplasms - mortality; Adrenal glands; Antineoplastic agents; Biological and medical sciences; Cancer therapies; Chemotherapy; Combined Modality Therapy; Cytotoxicity; Diagnosis; Drug dosages; Female; Follow-Up Studies; Hormones; Humans; Liver; Male; Medical prognosis; Medical sciences; Metastases; Metastasis; Middle Aged; Mitotane - adverse effects; Mitotane - therapeutic use; Multivariate analysis; Neoplasm Metastasis; Neuroendocrine tumors; Patients; Pharmacology. Drug treatments; Plasma; Prognosis; Retrospective Studies; Secretion; Surgery; Survival; Survival Rate; Tumors; Urine; Antineoplastic agent; Human; Chemotherapy; Prognosis; Treatment; Adrenal cortex diseases; Malignant tumor
• ISSN: 0028-4793; 1533-4406
• DOI: 10.1056/NEJM199004263221705

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, <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. (N Engl J Med 1990; 322:1195–201.)
ADRENOCORTICAL carcinoma is a rare tumor
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with a poor prognosis.
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Its true incidence is unknown,
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but according to the Third National Cancer Survey of 1975
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the incidence is approximately 0.5 to 2 cases per million people per year, accounting for 0.2 percent of the deaths from cancer in the United States.
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The infrequent occurrence of this carcinoma precludes extensive clinical experience with its diagnosis and treatment, and reports of more than 50 patients from single or multiple centers are exceptional.
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Surgical resection is the treatment of choice,
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but the role of adjuvant chemotherapy is controversial.
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Mitotane (
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DDD or . . .