Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy

Article Abstract:

Adrenocortical carcinoma, cancer of the cortex of the adrenal gland (a major producer of hormones), is rare and usually fatal. Because its incidence is low (0.5 to 2 cases per million per year), knowledge about its diagnosis and treatment is limited. To add to this knowledge, 105 patients during the years 1963 to 1987 with this diagnosis were studied. Eighty-eight underwent surgery, of whom 59 received therapy with mitotane, the only drug known to improve survival or reduce metastatic spread of the cancer. Eleven patients had second operations, and eight were considered to have inoperable tumors. The 59 patients also received cortisol, a hormone, and another anti-cancer drug. Four patients underwent chemotherapy, 11 received radiotherapy, and 14 took aminoglutethimide (a drug that suppresses adrenal hormones). In terms of prevalence, results showed that the female/male ratio for this cancer was 2.5, with an average age of 46. Seventy-one patients had endocrine symptoms representing excess glucocorticoids, androgens, both, mineralocorticoids, or estrogens (all hormones produced by the adrenal gland). Tumors were equally likely to be located in the left or right adrenal gland. In 57 patients, surgery appeared to be curative, and their average disease-free interval was 12.1 months. The most common site of tumor metastasis was the liver, followed by the lung and tissues adjacent to the adrenal glands. Sixty-seven patients died with metastatic disease. Median overall survival time was 14.5 months. Mitotane produced only transient regression of tumors and its side effects necessitated discontinuance in 12 patients. Overall, it appears that mitotane and aggressive surgery offer the best options currently available for prolonging life temporarily in patients with adrenocortical carcinoma. (Consumer Summary produced by Reliance Medical Information, Inc.)

Evaluation, Prognosis, Antineoplastic agents, Chemotherapy, Hormone antagonists, Endocrine gland tumors, Endocrine gland cancer, Mitotane

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Food-dependent Cushing's syndrome mediated by aberrant adrenal sensitivity to gastric inhibitory polypeptide

Article Abstract:

Food-dependent adrenal hyperplasia, or adrenal gland enlargement, and Cushing's syndrome may be caused by increased adrenal gland sensitivity to gastric inhibitor polypeptide (GIP) in some patients. Cushing's syndrome is a disorder characterized by increased secretion of the corticosteroid hormone cortisol by the adrenal glands. Evaluation of a 49-year-old woman with nodular adrenal hyperplasia and Cushing's syndrome found that her blood levels of cortisol were below normal during fasting, but above normal after a mixed meal. Her blood levels of cortisol increased after ingestion of glucose (sugar), protein or fat. Administration of intravenous GIP increased her blood levels of cortisol by a factor of four, but this did not occur in healthy individuals. Treatment with octreotide inhibited the secretion of cortisol in the patient following a meal. Her condition improved after three months' treatment with octreotide.

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New causes of Cushing's syndrome

Article Abstract:

Patients with spontaneous Cushing's syndrome usually either have a form of the disorder that is corticotropin-dependent or a form that is corticotropin-independent. Cushing's syndrome is a disorder characterized by increased secretion of the corticosteroid hormone cortisol by the adrenal glands. Both patients with a corticotropin-dependent and corticotropin-independent form of the disease can develop bilateral nodular adrenocortical hyperplasia, or enlargement of the adrenal glands with multiple nodules. Two research studies have described patients with corticotropin-independent Cushing's syndrome and adrenal hyperplasia that may be caused by increased response to gastric inhibitory polypeptide (GIP). The increased blood levels of GIP that occurred after eating appeared to stimulate increased secretion of cortisol in both patients.

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