Clinical management of malignant adrenal tumors

Malignant primary adrenal tumors are rare forms of cancer with an estimated incidence of two to ten new cases per one million inhabitants per year. Th e 5-year survival rate for adrenocortical carcinoma is approximately 35%, w hereas the 10-year survival rate of malignant pheochromocytoma reaches 40%. Clinical studies support repeated surgery as the mainstay of treatment, ei ther with curative or palliative intention. For adrenocortical carcinoma. a djunctive treatment with oral mitotane leads to well-documented improvement of survival. Rare malignant pheochromocytomas with distant metastases are preferably treated by I-131- MIBG. Chemotherapy is reserved for unresectabl e tumors without sufficient response to mitotane or (131)-I-MIBG, respectiv ely. Cisplatin and etoposide as single therapy. or in combination with doxo rubicine or etoposide. appear to be effective in adrenocortical carcinoma. Malignant pheochromocytoma may be treated with vincristine, dacarbazine, an d cyclophosphamide. Treatment with octreotide is currently being evaluated. Radiotherapy is indicated if unresectable tumor masses cause local symptom s. If symptoms of endocrine activity are not sufficiently controlled by mea sures aiming at tumor mass reduction, specific inhibitors of hormone synthe sis or action are available. Ketoconazole is widely used for adrenocortical carcinoma, and phenoxybenzamine and metyrosine are available for malignant pheochromocytoma. This review provides guidelines for rational disease man agement based on still scanty clinical evidence.