Treatment

The mainstay of treatment in all these tumours is surgical resection. This is the only treatment likely to achieve cure. Before considering surgery it is necessary to carry out staging investigations, as already described, to exclude the presence of metastases. It may still be appropriate to resect the primary tumour in the presence of metastases if it is slow-growing or where there are a small number of metastases, in order to achieve local control. The surgical resection of phaeochromo-cytomas requires a skilled multidisciplinary team approach. As a rule, surgery for adrenocortical tumours is less hazardous, but patients with electrolyte disturbances require careful preparation prior to surgery. Following surgery, persistent biochemical disturbance indicates residual disease. This is best handled by a multidisciplinary experienced team.

The identification of the gene causing MEN has led to the screening of affected families and prophylactic surgery in children. When performed in specialist units this carries minimal morbidity. Thyroid surgery for MCT is carried out at 5-7 years of age.

For phaeochromocytomas with residual or unresectable disease, anti-hypertensive medication may be required to control the blood pressure. If the tumour takes up the radionuclide MIBG, a therapeutic dose of up to 10 000 MBq of 131I-MIBG may be administered and repeated at 3-6 monthly intervals. A number of tumours will not take up MIBG and consideration should be given to chemotherapy. These tumours were traditionally considered to be chemo-resistant, but a combination of DTIC, vincristine, and cyclophosphamide shows activity. Other regimens which include cisplatin and etoposide have shown promise.

For unresectable or metastatic adrenocortical tumours, Mitotane (opDDD) is the first-line treatment. This drug may be associated with unpleasant symptoms and the dosage is usually built up slowly, to a maximum of 10-12 g daily. Control of the disease is transient and after 6-12 months there is evidence of biochemical progression or symptoms return. Metyrapone, aminoglutethamide, and ketocona-zole are second-line medical therapies.

Chemotherapy is of limited benefit. There are no randomized clinical trials, but the most active drugs appear to be cisplatin and eto-poside. There are anecdotal reports of long-term remission. The role of external radiotherapy is limited to the treatment of symptomatic metastases, usually in bone.

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