Management of hereditary medullary thyroid carcinoma

In this paper we have briefly outlined our views on the management of hereditary MTC. We have stressed the fact that although many patients with the usual form of disease have an indolent clinical course there exists a population of patients who may be at risk for widely disseminated virulent tumour. Also, some patients can have marked invasion of adjacent tissues in the cervical region from localized tumour. Hence it seems rational to attempt removal of the primary tumour prior to the development of regional or distant metastases. The role of the combined calcium-pentagastrin provocative test to identify patients with preclinical disease has been outlined. The only definitive treatment for the disease is total thyroidectomy with resection of adjacent cervical nodes. Many patients who are treated at a time when the disease has become clinically detectable will have residual tumour or recurrence as indicated by abnormal calcitonin levels in the blood. Since most of these patients seem to take an indolent clinical course, it is suggested that they should not be treated with radiation therapy and/or chemotherapy, even though residual MTC is known to be present. Even in patients with advanced stages of MTC, the use of chemotherapy and radiation therapy has not been encouraging. However, some aspects of the management of patients with aggressive disease are discussed. Finally, the lack of features to predict which patients may have a poor prognosis is outlined and some new approaches to defining such parameters are discussed.