MEDULLARY-THYROID CANCER

Medullary thyroid carcinoma (MTC) is a particularly interesting model of the gene expression in cancer. Progress in the knowledge and early cure of this rare disease benefits from a close collaboration between practioners and scientists. The description of the different forms, MTC only and multiple endocrine neoplasia (MEN II), either sporadic or familial, with the discovery of calcitonin (CT) as a specific marker of the tumor, has been improved in the last decade by different events: - The localization of the CT gene on chromosome 11, the alternative splicing of its messengers and the secretion of CGRP I, CGRP II, salmon-type CT and other hormones and peptides by MTC. - The discovery of an abnormality responsible for the familial disease linked to chromosome 10. - The improvement of the hormone assay, by the use of monoclonal antibodies, resulting in a more precocious MTC diagnosis. A nation-wide French study has allowed an extensive diagnosis of the familial form (up to 100). The concomitant use of genealogical studies resulted not only in their recognition by linkage of patients but also in joining of family branches, with diagnosis of MEN syndrome, limiting the screening to the descendants of the common ancestor... An epidemiological work is in progress for the period 1968-1988.