title: [Medullary cancer of the thyroid. Apropos of 20 years' experience in France].
abstract: The majority (1013) of cases of medullary carcinoma of the thyroid observed in France in the last twenty years have been registered in a national file. Once overcome the difficulty of diagnosing the index case, all first degree parents at least should undergo a pentagastrin stimulation test and calcitonin estimation. Though such a policy involves difficulties of several types, it has resulted in the detection of 203 cases belonging to 61 families. 29 families suffered only from isolated MCT; in the 32 other families this cancer was a part of polyendocrinopathies of type 2a (28 cases) or type 2b (4 cases). The tumour was apparently of the sporadic type in 208 subjects. In 602 other cases the data were insufficient for a correct classification. An epidemiological enquiry of the disease is in progress since 1986. Though the number of complete files analysed is still insufficient, this study will be essential in understanding the natural history of the disease, the causes of its heterogeneity and in deducing eventually preventive measures even if in meantime a genetic marker is available.
mesh_terms: D002277:Carcinoma / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics; D005602:France; D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000453:epidemiology; D011379:Prognosis; D013964:Thyroid Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics
vernacular_title: Cancer médullaire de la thyroïde. A propos d'une expérience de 20 ans en France.