Calmettes C
INSERM U113, CHU Saint-Antoine, Paris.
Ann Endocrinol (Paris). 1988;49(1):10-6.
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.
过去二十年间在法国观察到的大多数（1013例）甲状腺髓样癌病例已被录入一份国家档案。一旦克服了诊断索引病例的困难，所有一级亲属至少都应接受五肽胃泌素刺激试验和降钙素测定。尽管这样的政策存在多种困难，但它已导致发现了属于61个家族的203例病例。29个家族仅患有散发性甲状腺髓样癌；在其他32个家族中，这种癌症是2a型（28例）或2b型（4例）多发性内分泌病的一部分。在208名受试者中，肿瘤显然为散发性类型。在其他602例病例中，数据不足以进行正确分类。自1986年以来一直在对该疾病进行流行病学调查。尽管已分析的完整档案数量仍然不足，但这项研究对于了解该疾病的自然史、其异质性的原因以及最终推断预防措施至关重要，即使在此期间有可用的遗传标记。
