Delisle M J, Gardet P, Laplanche A, Rougier P, Parmentier C
Bull Cancer. 1984;71(3):165-71.
Medullary thyroid carcinoma is diagnosed with difficulty and at an advanced stage as a consequence of its rarity and its relatively recent knowledge. Therefore, the extent of the disease is already important at the therapeutic stage and the chance of cure is poor. One hundred and thirty four cases treated in the Gustave Roussy Institut (Villejuif) and the Jean Godinot Institut (Reims) between 1942 and 1983 have been studied retrospectively. One hundred and seventeen of them diagnosed after 1960 have been analyzed. We have pointed out the clinical features which should lead to diagnosis and with present means should assure it with the minimum of delay. We have outlined the multiple endocrine neoplasia syndromes IIa and IIb and repeated the necessity of clinical and biological familial screening, 11 per cent of cases are inherited.
由于甲状腺髓样癌罕见且人们对其了解相对较新，所以其诊断困难且往往在晚期才得以确诊。因此，在治疗阶段疾病范围就已很严重，治愈的机会也很渺茫。对1942年至1983年间在古斯塔夫·鲁西研究所（维勒瑞夫）和让·戈迪诺研究所（兰斯）接受治疗的134例病例进行了回顾性研究。其中117例于1960年后确诊并进行了分析。我们指出了应能导向诊断的临床特征，并且就目前的手段而言，应能以最短的延迟确保诊断。我们概述了多发性内分泌肿瘤综合征IIa和IIb，并再次强调了临床和生物学家族筛查的必要性，11%的病例是遗传性的。
