Bergholm U, Adami H O, Telenius-Berg M, Johansson H, Wilander E
Department of Surgery, University Hospital, Uppsala, Sweden.
Acta Oncol. 1990;29(1):9-15. doi: 10.3109/02841869009089985.
Medullary thyroid carcinoma (MTC) was identified in 276 patients (in 27 diagnosed at autopsy) by a review of virtually all 6,513 notifications of primary thyroid cancer ot the National Cancer Registry in Sweden 1959 through 1981. The diagnosis was confirmed in 268 of the 276 cases by histopathological and histochemical reexamination. Anamnestic data and morphological characteristics indicated that 208 (75%) patients had sporadic and 68 (25%) familial MTC. The mean ages at diagnosis of these two groups were 57.0 and 42.6 years respectively. The age-standardized incidence rate per 10(5) inhabitants was 0.18 for males and 0.23 for females. The age-specific incidence of sporadic MTC increased markedly with age, whereas no unequivocal rise was found after the age of 20 for familial disease. Standardized morbidity ratios (SMR), calculated separately for each of the six Swedish health care regions, revealed a roughly two-fold and mostly non-significant geographical variation in the occurrence of sporadic MTC. SMR for familial disease varied, however, between 0 and 306 and deviated highly significantly from the national average in four of the six regions. Regional differences in diagnostic intensity were considered unlikely as the sole explanation of this finding.
通过回顾1959年至1981年瑞典国家癌症登记处收到的几乎所有6513份原发性甲状腺癌报告，在276例患者中确诊为髓样甲状腺癌（MTC）（其中27例为尸检确诊）。276例中的268例经组织病理学和组织化学复查确诊。既往史资料和形态学特征表明，208例（75%）患者为散发性MTC，68例（25%）为家族性MTC。这两组患者的平均诊断年龄分别为57.0岁和42.6岁。每10万居民的年龄标准化发病率男性为0.18，女性为0.23。散发性MTC的年龄别发病率随年龄显著增加，而家族性疾病在20岁以后未发现明确上升。分别对瑞典六个医疗保健区域计算的标准化发病比（SMR）显示，散发性MTC的发生率存在大致两倍且大多无统计学意义的地理差异。然而，家族性疾病的SMR在0至306之间变化，在六个区域中的四个区域与全国平均水平有高度显著差异。诊断强度的区域差异不太可能是这一发现的唯一解释。
