Department of Registry and Research, Comprehensive Cancer Centre the Netherlands, Catharijnesingel 55--h Utrecht, The Netherlands.
Eur J Cancer. 2012 Sep;48(13):1923-31. doi: 10.1016/j.ejca.2012.01.029. Epub 2012 Feb 21.
The rarity or the asymptomatic character of endocrine tumours results in a lack of epidemiological studies on their incidence and survival patterns. The aim of this study was to describe the incidence, prevalence and survival of endocrine tumours using a large database, which includes cancer patients diagnosed from 1978 to 2002, registered in 89 population-based cancer registries (CRs) with follow-up until 31st December 2003. These data give an unique overview of the burden of endocrine carcinomas in Europe. A list of tumour entities based on the third International Classification of Diseases for Oncology was provided by the project Surveillance of rare cancer in Europe (RARECARE) project. Over 33,594 cases of endocrine carcinomas were analysed in this study. Incidence rates increased with age and were highest in patients 65 years of age or older. In 2003, more than 315,000 persons in the EU (27 countries) were alive with a past diagnosis of a carcinoma of endocrine organs. The incidence of pituitary carcinoma equalled four per 1,000,000 person years and showed the strongest decline in survival with increasing age. Thyroid cancer showed the highest crude incidence rates (four per 100,000 person years) and was the only entity with a gender difference: (female-to-male ratio: 2:9). Parathyroid carcinoma was the rarest endocrine entity with two new cases per 10,000,000 person years. For adrenal carcinoma, the most remarkable observations were a higher survival for women compared to men (40% compared to 32%, respectively) and a particularly low relative survival of 24% in patients 65 years of age or older. More high quality studies on rare cancers, with additional information, e.g. on stage and therapeutic approach, are needed and may be of help in partly explaining the observed variation in survival.
内分泌肿瘤的罕见性或无症状特征导致缺乏关于其发病率和生存模式的流行病学研究。本研究的目的是使用一个大型数据库描述内分泌肿瘤的发病率、患病率和生存率，该数据库包含 1978 年至 2002 年期间诊断的癌症患者，这些患者在 89 个基于人群的癌症登记处（CR）中登记，并随访至 2003 年 12 月 31 日。这些数据提供了欧洲内分泌癌负担的独特概述。肿瘤实体清单是基于第三版国际肿瘤疾病分类（ICD-O）由欧洲罕见癌症监测项目（RARECARE）项目提供的。本研究分析了超过 33594 例内分泌癌病例。发病率随年龄增长而增加，在 65 岁及以上的患者中最高。2003 年，欧盟（27 个国家）有超过 315000 人过去被诊断患有内分泌器官癌。垂体癌的发病率为每 100 万人中有 4 例，并且随着年龄的增长，生存率下降幅度最大。甲状腺癌的粗发病率最高（每 10 万人中有 4 例），并且是唯一存在性别差异的实体：（女性与男性的比例为 2:9）。甲状旁腺癌是最罕见的内分泌实体，每 1000 万人中有 2 例新发病例。对于肾上腺癌，最显著的观察结果是女性的生存率高于男性（分别为 40%和 32%），以及 65 岁及以上患者的相对生存率特别低（24%）。需要更多高质量的罕见癌症研究，这些研究应提供更多信息，例如分期和治疗方法，这可能有助于部分解释观察到的生存率差异。
