Cancer Epidemiology Unit, National Center for Epidemiology, Surveillance and Health Promotion, Istituto Superiore di Sanità, Viale Regina Elena 299, Rome, Italy.
Eur J Cancer. 2012 May;48(8):1167-75. doi: 10.1016/j.ejca.2011.10.004. Epub 2011 Nov 25.
This work provides descriptive epidemiological data of malignant mucosal and uveal melanomas and adnexal skin carcinomas in Europe as defined as in the RARECARE project. We analysed 8669 incident cases registered in the period 1995-2002 by 76 population-based cancer registries (CRs), and followed up for vital status to 31st December 2003. Age-standardised incidence to the European standard population was obtained restricting the analysis to 8416 cancer cases collected by 64 not specialised CRs or with information available only for some anatomical sites. Period survival rates at 2000-2002 were estimated on 45 CRs data. Twenty-two CRs which covered the period 1988-2002 were analysed to obtain the 15-year prevalence (1st January 2003 as reference date). Complete prevalence was calculated by using the completeness index method which estimates surviving cases diagnosed prior to 1988 ('unobserved' prevalence). The expected number of new cases per year and of prevalent cases in Europe was then obtained multiplying the crude incidence and complete prevalence rates to the European population at 2008. We estimated 5204 new cases per year (10.5 per million) to occur in Europe, of which 48.7% were melanomas of uvea, 24.8% melanomas of mucosa and 26.5% adnexal carcinomas of the skin. Five-year relative survival was 40.6% and 68.9% for mucosal and uveal melanomas, respectively. Adnexal skin carcinomas showed a good prognosis with a survival of 87.7% 5 years after diagnosis. Northern Europe, United Kingdom (UK) and Ireland showed the highest 5-year survival rate for uveal melanomas (72.6% and 73.4%), while Southern Europe showed the lowest rate (63.7%). More than 50,000 persons with a past diagnosis of one of these rare cancers were estimated to be alive at 2008 in Europe, most of them (58.8%, n=29,676) being patients with uveal melanoma. Due to the good prognosis and high incidence of uveal melanomas, these malignancies are highly represented among the long-term survivors of the studied rare cancer types. Therefore, maximising quality of life is particularly important in treatment of uveal melanoma. As regards mucosal melanomas, the centralisation of treatment to a select number of specialist centres as well as the establishment of expert pathology panels should be promoted. The geographical differences in incidence and survival should be further investigated analysing the centre of treatment, the stage at diagnosis and the treatment.
这项工作提供了在欧洲定义的恶性黏膜和葡萄膜黑色素瘤以及附属皮肤癌的描述性流行病学数据。我们分析了 1995 年至 2002 年间由 76 个基于人群的癌症登记处（CR）登记的 8669 例新发病例，并随访至 2003 年 12 月 31 日的生存状态。通过将分析限于 64 个非专门的 CR 收集的 8416 例癌症病例或仅提供部分解剖部位信息，获得了欧洲标准人群的年龄标准化发病率。在 45 个 CR 数据上估计了 2000-2002 年的期间生存率。对 22 个覆盖 1988-2002 年期间的 CR 进行了分析，以获得 15 年的患病率（以 2003 年 1 月 1 日为参考日期）。通过使用完整指数方法计算完整的患病率，该方法估计了在 1988 年之前诊断出的存活病例（“未观察到”的患病率）。然后，将每年的新发病例数和欧洲的现有病例数乘以欧洲 2008 年的人口，即可得出每年新发病例数和欧洲现有病例数。我们估计每年在欧洲会发生 5204 例新发病例（每百万 10.5 例），其中 48.7%为葡萄膜黑色素瘤，24.8%为黏膜黑色素瘤，26.5%为皮肤附属器癌。黏膜和葡萄膜黑色素瘤的 5 年相对生存率分别为 40.6%和 68.9%。皮肤附属器癌的预后较好，诊断后 5 年的生存率为 87.7%。北欧、英国（UK）和爱尔兰的葡萄膜黑色素瘤 5 年生存率最高（72.6%和 73.4%），而南欧的生存率最低（63.7%）。在欧洲，到 2008 年，估计有超过 50000 人患有这些罕见癌症之一，其中大多数（58.8%，n=29676）是葡萄膜黑色素瘤患者。由于葡萄膜黑色素瘤的预后良好且发病率较高，因此这些恶性肿瘤在研究的罕见癌症类型的长期幸存者中所占比例很高。因此，在治疗葡萄膜黑色素瘤时，提高生活质量尤为重要。至于黏膜黑色素瘤，应将治疗集中到少数专门中心，并建立专家病理学小组。发病率和生存率的地理差异应进一步通过分析治疗中心、诊断时的阶段和治疗来研究。
