Background: To date, only a few population-representative studies have been carried out on the rare Merkel cell carcinoma (MCC). We provide incidence and survival estimates of MCC, including the conditional relative survival. Methods: We analyzed data from the cancer registry of North Rhine-Westphalia, Germany, 2008–2021, covering a population of 18 million. We included all newly diagnosed MCCs and calculated age-standardized (old European Standard population) incidence rates and unconditional and conditional relative survival. Results: Our analysis included 2164 MCC patients. The age-standardized incidence of MCC was 5.2 (men) and 3.8 (women) per million person-years. The 5-year relative survival was 58.8% (men) and 70.7% (women). Survival was lower among men than women in all age–sex groups and was highest for MCC of the upper extremity in both men (68.2%) and women (79.3%). The sex difference in survival is particularly due to the better survival of women with MCC of the head and neck. In terms of survival, the first two years are particularly critical. Conclusions: Our data validate the worse survival among men and highlights a more favorable prognosis for MCCs located on the limbs. The first two years after diagnosis of MCC are the years with the highest excess mortality.
背景:迄今为止,针对罕见皮肤神经内分泌癌(MCC)开展的具有人群代表性的研究为数不多。本研究提供了MCC的发病率和生存率评估,包括条件相对生存率。方法:我们分析了德国北莱茵-威斯特法伦州癌症登记处2008-2021年的数据,覆盖人口达1800万。纳入所有新诊断的MCC病例,计算年龄标准化(按欧洲旧标准人口)发病率、无条件及条件相对生存率。结果:本研究共纳入2164例MCC患者。MCC年龄标准化发病率男性为每百万人年5.2例,女性为3.8例。5年相对生存率男性为58.8%,女性为70.7%。所有年龄-性别组中男性生存率均低于女性,其中上肢MCC生存率在男性(68.2%)和女性(79.3%)中均为最高。生存率的性别差异主要源于女性头颈部MCC患者生存状况更佳。在生存预后方面,确诊后的前两年尤为关键。结论:我们的数据证实了男性患者生存预后较差,并提示四肢部位MCC具有更良好的预后。MCC确诊后的前两年是超额死亡率最高的时期。