Zaar O, Gillstedt M, Lindelöf B, Wennberg-Larkö A-M, Paoli J
Department of Dermatology and Venereology, Sahlgrenska University Hospital, Institute of Clinical Sciences at the Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
Department of Dermatology, Karolinska University Hospital, Stockholm, Sweden.
J Eur Acad Dermatol Venereol. 2016 Oct;30(10):1708-1713. doi: 10.1111/jdv.13698. Epub 2016 May 2.
Merkel cell carcinoma (MCC) is a rare aggressive neuroectodermal skin cancer with a high recurrence rate and a high mortality rate. Risk factors for MCC are reported to include high age, UV exposure, Caucasian skin type and immunosuppression. The incidence is reported to be increasing.
The purpose of this study was to describe a Swedish cohort and calculate incidence.
The study design is a retrospective cohort study of population-based data for MCC collected by the Swedish Cancer Registry to determine the incidence of MCC in Sweden and the clinical characteristics of these tumours including demographics, TNM classification, body part distribution and overall survival after diagnosis. De-identified data were collected from 1993 to 2012.
A total of 606 cases of MCC were identified during the study period. The median age was 81 years (range 21-99) and a majority, 54.4% were women but age-adjusted incidence is higher in men. The incidence (per 100,000) of MCC in Sweden in 1993-2012 increased from 0.09 to 0.20 for men and 0.12-0.17 for women, adjusted for age to the world standard population. For the both sexes, the increase was from 0.11 to 0.19 per 100 000, an increase of 73%. The most common site of the primary tumour was the head and neck, with 51.8% of the cases. The size of the tumour was <5 cm in 82.1% of the cases. The majority of the tumours (90.7%) had no known lymphatic spread and only a few patients had confirmed distant metastases (2.9%) when diagnosed.
MCC is a rare disease in Sweden, but the incidence is increasing. This study supports the finding that high age, male sex and UV exposure are risk factors for MCCs. Interventions are required to increase awareness of MCC among clinicians and the public.
默克尔细胞癌（MCC）是一种罕见的侵袭性神经外胚层皮肤癌，复发率和死亡率都很高。据报道，MCC的风险因素包括高龄、紫外线暴露、白种人皮肤类型和免疫抑制。据报道，其发病率正在上升。
本研究旨在描述一个瑞典队列并计算发病率。
本研究设计为一项回顾性队列研究，基于瑞典癌症登记处收集的MCC人群数据，以确定瑞典MCC的发病率以及这些肿瘤的临床特征，包括人口统计学、TNM分类、身体部位分布和诊断后的总生存率。从1993年至2012年收集了去识别化的数据。
在研究期间共识别出606例MCC病例。中位年龄为81岁（范围21 - 99岁），大多数（54.4%）为女性，但经年龄调整后男性的发病率更高。1993 - 2012年瑞典MCC的发病率（每10万人），经年龄调整至世界标准人群后，男性从0.09增至0.20，女性从0.12增至0.17。对于两性而言，每10万人的发病率从0.11增至0.19，增长了73%。原发性肿瘤最常见的部位是头颈部，占病例的51.8%。82.1%的病例肿瘤大小<5厘米。大多数肿瘤（90.7%）没有已知的淋巴转移，诊断时只有少数患者有确诊的远处转移（2.9%）。
MCC在瑞典是一种罕见疾病，但发病率正在上升。本研究支持高龄、男性和紫外线暴露是MCC风险因素这一发现。需要采取干预措施以提高临床医生和公众对MCC的认识。
