关键词: Head and neck cancer Mucosal melanoma Nose cancer Paranasal sinus cancer Sinonasal cancer

来  源:   DOI:10.1007/s00405-024-08717-7

Abstract:
OBJECTIVE: Sinonasal mucosal melanoma (SNMM) is a rare malignancy, characterised by high (local) recurrence rates and poor survival. Comprehensive understanding of tumour etiology is currently lacking, which complicates adequate tumour treatment. Besides examining trends in incidence, this study aims to assess the association between clinical characteristics, treatment practices and patient outcomes, with the objective of establishing a baseline from which SNMM management can be enhanced.
METHODS: All newly diagnosed SNMM cases in The Netherlands between 2001 and 2021 were included using data from The Netherlands Cancer Registry (NCR).
RESULTS: A total of 320 patients were included. The annual incidence rate for the overall population was stable over the inclusion period with an annual percentage change (APC) of only - 0.01%. The 5-year overall survival (OS) and relative survival (RS) were 24.5 and 32.4%, respectively. Relative survival did not increase over time. The addition of adjuvant radiotherapy to surgery was not associated with a higher OS and RS compared to surgery alone.
CONCLUSIONS: Sinonasal mucosal melanoma is a rare disease with stable incidence rates in the Netherlands between 2001 and 2021. There has been no improvement in survival over the course of the inclusion period. The study reaffirms that adjuvant radiotherapy does not seem to improve patient outcomes. Given the generally poor outcomes for SNMM patients, novel therapeutic options ought to be considered in order to improve care.
摘要:
目的:鼻窦粘膜黑色素瘤(SNMM)是一种罕见的恶性肿瘤,以高(局部)复发率和低生存率为特征。目前缺乏对肿瘤病因的全面了解,这使得适当的肿瘤治疗复杂化。除了检查发病率的趋势,这项研究旨在评估临床特征之间的关联,治疗实践和患者结果,目的是建立可以增强SNMM管理的基线。
方法:使用荷兰癌症登记处(NCR)的数据纳入了2001年至2021年荷兰所有新诊断的SNMM病例。
结果:共纳入320例患者。在纳入期内,总体人群的年发病率是稳定的,年百分比变化(APC)仅为0.01%。5年总生存率(OS)和相对生存率(RS)分别为24.5%和32.4%,分别。相对存活率没有随时间增加。与单独手术相比,在手术中增加辅助放疗与较高的OS和RS无关。
结论:鼻窦粘膜黑色素瘤是一种罕见的疾病,在2001年至2021年期间在荷兰发病率稳定。在纳入期内,生存率没有改善。该研究重申,辅助放疗似乎并不能改善患者的预后。鉴于SNMM患者的预后普遍较差,为了改善护理,应该考虑新的治疗方案。
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