肉瘤是罕见的癌症，分为软组织、骨骼和软骨等多种亚型。这些癌症在国际上的生存趋势尚不为人所知。我们在丹麦（DK）、芬兰（FI）、挪威（NO）和瑞典（SE）展示了软组织肉瘤（STS）和骨骼肉瘤（BS）的50年生存趋势。我们从NORDCAN数据库中获取了1971年至2020年的1年、5/1条件和5年生存数据。我们还估计了生存率的年度变化，并确定了显著的转折点。 在最后一个时期，即2016年至2020年，NO男性（74.6%）和FI女性（71.1%）的STS 5年生存率最高。对于较为罕见的BS，SE男性（72.0%）和DK女性（71.1%）的生存率最佳。在1971年至1975年期间，BS的生存率低于STS，并且在2016年至2020年期间男性之间的差异仍然存在，但对于女性而言，这两者的生存率几乎相等。在STS的生存中，性别和国家之间的差异很小。在NO男性中，5年生存率的50年改善最高，为34.0个百分点，FI女性为30.0个百分点。在BS中，SE男性为26.2个百分点，FI女性为29.2个百分点。在半个世纪的时间里，生存率的稳定发展表明了诊断、治疗和护理逐步改进的贡献。第一年的10-15%死亡率可能表示了诊断延误，而通过为进展性罕见疾病组织患者提供途径，可以改善这种情况。早期诊断还会减少转移性疾病，而治疗的突破是当前的挑战。 版权所有©2023作者。由Elsevier Ltd.发表。保留所有权利。

Sarcomas are rare cancers with many subtypes in soft tissues, bone and cartilage. International survival trends in these cancers are not well known. We present 50-year survival trends for soft tissue sarcoma (STS) and bone sarcoma (BS) in Denmark (DK), Finland (FI), Norway (NO) and Sweden (SE).Relative 1-, 5/1 conditional- and 5-year survival data were obtained from the NORDCAN database for years 1971-20. We additionally estimated annual changes in survival rates and determined significant break points.In the last period, 2016-20, 5-year survival in STS was best for NO men (74.6%) and FI women (71.1%). For the rarer BS, survival rates for SE men (72.0%) and DK women (71.1%) were best. Survival in BS was lower than that in STS in 1971-75 and the difference remained in 2016-20 for men, but for women the rates were almost equal. Sex- and country-specific differences in survival in STS were small. The 50-year improvement in 5-year survival in STS was highest in NO men, 34.0 % units and FI women, 30.0 % units. The highest improvements in BS were in SE men 26.2 % units and in FI women 29.2 % units.The steady development in survival over the half century suggests contribution by stepwise improvements in diagnostics, treatment and care. The 10-15% mortality in the first year probably indicates diagnostic delays which could be improved by organizing patient pathways for aggressive rare diseases. Early diagnosis would also reduce metastatic disease and breakthroughs in treatment are a current challenge.Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.