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美国朗格汉斯细胞肉瘤的流行病学和临床特征:基于 2000 年至 2019 年 SEER 数据的人群研究。

The epidemiological and clinical characteristics of Langerhans cell sarcoma in the United States: A population study based on SEER data from 2000 to 2019.

机构信息

Faculty of Medicine, Al Quds University, Jerusalem, Palestine.

Faculty of Medicine, University of Aleppo, Aleppo, Syria.

出版信息

Medicine (Baltimore). 2024 Aug 16;103(33):e39315. doi: 10.1097/MD.0000000000039315.

DOI:10.1097/MD.0000000000039315
PMID:39151546
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11332751/
Abstract

Langerhans cell sarcoma (LCS) is a rare aggressive malignancy with a poor prognosis. Our knowledge about this condition is limited and mainly based on case reports, making it challenging to understand its epidemiology, clinical features, and patient outcomes. We conducted a retrospective study of LCS patients diagnosed between 2000 and 2019 using the Surveillance, Epidemiology, and End Results (SEER) database. The data were stratified based on age, race, stage, clinical pattern, and treatment method. Our study found that 57 LCS cases were reported in SEER registries between 2000 and 2019. Among these cases, most patients (50.9%) were over 60 years old and White (71.9%) with almost equal males to females ratio. About 45.6% of cases were localized while 47.4% were at distant stages. Of the patients, 50.9% underwent surgery, 45.6% received chemotherapy, and only 21.1% received radiotherapy. The overall survival rate for patients diagnosed with LCS in the United States is generally low with a 1-year overall rate of 63.8%. Certain factors can negatively impact prognosis, such as advanced stages of the disease, secondary tumors, or more than 1 tumor per patient. LCS is a rare disease with poor survival rates. Future research should incorporate global data for further statistically significant results. Moreover, investigating the molecular, genetic, and pathophysiological backgrounds of these tumors is crucial for developing targeted management strategies and improving prognosis.

摘要

朗格汉斯细胞肉瘤(LCS)是一种罕见的侵袭性恶性肿瘤,预后较差。我们对这种疾病的了解有限,主要基于病例报告,因此难以了解其流行病学、临床特征和患者结局。我们使用监测、流行病学和最终结果(SEER)数据库对 2000 年至 2019 年间诊断的 LCS 患者进行了回顾性研究。根据年龄、种族、分期、临床模式和治疗方法对数据进行分层。我们的研究发现,2000 年至 2019 年间,SEER 登记处报告了 57 例 LCS 病例。在这些病例中,大多数患者(50.9%)年龄超过 60 岁,且为白人(71.9%),男女比例几乎相等。约 45.6%的病例为局限性,47.4%为远处转移。在患者中,50.9%接受了手术,45.6%接受了化疗,只有 21.1%接受了放疗。在美国,LCS 患者的总体生存率普遍较低,1 年总体生存率为 63.8%。某些因素会对预后产生负面影响,例如疾病晚期、继发性肿瘤或每个患者超过 1 个肿瘤。LCS 是一种罕见疾病,生存率较低。未来的研究应纳入全球数据,以获得更具统计学意义的结果。此外,研究这些肿瘤的分子、遗传和病理生理学背景对于制定靶向管理策略和改善预后至关重要。

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