11132例脂肪肉瘤患者的临床表现、治疗和结局：一项来自NETSARC+注册的基于人群的研究

IF 13 Q1 HEALTH CARE SCIENCES & SERVICES

Lancet Regional Health-Europe Pub Date : 2025-07-31 DOI:10.1016/j.lanepe.2025.101403

Jean-Yves Blay , Maud Toulmonde , Thibaud Valentin , Nelly Firmin , Sarah Watson , Florence Duffaud , Emmanuelle Bompas , Maria Rios , Mikael Ropars , Philippe Anract , Armelle Dufresne , Justine Gantzer , Louis-Romée Le Nail , Esma Saada , Alice Hervieu , Francois Bertucci , Pascale Dubray-Longeras , Vincent Crenn , Christophe Perrin , Pauline Soibinet , Axel Le Cesne

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引用次数: 0

摘要

背景脂肉瘤（LPS）是最常见的肉瘤之一，但聚集了多种罕见到超罕见的分子亚型，其表现和自然历史部分表征。这项工作的目的是描述来自NETSARC+注册表的不同LPS组织型的表现和结果。方法：netsarc + (netsarc.org)是由26个具有专业多学科肿瘤委员会（MDTB）的参考肉瘤中心组成的网络，自2010年以来由法国INCA资助，旨在提高肉瘤患者的护理质量。患者的特征、治疗和结果被收集在一个全国性的数据库中。本研究描述了2010年至2023年在NETSARC+数据库中整合的所有经中心综述病理综述证实的LPS的结果。发现11132例脂肪肉瘤被纳入数据库，估计发病率为11.5/106/年。中位年龄65岁（第一季至第三季：53-74岁，范围0-97岁），男性6529例（58.7%），去分化（DDLPS） 4220例（37.9%），高分化（wdlp） 1838例（16.5%）；非典型脂肪瘤（ALT） 2424例（21.8%），黏液样脂多糖（MyxLPS） 1371例（12.3%），多形性脂多糖（PLPS） 450例（4.0%），高级别黏液样脂多糖（HGMLPS） 177例（1.6%），混合型脂肪肉瘤（MTLPS） 24例（0.2%），黏液样多形性脂多糖（MPLPS） 14例（0.1%），非分类脂多糖（NCLPS） 614例（5.5%）。不同组织类型的年龄、性别和部位不同，但总体而言，所有组织类型在所有年龄组和部位都有代表。我们首先报道了不同年龄组脂肪肉瘤的性别比例差异。女性在50岁之后，在DDLPS、MyxLPS和HGMLPS中，脂肪肉瘤的发生率较低。DDLPS的中位总生存期为144个月，显著低于MyxLPS （HR: 0.26 [95% CI 0.21-0.33]）、PLPS （HR: 0.76 [95% CI 0.59-0.98]）、HGMLPS （HR: 0.30 [95% CI 0.18-0.50]）、WDLPS （HR: 0.30 [95% CI 0.24-0.37]）、未分类LPS （HR: 0.53 [95% CI 0.37-0.75]）。除了发病率较低外，50岁的女性比男性有更好的无复发和总生存率，而在50岁或以下的组中没有观察到这一点。在多变量分析中，体型和年龄是最常见亚组的独立预后因素，但在每个分子亚组中观察到特定的预后参数。女性50岁是最大群体DDLPS的独立有利预后因素。在这个全国范围内病理证实的LPS系列中，组织型的临床表现、处理和生存非常不同，年龄相关的性别差异有利于女性[gt；50]。DDLPS是预后较差的亚型。本研究由以下项目资助：NetSARC+ (INCA)、RREPS （INCA）、RESOS （INCA）、INTERSARC+ (INCA)、LabEx DEvweCAN （ANR-10-LABX-0061）、LYriCAN+ (INCA - dgos - inserm - itmo cancer_18003)、法国国家癌症防治联盟、法国癌症防治联盟（法国癌症防治委员会）、ARC基金会和EURACAN（欧盟项目739521）。

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Clinical presentation, management and outcome of 11,132 patients with liposarcoma patients: a population-based study from the NETSARC+ registry

Background

Liposarcomas (LPS) are among the most common sarcomas, but gather a diversity of rare to ultrarare molecular subtypes whose presentations and natural histories are partially characterized. The aim of the work was to describe the presentation and outcome of the different LPS histotypes from the NETSARC+ registry.

Methods

NETSARC+ (netsarc.org) is a network of 26 reference sarcoma centers with specialized multidisciplinary tumor boards (MDTB), funded by the French INCA since 2010 aiming to improve the quality of care of sarcoma patients. Patients’ characteristics, treatment and outcomes are collected in a nationwide database. This work describes the outcome of all LPS confirmed by central review pathology review and integrated between 2010 and 2023 in the NETSARC+ database.

Findings

11,132 liposarcomas are included in the database for an estimated incidence of 11.5/10⁶/y. Median age was 65 (Q1–Q3: 53–74, range 0–97 y), with 6529 males (58.7%), with 4220 (37.9%) dedifferentiated (DDLPS), 1838 (16.5%) well differentiated LPS (WDLPS) & 2424 (21.8%) atypical lipomatous tumours (ALT), 1371 (12.3%) myxoid LPS (MyxLPS), 450 (4.0%) pleomorphic LPS (PLPS), 177 (1.6%) high grade myxoid LPS (HGMLPS), 24 (0.2%) mixed type liposarcomas (MTLPS), 14 (0.1%) myxoid pleomorphic LPS (MPLPS) and 614 (5.5%) non classified LPS (NCLPS). Age, sex and sites differed across histotypes, but overall, all histotypes were represent in all age groups and sites. We report first on a difference in the sex ratio of liposarcoma in different age groups. Women were less frequently affected with liposarcomas after 50, in DDLPS, MyxLPS and HGMLPS. The median overall survival of DDLPS was 144 months, significantly worse than that of MyxLPS (HR: 0.26 [95% CI 0.21–0.33]), PLPS (HR: 0.76 [95% CI 0.59–0.98]), HGMLPS (HR: 0.30 [95% CI 0.18–0.50]), WDLPS (HR: 0.30 [95% CI 0.24–0.37]), unclassified LPS (HR: 0.53 [95% CI 0.37–0.75]). In addition to a lower incidence, women aged >50 had a better relapse free and overall survival than male, while this was not observed in the group aged 50 or under. In multivariate analyses, size and age were independent prognostic factors for the most common subgroups, but specific prognostic parameters were observed in each molecular subgroup. Female >50 was an independent favorable prognostic factor for the largest groups of DDLPS.

Interpretation

In this nationwide series of pathology-confirmed LPS, the clinical presentation, management and survival of histotypes are very different with age-related sex differences favoring women >50. DDLPS is the subtype with the worse prognosis.

Funding

This work was supported by the following grants: NetSARC+ (INCA), RREPS (INCA), RESOS (INCA), INTERSARC+ (INCA), LabEx DEvweCAN (ANR-10-LABX-0061), LYriCAN+ (INCa-DGOS-INSERM-ITMO cancer_18,003), Ligue Nationale contre le Cancer, Ligue Contre le Cancer (Comité de l’Ain), Fondation ARC, and EURACAN (EU project 739521).

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来源期刊

Lancet Regional Health-Europe Multiple-

CiteScore

19.90

自引率

1.40%

发文量

260

审稿时长

9 weeks

期刊介绍： The Lancet Regional Health – Europe, a gold open access journal, is part of The Lancet's global effort to promote healthcare quality and accessibility worldwide. It focuses on advancing clinical practice and health policy in the European region to enhance health outcomes. The journal publishes high-quality original research advocating changes in clinical practice and health policy. It also includes reviews, commentaries, and opinion pieces on regional health topics, such as infection and disease prevention, healthy aging, and reducing health disparities.