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去分化脂肪肉瘤的诊断和治疗:多学科立场声明。

Diagnosis and management of dedifferentiated liposarcoma: A multidisciplinary position statement.

机构信息

Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, United States.

Brigham & Women's Hospital, Harvard Medical School, Boston, MA, United States.

出版信息

Cancer Treat Rev. 2024 Dec;131:102846. doi: 10.1016/j.ctrv.2024.102846. Epub 2024 Oct 18.

Abstract

Dedifferentiated liposarcoma (DDLPS) is a malignant mesenchymal neoplasm in desperate need of novel therapeutic approaches. Often occurring in conjunction with well-differentiated liposarcoma (WDLPS), DDLPS can behave more aggressively and exhibits a significant risk for developing recurrence or metastatic disease when compared to its well-differentiated counterpart. A multidisciplinary approach is critically important, particularly for patients with localized disease, as disease presentations are often complex, and the management of patients has become increasingly nuanced as treatment approaches have become more refined. Expert pathology review and appropriate application of diagnostic molecular techniques are key components of DDLPS diagnosis and also reflect an improved understanding of the underlying pathogenesis of the disease. Systemic therapies remain limited for DDLPS, but novel therapies targeting important underlying molecular drivers have resulted in ongoing clinical trials aiming to improve outcomes for patients with advanced disease. In recognition of the increased activity and interest within the DDLPS field, a multidisciplinary group of nationally recognized experts in medical oncology, surgical oncology, radiation oncology, and pathology was convened to summarize key insights. This position paper highlights important points from the meeting and provides evidence-based recommendations for practicing clinicians.

摘要

去分化脂肪肉瘤(DDLPS)是一种恶性间叶性肿瘤,迫切需要新的治疗方法。DDLPS 常与高分化脂肪肉瘤(WDLPS)同时发生,与分化良好的对应物相比,DDLPS 的行为更为激进,并且复发或转移疾病的风险显著增加。多学科方法至关重要,特别是对于局部疾病患者,因为疾病表现通常很复杂,并且随着治疗方法的不断完善,患者的管理变得越来越复杂。专家病理审查和适当应用诊断分子技术是 DDLPS 诊断的关键组成部分,也反映了对疾病潜在发病机制的理解的提高。DDLPS 的系统治疗仍然有限,但针对重要潜在分子驱动因素的新型治疗方法已导致正在进行的临床试验旨在改善晚期疾病患者的预后。认识到 DDLPS 领域的活动和兴趣增加,召集了一组在肿瘤内科、外科肿瘤学、放射肿瘤学和病理学方面具有全国公认专业知识的多学科专家,以总结关键见解。本立场文件突出了会议的重要要点,并为临床医生提供了循证建议。

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