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黏液样脂肪肉瘤的诊断与治疗。

Diagnosis and Treatment of Myxoid Liposarcoma.

机构信息

Department of Bone and Soft Tissue Cancer, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Dongming Road 127, Zhengzhou, China.

Department of Hematology, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Dongming Road 127, Zhengzhou, China.

出版信息

Curr Treat Options Oncol. 2024 Oct;25(10):1289-1296. doi: 10.1007/s11864-024-01262-9. Epub 2024 Sep 20.

Abstract

Myxoid liposarcoma (MLS) is a rare subtype of soft tissue sarcoma that distinguishes itself from conventional subtypes through its propensity for extrapulmonary metastasis. The distinctive magnetic resonance imaging (MRI) characteristics of MLS render it an invaluable tool for identifying primary and secondary lesions. Pathologically, MLS is characterized by the FUS-DDIT3 gene fusion. Accurate diagnosis, facilitated by MRI and pathological assessment, is critical for prognostication and the formulation of appropriate treatment strategies. Surgery remains the cornerstone of local management for MLS. The combination of surgery and radiotherapy can significantly reduce the local recurrence rate in MLS, as it is highly sensitive to both radiotherapy and chemotherapy. Additionally, for high-risk MLS cases with a large tumor diameter, chemotherapy has been shown to improve survival. The comprehensive treatment approach for MLS demonstrates superior local recurrence rates and survival rates compared to most soft tissue sarcomas. Current research focuses on developing effective therapies for unresectable or advanced disease based on genomic and phenotypic characteristics as well as the immune-tumor microenvironment.

摘要

黏液样脂肪肉瘤(MLS)是一种罕见的软组织肉瘤亚型,其通过肺外转移倾向与常规亚型区分开来。MLS 的独特磁共振成像(MRI)特征使其成为识别原发性和继发性病变的宝贵工具。从病理学角度来看,MLS 的特征在于 FUS-DDIT3 基因融合。MRI 和病理学评估有助于准确诊断,这对于预测预后和制定适当的治疗策略至关重要。手术仍然是 MLS 局部管理的基石。手术和放疗的结合可以显著降低 MLS 的局部复发率,因为它对放疗和化疗均高度敏感。此外,对于肿瘤直径较大的高危 MLS 病例,化疗已被证明可以提高生存率。与大多数软组织肉瘤相比,MLS 的综合治疗方法显示出更好的局部复发率和生存率。目前的研究重点是根据基因组和表型特征以及免疫-肿瘤微环境,为不可切除或晚期疾病开发有效的治疗方法。

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