Division of Cancer Research, Regional Cancer Centre (Research Centre, University of Kerala), Thiruvananthapuram, Kerala, 695011, India.
Division of Surgical Oncology, Regional Cancer Centre (Research Centre, University of Kerala), Thiruvananthapuram, Kerala, 695011, India.
J Mol Histol. 2024 Jun;55(3):227-240. doi: 10.1007/s10735-024-10195-4. Epub 2024 May 2.
Liposarcoma (LPS) is a rare malignancy of adipocytic differentiation. According to World Health Organization classification, LPS comprises of four principle subtypes Atypical lipomatous tumor/Well-differentiated liposarcoma (ATL/WDLPS), Dedifferentiated liposarcoma (WDLPS), Myxoid liposarcoma (MLPS), and Pleomorphic liposarcoma (PLPS). Each subtype can develop at any location and shows distinct clinical behavior and treatment sensitivity. ATL/ WDLPS subtype has a higher incidence rate, low recurrence, and is insensitive to radiation and chemotherapy. DDLPS is the focal progression of WDLPS, which is aggressive and highly metastasizing. MLPS is sensitive to radiation and chemotherapy, with a higher recurrence rate and metastasis. PLPS subtype is highly metastasizing, has a poor prognosis, and exhibiting higher recurrence rate. Initial histological analysis provides information for the characterization of LPS subtypes', further molecular and genetic analysis provides certain subtype specifications, such as gene amplifications and gene fusions. Such molecular genetic alterations will be useful as therapeutic targets in various cancers, including the LPS subtypes. A wide range of novel therapeutic agents based on genetic alterations that aim to target LPS subtypes specifically are under investigation. This review summarizes the LPS subtype classification, their molecular genetic characteristics, and the implications of genetic alterations in therapeutics.
脂肪肉瘤(LPS)是一种罕见的脂肪细胞分化的恶性肿瘤。根据世界卫生组织的分类,LPS 包括四个主要亚型:非典型性脂肪肉瘤/高分化脂肪肉瘤(ATL/WDLPS)、去分化脂肪肉瘤(DDLPS)、黏液样脂肪肉瘤(MLPS)和多形性脂肪肉瘤(PLPS)。每种亚型都可以在任何部位发生,并表现出不同的临床行为和治疗敏感性。ATL/WDLPS 亚型发病率较高,复发率低,对放疗和化疗不敏感。DDLPS 是 WDLPS 的局部进展,具有侵袭性和高度转移性。MLPS 对放疗和化疗敏感,复发率和转移率较高。PLPS 亚型具有高度转移性,预后不良,复发率较高。初始组织学分析为 LPS 亚型的特征提供了信息,进一步的分子和遗传学分析提供了某些亚型的特征,如基因扩增和基因融合。这些分子遗传学改变将作为各种癌症(包括 LPS 亚型)的治疗靶点。正在研究广泛的基于遗传改变的新型治疗药物,旨在针对 LPS 亚型特异性。本综述总结了 LPS 亚型分类、分子遗传学特征以及遗传改变在治疗中的意义。