Liposarcomas are rare malignant tumors of adipocytic differentiation. The classification of liposarcomas into four principal subtypes reflects the distinct clinical behavior, treatment sensitivity, and underlying biology encompassed by these diseases. Increasingly, clinical management decisions and the development of investigational therapeutics are informed by an improved understanding of subtype-specific molecular pathology. Well-differentiated liposarcoma is the most common subtype and is associated with indolent behavior, local recurrence, and insensitivity to radiotherapy and chemotherapy. Dedifferentiated liposarcoma represents focal progression of well-differentiated disease into a more aggressive, metastasizing, and fatal malignancy. Both of these subtypes are characterized by recurrent amplifications within chromosome 12, resulting in the overexpression of disease-driving genes that have been the focus of therapeutic targeting. Myxoid liposarcoma is characterized by a pathognomonic chromosomal translocation that results in an oncogenic fusion protein, whereas pleomorphic liposarcoma is a karyotypically complex and especially poor-prognosis subtype that accounts for less than 10% of liposarcoma diagnoses. A range of novel pharmaceutical agents that aim to target liposarcoma-specific biology are under active investigation and offer hope of adding to the limited available treatment options for recurrent or inoperable disease.

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脂肪肉瘤的临床和分子谱

脂肪肉瘤是一种罕见的脂肪细胞分化恶性肿瘤。将脂肪肉瘤分为四个主要亚型反映了这些疾病所包含的不同临床行为、治疗敏感性和潜在生物学。对亚型特异性分子病理学的深入了解，越来越多地为临床管理决策和研究性疗法的开发提供信息。分化良好的脂肪肉瘤是最常见的亚型，与惰性行为、局部复发以及对放疗和化疗不敏感有关。去分化脂肪肉瘤代表分化良好的疾病局部进展为更具侵袭性、转移性和致命性的恶性肿瘤。这两种亚型的特点是在 12 号染色体内反复扩增，导致疾病驱动基因的过度表达，这些基因一直是治疗靶向的焦点。粘液样脂肪肉瘤的特征在于导致致癌融合蛋白的致病性染色体易位，而多形性脂肪肉瘤是一种核型复杂且预后特别差的亚型，占脂肪肉瘤诊断的不到 10%。一系列旨在针对脂肪肉瘤特异性生物学的新型药剂正在积极研究中，并为治疗复发或无法手术的疾病提供了增加有限可用治疗选择的希望。而多形性脂肪肉瘤是一种核型复杂且预后特别差的亚型，占脂肪肉瘤诊断的不到 10%。一系列旨在针对脂肪肉瘤特异性生物学的新型药剂正在积极研究中，并为治疗复发或无法手术的疾病提供了增加有限可用治疗选择的希望。而多形性脂肪肉瘤是一种核型复杂且预后特别差的亚型，占脂肪肉瘤诊断的不到 10%。一系列旨在针对脂肪肉瘤特异性生物学的新型药剂正在积极研究中，并为治疗复发或无法手术的疾病提供了增加有限可用治疗选择的希望。