Localized and metastatic myxoid/round cell liposarcoma Clinical and Molecular Observations
Localized and metastatic myxoid/round cell liposarcoma Clinical and Molecular Observations
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DOI：
10.1002/cncr.27847
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发表时间：
2013-05-15
期刊：
影响因子：
6.2
通讯作者：
Pollock, Raphael E.
中科院分区：
文献类型：
--
作者：
Hoffman, Aviad;Ghadimi, Markus P. H.;Pollock, Raphael E.
背景。黏液样脂肪肉瘤（MLPS）是一种特别发生于年轻人的疾病，具有局部复发和转移的可能性，目前缺乏可靠的预后因素和治疗靶点。本报告的作者评估了MLPS患者的自然病史和结果，以及通常不受调节的蛋白质生物标志物。方法。回顾性回顾了1990年至2010年期间在笔者所在机构就诊的局限性（n = 207）或转移性（n = 61） MLPS患者的医疗记录。构建MLPS患者标本（n = 169）的组织芯片，进行分子标记的免疫组织化学分析。结果。局限性疾病患者的5年和10年疾病特异性生存率分别为93%和87%；男性、年龄≥45岁、肿瘤复发预测预后较差。局部复发率为7.4%，局部复发风险与肿瘤复发及非肢体部位有关。男性是转移性疾病的主要危险因素，发生在13%的患者中。40%的局部疾病患者接受了化疗，主要是新辅助治疗。免疫组织化学分析显示，C-X-C趋化因子受体4型（CXCR4）和血小板衍生生长因子β (PDGFR-)在转移性病变中的表达明显高于局限性病变。圆形细胞表型的肿瘤表达的CXCR4、p53、亲脂素、PDGFR-、PDGFR-和血管内皮生长因子的水平相对于粘液样表型升高。在单变量分析中，只有AXL基因编码的受体酪氨酸激酶（AXL）被确定为疾病特异性生存的预后因子。结论。在这项研究中，作者确定了MLPS患者的临床和分子预后指标以及几个潜在的治疗靶点。2013年癌症。(c) 2013年美国癌症协会。
BACKGROUND. Myxoid liposarcoma (MLPS), a disease especially of young adults with potential for local recurrence and metastasis, currently lacks solid prognostic factors and therapeutic targets. The authors of this report evaluated the natural history and outcome of patients with MLPS and commonly deregulated protein biomarkers. METHODS. Medical records were retrospectively reviewed for patients who presented to the authors' institution with localized (n = 207) or metastatic (n = 61) MLPS (1990 to 2010). A tissue microarray of MLPS patient specimens (n = 169) was constructed for immunohistochemical analysis of molecular markers. RESULTS. The 5-year and 10-year disease-specific survival rates among patients with localized disease were 93% and 87%, respectively; male gender, age >45 years, and recurrent tumor predicted poor outcome. The local recurrence rate was 7.4%, and the risk of local recurrence was associated with recurrent tumors and nonextremity disease location. Male gender was the main risk factor for metastatic disease, which occurred in 13% of patients. Forty percent of patients who had localized disease received chemotherapy, mostly in the neoadjuvant setting. Immunohistochemical analysis revealed significantly higher expression of C-X-C chemokine receptor type 4 (CXCR4) and platelet-derived growth factor beta (PDGFR-) in metastatic lesions versus localized lesions. Tumors with a round cell phenotype expressed increased levels of CXCR4, p53, adipophilin, PDGFR-, PDGFR-, and vascular endothelial growth factor relative to myxoid phenotype. Only the receptor tyrosine kinase encoded by the AXL gene (AXL) was identified as a prognosticator of disease-specific survival in univariate analysis. CONCLUSIONS. In this study, the authors identified clinical and molecular outcome prognosticators for patients with MLPS as well as several potential therapeutic targets. Cancer 2013. (c) 2013 American Cancer Society.