A comprehensive clinico-pathological review of a series of pediatric, adolescents and young adults with high-grade osteosarcoma: from clinics to biomarker discovery.
Susana Buendía-López, Alba Rubio-San-Simón, Jen-Hao Wu, Daniel Azorín-Cuadrillero, Ana Sanz-Miguel, Álvaro Lassaletta, Sara Inmaculada Sirvent-Cerdá, Laura Rey-Portela, Juan Carlos Abril, Lucas Moreno, Luis Madero-López, Javier García-Castro, Francisco Bautista
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引用次数: 0
Abstract
Background: We analyzed clinical and immunohistochemical characteristics of pediatric, adolescents and young adults with high-grade osteosarcoma (HGOS) to validate prognostic factors, identify targetable and prognostic biomarkers and define management of multiple relapses.
Methods: Retrospective analysis of 67 patients with HGOS between 2001 and 2020 was studied. BTN3A2, HSP90 and GLYPICAN1 were further analyzed based on their high expression on in silico model.
Results: Conventional osteosarcoma was the most frequent histology subtype (89.5%); 26.9% of patients had metastases at diagnosis. Proportion of limb-sparing surgery and R0 resection increased before and after 2011 (66.6% vs. 96.2%; 78.5% vs 87.5% respectively), while no treatment-related deaths occurred after 2011. 5-year OS and EFS were 61% and 56.6%, (5.4-year median follow-up (0.20-17.40). In multivariate analysis, metastatic disease was the sole independent prognostic factor. 5-year EFS and OS for patients with 1st, 2nd, and 3rd relapse were 8-12%, 0-5%, and 11.1-11.1% respectively. BTN3A2 was highly expressed at diagnosis, surgery, and relapse.
Conclusion: Metastatic disease remains the most important prognosis factor in HGOS. Improvements in surgical procedures and reduction in treatment-related mortality were observed. Survival after multiple relapses remains poor; we define figures to be used for benchmarking in clinical trials. BTN3A2 is a potential therapeutic target.
研究背景我们分析了患有高级别骨肉瘤（HGOS）的儿童、青少年和年轻成人的临床和免疫组化特征，以验证预后因素、确定可靶向的预后生物标志物并确定多次复发的处理方法：研究对2001年至2020年间的67例HGOS患者进行了回顾性分析。方法：对2001年至2020年间的67例HGOS患者进行了回顾性分析，并根据BTN3A2、HSP90和GLYPICAN1在硅模型中的高表达情况对其进行了进一步分析：传统骨肉瘤是最常见的组织学亚型（89.5%）；26.9%的患者在确诊时有转移。保肢手术和R0切除的比例在2011年前后有所上升（分别为66.6% vs. 96.2%；78.5% vs. 87.5%），而2011年后没有发生与治疗相关的死亡。5年OS和EFS分别为61%和56.6%（中位随访5.4年（0.20-17.40））。在多变量分析中，转移性疾病是唯一独立的预后因素。第一次、第二次和第三次复发患者的5年生存率和OS分别为8-12%、0-5%和11.1-11.1%。BTN3A2在诊断、手术和复发时均高表达：结论：转移性疾病仍然是HGOS最重要的预后因素。据观察，手术方法有所改进，治疗相关死亡率有所降低。多次复发后的生存率仍然很低；我们确定了用于临床试验基准的数据。BTN3A2 是一个潜在的治疗靶点。
期刊介绍：
Clinical and Translational Oncology is an international journal devoted to fostering interaction between experimental and clinical oncology. It covers all aspects of research on cancer, from the more basic discoveries dealing with both cell and molecular biology of tumour cells, to the most advanced clinical assays of conventional and new drugs. In addition, the journal has a strong commitment to facilitating the transfer of knowledge from the basic laboratory to the clinical practice, with the publication of educational series devoted to closing the gap between molecular and clinical oncologists. Molecular biology of tumours, identification of new targets for cancer therapy, and new technologies for research and treatment of cancer are the major themes covered by the educational series. Full research articles on a broad spectrum of subjects, including the molecular and cellular bases of disease, aetiology, pathophysiology, pathology, epidemiology, clinical features, and the diagnosis, prognosis and treatment of cancer, will be considered for publication.
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