Bone sarcomas: from biology to targeted therapies.
Bone sarcomas: from biology to targeted therapies.
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作者：
Gaspar N;Di Giannatale A;Geoerger B;Redini F;Corradini N;Enz-Werle N;Tirode F;Marec-Berard P;Gentet JC;Laurence V;Piperno-Neumann S;Oberlin O;Brugieres L
原发性恶性骨肿瘤、骨肉瘤和尤文肉瘤是罕见疾病，主要发生于青少年和年轻人。在目前的治疗方法下，有些患者仍然很难治疗，例如术前CT组织学反应较差的肿瘤（或未手术的尤文肉瘤初始肿瘤体积较大）、多发转移或复发的患者。为了开发针对这些罕见肿瘤的新疗法，我们需要揭示恶性特征背后的关键驱动因素和分子异常，并扩大我们对维持这些肿瘤转移表型和治疗耐药性的现象的理解。在本文中，我们将从这些肿瘤的生物学入手，讨论旨在增强局部肿瘤控制、限制转移扩散并最终提高患者生存率的潜在治疗靶点。
Primary malignant bone tumours, osteosarcomas, and Ewing sarcomas are rare diseases which occur mainly in adolescents and young adults. With the current therapies, some patients remain very difficult to treat, such as tumour with poor histological response to preoperative CT (or large initial tumour volume for Ewing sarcomas not operated), patients with multiple metastases at or those who relapsed. In order to develop new therapies against these rare tumours, we need to unveil the key driving factors and molecular abnormalities behind the malignant characteristics and to broaden our understanding of the phenomena sustaining the metastatic phenotype and treatment resistance in these tumours. In this paper, starting with the biology of these tumours, we will discuss potential therapeutic targets aimed at increasing local tumour control, limiting metastatic spread, and finally improving patient survival.