Chow Warren A
City of Hope Medical Center, Division of Medical Oncology & Therapeutics Research, Duarte, CA 91010, USA.
Curr Opin Oncol. 2007 Jul;19(4):371-6. doi: 10.1097/CCO.0b013e32812143d9.
This paper reviews recent molecular, biologic, developmental therapeutic, and clinical findings in conventional and variant chondrosarcomas.
The prognosis of chondrosarcomas traditionally correlates with histologic grade and adequacy of surgery. Newer markers of cell differentiation, activation, genetics, and cell signaling may offer important prognostic information. Translational research has validated platelet-derived growth factor receptor, estrogen signaling, matrix metalloproteinase-1, histone deacetylase, methylthioadenosine phosphorylase, and vascular endothelial growth factor-A as potential therapeutic targets. Bisphosphonates may also possess important antitumoral effects. Molecular studies have established that extraskeletal myxoid chondrosarcoma is a unique entity defined by the presence of a fusion gene between the orphan nuclear receptor, CHN/NOR1, and a promiscuous partner, most commonly EWSR1. Clinical studies have shown that development of second malignancies is an uncommon but real risk for chondrosarcoma survivors; the benefit of chemotherapy for dedifferentiated chondrosarcomas remains questionable; and late recurrences of clear cell chondrosarcomas emphasize the need for long-term follow up.
Chondrosarcomas are a heterogeneous group of bone and soft tissue tumors. Recent advances in molecular diagnostics, pathobiology, and developmental therapeutics will aid both scientists and clinicians in improving the classification and therapy of this diverse family of cartilaginous tumors.
本文综述了传统型和变异型软骨肉瘤近期在分子、生物学、发育治疗学及临床方面的研究结果。
传统上，软骨肉瘤的预后与组织学分级及手术的充分性相关。细胞分化、激活、遗传学及细胞信号传导的新型标志物可能提供重要的预后信息。转化研究已证实血小板衍生生长因子受体、雌激素信号传导、基质金属蛋白酶-1、组蛋白脱乙酰基酶、甲硫腺苷磷酸化酶及血管内皮生长因子-A为潜在治疗靶点。双膦酸盐可能也具有重要的抗肿瘤作用。分子研究已证实骨外黏液样软骨肉瘤是一种独特的实体瘤，其特征为孤儿核受体CHN/NOR1与一个多配体伴侣（最常见的为EWSR1）之间存在融合基因。临床研究表明，发生第二原发性恶性肿瘤是软骨肉瘤幸存者中一种不常见但确实存在的风险；去分化软骨肉瘤化疗的益处仍存在疑问；透明细胞软骨肉瘤的晚期复发强调了长期随访的必要性。
软骨肉瘤是一组异质性的骨和软组织肿瘤。分子诊断、病理生物学及发育治疗学方面的近期进展将有助于科学家和临床医生改进这类多样的软骨肿瘤的分类和治疗。
