脊柱软骨肉瘤不同病理亚型的临床特征、治疗方法和结果比较

IF 4.3 3区 材料科学 Q1 ENGINEERING, ELECTRICAL & ELECTRONIC ACS Applied Electronic Materials Pub Date : 2024-09-14 DOI:10.1007/s11060-024-04823-y
Jian Sun, Zhipeng Wu, Jian Jiao, Haifeng Wei, Xinghai Yang, Tielong Liu, Jian Zhao, Cheng Yang, Wei Xu, Zhenhua Zhou, Ting Wang, Jianru Xiao
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摘要

导言脊柱软骨肉瘤与四肢软骨肉瘤相比,侵袭性更强,预后更差。不同病理亚型软骨肉瘤的预后和治疗方案差异很大。本研究旨在分析脊柱软骨肉瘤不同亚型的临床特征、分子特征、治疗效果和预后因素的差异。方法对205例脊柱软骨肉瘤患者进行回顾性研究,比较了1级、2级、3级软骨肉瘤、间充质软骨肉瘤(MCS)、去分化软骨肉瘤(DCS)和透明细胞软骨肉瘤(CCCS)等病理亚型的临床特征和免疫组化(IHC)标记物。在多变量生存分析中,1/2/3级软骨肉瘤统称为常规软骨肉瘤(CCS)。通过单变量和多变量分析,研究脊柱软骨肉瘤患者总生存期(OS)和无复发生存期(RFS)的独立预后因素。结果脊柱软骨肉瘤患者比其他亚型更年轻。1/2级软骨肉瘤患者的OS优于3级、MCS和DCS患者,而只有1级软骨肉瘤患者的RFS优于2/3级、MCS和DCS患者。3级、MCS和DCS软骨肉瘤患者的Ki-67指数高于1/2级软骨肉瘤患者。IHC标记物的比较进一步突出了P53/MDM2在MCS和DCS中的过度表达。全切(包括全切和分块切除)能显著改善CCS患者的OS和RFS,而只有全切能显著改善MCS患者的预后。结论与1/2级软骨肉瘤相比,P53/MDM2通路在MCS和DCS中上调。肿瘤根治性切除是治疗脊柱软骨肉瘤的关键,而MCS患者需要在围手术期进一步接受综合治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Comparisons of clinical characteristics, treatments, and outcomes among different pathological subtypes of chondrosarcoma in the spine

Introduction

Spinal chondrosarcoma exhibits higher invasiveness and a worse prognosis compared to chondrosarcoma in the extremities. The prognosis and therapeutic plan vary greatly among different pathological subtypes of chondrosarcoma. This study aimed to analyze the differences in clinical characteristics, molecular features, therapeutic effects, and prognostic factors among the subtypes of chondrosarcoma in the spine.

Methods

A retrospective review was conducted on 205 patients with spinal chondrosarcoma. The clinical features and immunohistochemical (IHC) markers were compared among the pathological subtypes of chondrosarcoma grade 1, grade 2, grade 3, mesenchymal chondrosarcoma (MCS), dedifferentiated chondrosarcoma (DCS), and clear cell chondrosarcoma (CCCS). Chondrosarcoma grade 1/2/3 are collectively referred to as conventional chondrosarcoma (CCS) for multivariate survival analysis. Univariate and multivariate analyses were performed to investigate independent prognostic factors for overall survival (OS) and recurrence-free survival (RFS) in patients with spinal chondrosarcoma. Furthermore, independent prognostic factors for OS and RFS were identified in CCS and MCS.

Results

MCS patients were younger than the other subtypes. Patients with chondrosarcoma grade 1/2 had better OS than those with chondrosarcoma grade 3, MCS and DCS, while only chondrosarcoma grade 1 patients showed better RFS than chondrosarcoma grade 2/3, MCS and DCS patients. Ki-67 index was higher in chondrosarcoma grade 3, MCS and DCS than chondrosarcoma grade 1/2. The comparison of IHC markers further highlighted the overexpression of P53/MDM2 in MCS and DCS. Gross total resection, including en-bloc and piecemeal resection, significantly improved OS and RFS for CCS patients, while only en-bloc resection significantly improved the prognosis of MCS patients. Chemotherapy appeared to be important for the OS of MCS patients.

Conclusion

P53/MDM2 pathway was upregulated in MCS and DCS compared to chondrosarcoma grade 1/2. Radical tumor resection is crucial for the treatment of spinal chondrosarcoma, while MCS patients require further comprehensive treatments perioperatively.

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