Sun Jian, Wu Zhipeng, Jiao Jian, Wei Haifeng, Yang Xinghai, Liu Tielong, Zhao Jian, Yang Cheng, Xu Wei, Zhou Zhenhua, Wang Ting, Xiao Jianru
Department of Orthopedic Oncology, Shanghai Changzheng Hospital, Naval Medical University, #415 Fengyang Road, Shanghai, 200003, China.
Wuxi School of Medicine, Jiangnan University, 1800 Lihu Avenue, Wuxi, 214122, Jiangsu, China.
J Neurooncol. 2024 Dec;170(3):641-653. doi: 10.1007/s11060-024-04823-y. Epub 2024 Sep 14.
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.
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.
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.
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.
与肢体软骨肉瘤相比，脊柱软骨肉瘤具有更高的侵袭性和更差的预后。软骨肉瘤不同病理亚型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各亚型在临床特征、分子特征、治疗效果和预后因素方面的差异。
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、间叶性软骨肉瘤（MCS）、去分化软骨肉瘤（DCS）和透明细胞软骨肉瘤（CCCS）病理亚型的临床特征和免疫组化（IHC）标志物。1/2/3级软骨肉瘤统称为传统软骨肉瘤（CCS）进行多因素生存分析。进行单因素和多因素分析以研究脊柱软骨肉瘤患者总生存（OS）和无复发生存（RFS）的独立预后因素。此外，还确定了CCS和MCS中OS和RFS的独立预后因素。
MCS患者比其他亚型患者年轻。1/2级软骨肉瘤患者的OS优于3级软骨肉瘤、MCS和DCS患者，而只有1级软骨肉瘤患者的RFS优于2/3级软骨肉瘤、MCS和DCS患者。3级软骨肉瘤、MCS和DCS的Ki-67指数高于1/2级软骨肉瘤。IHC标志物的比较进一步突出了MCS和DCS中P53/MDM2的过表达。包括整块切除和分块切除在内的根治性全切除显著改善了CCS患者的OS和RFS，而只有整块切除显著改善了MCS患者的预后。化疗似乎对MCS患者的OS很重要。
与1/2级软骨肉瘤相比，MCS和DCS中P53/MDM2通路上调。根治性肿瘤切除对脊柱软骨肉瘤的治疗至关重要，而MCS患者围手术期需要进一步的综合治疗。
