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骨恶性巨细胞瘤:来自单一中心的临床、病理及预后特征研究

Malignant Giant Cell Tumor of Bone: A Study of Clinical, Pathological, and Prognostic Profile from One Single Center.

作者信息

Shi Jingtian, Sun Xin, Wang Jichuan, Liang Haijie, Liu Xingyu, Yang Yi, Tang Xiaodong, Guo Wei

机构信息

Musculoskeletal Tumor Center, Peking University People's Hospital, Beijing 100044, China.

出版信息

Bioengineering (Basel). 2025 Aug 25;12(9):911. doi: 10.3390/bioengineering12090911.

Abstract

Malignant giant cell tumor of bone (GCTB) is a rare malignant bone tumor. This analysis was conducted on patients with malignant GCTB at our center. The clinical, demographic, and prognostic characteristics were evaluated and compared. During 1 January 2015 to 31 December 2022, fifty patients were included in the study, which made up 3.3% of the contemporary GCTB patients. The clinical characteristics were comparable between the 24 patients with primary malignant GCTB (PMGCTB) and 26 patients with secondary malignant GCTB (SMGTCB). The tumor location was mainly at the axial and pelvic region (70%) and differed between the two types ( = 0.040). pathogenic variant presented frequently in SMGCTB ( = 0.020). Cox regression analysis showed the prognostic outcomes were poor in those with a tumor located in the axial bone and sacrum with invasion of other places. mutation status is also a risk factor, while chemotherapy and denosumab failed to demonstrate prognostic benefits. Malignant GCTB is a rare condition with a poor prognosis, especially in SMGTCB. The location and mutation status had an influence on prognosis, and systemic therapy should be taken into consideration for patients with unfavorable prognostic features.

摘要

骨恶性巨细胞瘤(GCTB)是一种罕见的恶性骨肿瘤。本分析针对我们中心的骨恶性巨细胞瘤患者进行。对其临床、人口统计学和预后特征进行了评估和比较。在2015年1月1日至2022年12月31日期间,50名患者被纳入研究,占同期GCTB患者的3.3%。24例原发性骨恶性巨细胞瘤(PMGCTB)患者和26例继发性骨恶性巨细胞瘤(SMGTCB)患者的临床特征具有可比性。肿瘤部位主要位于轴向和盆腔区域(70%),且两种类型之间存在差异(P = 0.040)。致病变体在SMGCTB中频繁出现(P = 0.020)。Cox回归分析显示,肿瘤位于轴向骨和骶骨且侵犯其他部位的患者预后较差。X突变状态也是一个危险因素,而化疗和地诺单抗未能显示出预后益处。骨恶性巨细胞瘤是一种罕见疾病,预后较差,尤其是在SMGTCB中。肿瘤位置和X突变状态对预后有影响,对于具有不良预后特征的患者应考虑进行全身治疗。

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