Department of Orthopedic Oncology, Changzheng Hospital, Second Military Medical University, Shanghai, China.
Department of Orthopedic Oncology, Changzheng Hospital, Second Military Medical University, Shanghai, China; Department of Orthopedics, Peking University Third Hospital, Beijing, China.
Spine J. 2019 Jul;19(7):1232-1241. doi: 10.1016/j.spinee.2019.02.011. Epub 2019 Feb 15.
Giant cell tumors (GCTs) of the bone are benign but locally aggressive. Pediatric spine giant-cell tumors (PSGCTs) have been infrequently reported in the literature because of the rarity of the disease.
The purpose of this study was to define the overall occurrence rate of PSGCTs among all spinal GCTs in our center and investigate the clinical features and prognostic factors of this rare disease.
A retrospective review.
Thirty-one PSGCT patients, screened from 226 patients with spine GCTs who received treatment in our center between 1998 to 2017.
The clinical symptoms, neurologic status, radiologic manifestations, treatment, outcome, and complications were recorded and analyzed.
The postoperative recurrence-free survival (RFS) rate was estimated by the Kaplan-Meier method. Factors with p values ≤.1 were subjected to multivariate analysis for RFS by proportional hazard analysis, among which p values ≤.05 were considered statistically significant.
A total of 31 (31 of 226, 13.7%) PSGCTs patients (9 male and 22 female) were included in the study, with a mean age of 15.9 years and a mean follow-up period of 85.1 (median 84.0; range 12-221) months. The majority of patients (80.6 %) were 14-18 years of age. Recurrence was detected in 12 (38.7%) of the 31 patients. Univariate and multivariate analyses suggested that Jaffe grade II-III was an adverse prognostic factor for RFS, while total spondylectomy and bisphosphonate treatment were positive prognostic factors.
Total en bloc spondylectomy (TES) is associated with excellent prognosis for PSGCTs, and total piecemeal spondylectomy is a viable alternative if total en bloc spondylectomy is unfeasible. Long-term bisphosphonate administration could significantly reduce the recurrence risk of PSGCTs. Denosumab treatment is recommended, especially for advanced PSGCTs. Jaffe grade II-III is an adverse prognostic factor for recurrence.
骨巨细胞瘤（GCT）是良性的，但具有局部侵袭性。儿科脊柱巨细胞瘤（PSGCT）在文献中报道较少，因为这种疾病很少见。
本研究的目的是确定我们中心所有脊柱 GCT 中 PSGCT 的总体发生率，并研究这种罕见疾病的临床特征和预后因素。
回顾性研究。
从 1998 年至 2017 年在我们中心接受治疗的 226 例脊柱 GCT 患者中筛选出 31 例 PSGCT 患者。
记录并分析临床症状、神经状态、影像学表现、治疗、预后和并发症。
采用 Kaplan-Meier 法估计术后无复发生存率（RFS）。对 p 值≤.1 的因素进行多因素分析，通过比例风险分析对 RFS 进行分析，其中 p 值≤.05 认为具有统计学意义。
共纳入 31 例（226 例中的 31 例，占 13.7%）PSGCT 患者（男 9 例，女 22 例），平均年龄 15.9 岁，平均随访 85.1 个月（中位数 84.0；范围 12-221）个月。大多数患者（80.6%）为 14-18 岁。31 例患者中有 12 例（38.7%）出现复发。单因素和多因素分析表明，Jaffe 分级 II-III 是 RFS 的不良预后因素，而全脊椎切除术和双膦酸盐治疗是阳性预后因素。
全脊椎整块切除术（TES）与 PSGCT 预后良好相关，如果无法进行全脊椎整块切除术，全节段切除术是一种可行的替代方法。长期使用双膦酸盐可显著降低 PSGCT 复发风险。建议使用地舒单抗治疗，尤其是晚期 PSGCT。Jaffe 分级 II-III 是复发的不良预后因素。
