Clinical features and prognostic factors of pediatric spine giant cell tumors: report of 31 clinical cases in a single center.

BACKGROUND CONTEXT

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.

PURPOSE

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.

STUDY DESIGN

A retrospective review.

PATIENT SAMPLE

Thirty-one PSGCT patients, screened from 226 patients with spine GCTs who received treatment in our center between 1998 to 2017.

OUTCOME MEASURES

The clinical symptoms, neurologic status, radiologic manifestations, treatment, outcome, and complications were recorded and analyzed.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.