Adjuvant radiation therapy and chondroid chordoma subtype are associated with a lower tumor recurrence rate of cranial chordoma

Abstract

Cranial chordomas are rare tumors that have been difficult to study given their low prevalence. Individual case series with decades of data collection provide some insight into the pathobiology of this tumor and its responses to treatment. This meta-analysis is an attempt to aggregate the sum experiences and present a comprehensive review of their findings. We performed a comprehensive review of studies published in English language literature and found a total of over 2,000 patients treated for cranial chordoma. Patient information was then extracted from each paper and aggregated into a comprehensive database. The tumor recurrences in these patients were then stratified according to age (<21 vs. >21 years), histological findings (chondroid vs. typical) and treatment (surgery and radiation vs. surgery only). Data was analyzed via Pearson chi-square and t-test. A total of 464 non-duplicated patients from 121 articles treated for cranial chordoma met the inclusion criteria. The recurrence rate among all patients was 68% (314 patients) with an average disease-free interval of 45 months (median, 23 months). The mean follow-up time was 39 months (median, 27 months). The patients in younger group, patients with chordoma with chondroid histologic type, and patients who received surgery and adjuvant radiotherapy had significantly lower recurrence rate than their respective counterparts. The results of our systematic analysis provide useful data for practitioners in objectively summarizing the tumor recurrence in patients with cranial chordomas. Our data suggests that younger patients with chondroid type cranial chordoma treated with both surgery and radiation may have improved rates of tumor recurrence in the treatment of these tumors.