Jiwei Bai1, Shuheng Zhang2, Yixuan Zhai3, Shuai Wang4, Mingxuan Li4, Chuzhong Li4, Peng Zhao5, Sumin Geng5, Songbai Gui5, Yazhuo Zhang6. 1. Department of Neurosurgery, Beijing Tiantan Hospital; Department of Cell Biology, Beijing Neurosurgical Institute, Capital Medical University, Beijing, 100070, China. 2. Department of Cell Biology, Beijing Neurosurgical Institute, Capital Medical University, Beijing, 100070; Department of Neurosurgery, Anshan Central Hospital, Anshan, 114001, China. 3. Department of Cell Biology, Beijing Neurosurgical Institute, Capital Medical University, Beijing, 100070; Department of Neurosurgery, First Affiliated Hospital, Zhengzhou University, Zhengzhou, 450052, China. 4. Department of Cell Biology, Beijing Neurosurgical Institute, Capital Medical University, Beijing, 100070, China. 5. Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China. 6. Department of Neurosurgery, Beijing Tiantan Hospital; Department of Cell Biology, Beijing Neurosurgical Institute, Capital Medical University; Beijing Institute for Brain Disorders Brain Tumor Center; China National Clinical Research Center for Neurological Diseases, Beijing, 100070, China.
Abstract
BACKGROUND: Skull base chordomas in pediatric and adolescent patients are rare and challenging for surgeons. OBJECTIVE: Well-specified diagnosis and treatment are of great value for the long-term control of chordoma. This study summarizes well-followed pediatric and adolescent chordoma (PAC) patients treated in a single Asian center. METHODS: PAC patients were enrolled. Data collected included clinical presentation, tumor volume, texture, surgical approach, pathology, complications, adjuvant radiotherapy (RT), and long-term outcomes. RESULTS: Sixty-two patients were identified from a total of 516 skull base chordoma patients (12%). Diplopia was the most prominent complaint (30%). The craniocervical junction area was the most common location (41.8%) and had the highest proportion of large tumors. The gross total resection (GTR) rate was 20.3%. The GTR rate was lowest for tumors located in the craniocervical junction area. Thirty-eight cases experienced surgical complications. Of note, there was a significant difference in the complication rate between endoscopic approaches (22.7%) and open approaches (57.9%) (P = 0.005). The mean follow-up was 66.5 months. The GTR group showed better survival compared with the non-GTR group (P = 0.043). Metastases were found in two cases. No significant difference in the overall survival (OS) time was found between the group with RT and the group without RT (P = 0.559). CONCLUSIONS: A higher proportion of PAC patients than previously reported exist in the population in Asia, and the metastatic rate is lower. GTR predicts excellent long-term control of the disease. RT should be considered on an individual basis.
BACKGROUND: Skull base chordomas in pediatric and adolescent patients are rare and challenging for surgeons. OBJECTIVE: Well-specified diagnosis and treatment are of great value for the long-term control of chordoma. This study summarizes well-followed pediatric and adolescent chordoma (PAC) patients treated in a single Asian center. METHODS: PAC patients were enrolled. Data collected included clinical presentation, tumor volume, texture, surgical approach, pathology, complications, adjuvant radiotherapy (RT), and long-term outcomes. RESULTS: Sixty-two patients were identified from a total of 516 skull base chordoma patients (12%). Diplopia was the most prominent complaint (30%). The craniocervical junction area was the most common location (41.8%) and had the highest proportion of large tumors. The gross total resection (GTR) rate was 20.3%. The GTR rate was lowest for tumors located in the craniocervical junction area. Thirty-eight cases experienced surgical complications. Of note, there was a significant difference in the complication rate between endoscopic approaches (22.7%) and open approaches (57.9%) (P = 0.005). The mean follow-up was 66.5 months. The GTR group showed better survival compared with the non-GTR group (P = 0.043). Metastases were found in two cases. No significant difference in the overall survival (OS) time was found between the group with RT and the group without RT (P = 0.559). CONCLUSIONS: A higher proportion of PAC patients than previously reported exist in the population in Asia, and the metastatic rate is lower. GTR predicts excellent long-term control of the disease. RT should be considered on an individual basis.
Entities:
Keywords:
Adolescence; chordoma; outcome; pediatric; skull base