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 ORIGINAL ARTICLE
Year : 2020  |  Volume : 68  |  Issue : 5  |  Page : 1030--1036

A Series of 62 Skull Base Chordomas in Pediatric and Adolescent Patients: Clinical Characteristics, Treatments, and Outcomes


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

Correspondence Address:
Dr. Yazhuo Zhang
Beijing Neurosurgical Institute, Capital Medical University, Beijing, 100070
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.294548

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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.






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