Visiting Scholar Department of Neurosurgery, University of Cincinnati College of Medicine Cincinnati, Ohio, United States
Introduction: Intramedullary spinal cord ependymomas (IMSCE) are rare tumors that mostly occur in adult patients. Management strategies and related outcomes are heterogeneously reported across the literature, demanding a comprehensive analysis to standardize guidelines. We systematically reviewed the literature on IMSCEs.
Methods: A literature search was conducted on 6 databases from inception up to July 28 the, 2022 without any restrictions. Studies with data on clinical characteristics, management strategies, and related outcomes in adult patients with histopathologically confirmed IMSCE were pooled and analyzed. Analysis was conducted using SPSS Version 25 (Chicago, IL: IBM® SPSS® Statistics).
Results: A total of 69 studies were included, comprising 457 patients (52.7% males). The mean age was 42.4 years old (SD; ±7.4). Sensory deficit (58.0%) was the most prevalent symptom, followed by radicular pain (50.5%). Tumors mostly involved the cervical (64.4%) or thoracic (18.8%) spinal cord and were mainly of WHO grade-II (80.5%) and classic subtype (72.4%). Gross-total resection was performed in most cases (83.4%), with adjuvant radiotherapy delivered in 10.5% of cases. Progression-free survival ≥ 2 years was reported in 61.1% of cases, and tumor recurrence or progression was only reported in 7.0% of the patients. 97.4% of patients were alive at the last follow-up.
Conclusion : IMSCEs are uncommon tumors that frequently present debilitating symptoms requiring surgical treatment. When feasible, gross-total tumor resection may be pursued to improve the patient’s functional status and prevent tumor progression, with adjuvant radiotherapy only needed in some more aggressive grade-III lesions. Future studies should investigate growth patterns and prognoses based on different IMSCEs’ subtypes.
