Clinical Characteristics, Management, and Outcomes of Intramedullary Spinal Cord Ependymomas in Adults: A Systematic Review

Background: Intramedullary spinal cord ependymomas (IMSCEs) are rare tumors that mostly occur in adults. Management strategies and related outcomes are heterogeneously reported across the literature, demanding a comprehensive analysis to standardize guidelines. We performed a systematic review of the literature on IMSCEs. Methods: A literature search was conducted using 6 databases from inception up to July 28, 2022. Studies with data on clinical characteristics, management strategies, and related outcomes in adult patients with histopathologically confirmed IMSCEs were pooled and analyzed. Results: The analysis included 69 studies comprising 457 patients (52.7% males). Mean age was 42.4 ± 7.4 years. 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 mostly World Health Organization 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 reported in only 7.0% of the patients. At last follow-up, 97.4% of patients were alive. Conclusions: IMSCEs are uncommon tumors that frequently manifest with debilitating symptoms that require surgical treatment. When feasible, gross total resection may be pursued to improve the patient's functional status and prevent tumor progression, with adjuvant radiotherapy required only in some more aggressive grade III lesions. Future studies should investigate different growth patterns and prognoses based on different IMSCE subtypes.