Title : Clinical characteristics, management, and outcomes of intramedullary spinal cord ependymomas in adults: A systematic review
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.
A literature search was conducted on 6 databases from inception up to July 28th, 2022. Studies with data on clinical characteristics, management strategies, and related outcomes in adult patients with histopathologically confirmed IMSCE were pooled and analyzed.
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 mostly 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% cases. Progression-free survival ≥2-years was reported in 61.1% cases, and tumor recurrence or progression was only reported in 7.0% of the patients. 97.4% of patients were alive at last follow-up.
IMSCEs are uncommon tumors frequently presenting with debilitating symptoms that require surgical treatment. When feasible, gross-total tumor resection may be pursued to improve 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 IMSCEs’ subtypes.
Audience Take Away
• Sensory deficit and/or radicular pain were commonly present that debilitate quality of life and require surgical intervention
• In selected patients, gross total resection is achievable and effective, showing prolonged PFS rates (≥2 years) with low rates of recurrence/progression
• Future research should focus more on the different characteristics, management strategies, and related outcomes based on IMSCEs’ grades and subtypes, also providing more data from low and middle-income countries