Title : Clinical characteristics, management, and outcomes of intramedullary spinal cord ependymomas in adults: A systematic review
Abstract:
Background:
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 28th, 2022. Studies with data on clinical characteristics, management strategies, and related outcomes in adult patients with histopathologically confirmed IMSCE were pooled and analyzed.
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 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.
Conclusions:
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
