Clinical experience and management outcome of WHO grade II pediatric intramedullary ependymomas: A descriptive study

Abstract

Abstract Background: Intramedullary ependymom‑a is a rare primary tumor in the pediatric population. The World Health Organization (WHO) grade II pediatric intramedullary ependymoma (PIME) is the most common in frequency, though it is a less discussed pathological grade. This study aims at sharing the clinical experience and management outcome of WHO grade II PIME from a tertiary care center. Materials and Methods: The clinico-radiological details of all histologically proven, newly diagnosed primary PIME that underwent management were collected. Recurrent lesions and other spinal cord tumors were excluded. Details of surgical intervention and follow-up visits were reviewed from the record-keeping system. The surgical nuances and outcome predictors in the form of functional grade were analyzed. The statistical data were calculated by using the appropriate statistical tests. Results: The mean age of the study population was 16.3 ± 4.0 years. The male-to-female ratio was 2:1. The pain was noticed in 14 patients (14/18 [77.8%]), and four (22.2%) patients were ambulant at the time of admission. Overall, 15 (83.3%) patients underwent total excision, and subtotal excision was achieved in three patients. The median follow-up period was 31.0 months, and the range was 2–126 months. After surgical decompression of tumor, there was a significant improvement in the modified McCormick’s grade, when median score was compared between preoperative period and the last follow-up visits (4 [2.8–4] vs. 2 [2–3], P = 0.001). There was one case of surgical mortality, with two symptomatic recurrences after surgical resection. Conclusions: The progression-free survival (PFS) and overall survival (OS) depend on early excision at better functional status. Age, gender, and vertical extension of tumor have a debatable impact on the overall outcome in PIME.