Abstract
ABSTRACT
Pediatric intramedullary low‐grade gliomas (PIMLGGs) are rare neoplasms that present unique clinical and management challenges. Although surgery remains the primary tool for tissue diagnosis and decompression, complete gross‐total resection is frequently unfeasible due to the infiltrative nature of these lesions. To define the role of chemotherapy, we conducted a Preferred Reporting Items for Systematic Reviews and Meta‐Analyses (PRISMA)‐based systematic review of 21 studies published between 2000 and 2025, comprising 98 patients who started chemotherapy at a median age of 3.9 years (ranging from 3 months to 17.7 years). In half of the cases, chemotherapy was used as primary therapy in cases of incomplete resection or biopsy, and half of the patients received salvage chemotherapy in cases of progressive tumor or recurrence. A platinum‐based regimen was used in most of the patients. Complete response was achieved in 9% of cases, 9% had an incomplete response, and 42% had stable disease. Disease progression occurred in 26% of patients, and 15% died, most commonly due to disease progression. Median follow‐up was 5 years (ranging from 3 months to 18 years).