Intramedullary mature teratomas in adults are rare tumors that pose significant diagnostic and therapeutic challenges due to their diverse tissue composition and intramedullary location. This review, which includes an illustrative case, aims to provide a comprehensive overview of the clinical presentation, diagnosis, treatment, and outcomes of adult intramedullary mature teratomas through a systematic review of 89 cases. A systematic review was conducted following PRISMA guidelines, using keyword combinations in PubMed, MEDLINE, and Web of Science databases until June 2024. Inclusion criteria were adult patients (> 18 years) with histopathologically confirmed intramedullary mature teratomas. Statistical analyses examined relationships between the extent of resection (EoR), tumor location, and patient outcomes. Among the 89 cases, the mean age was 39.94 ± 13.52 years, with a male-to-female ratio of 1.39:1. Tumors were most commonly located in the conus region (51.2%). Surgical resection was the primary treatment, with 43 cases undergoing partial resection and 33 gross total resection. No statistically significant differences in outcomes were found between EoR, sex, associated anomalies, or specific outcomes. However, younger patients showed improved outcomes for lower limb weakness and sphincter dysfunction. Recurrence was noted in three cases, all located in the conus. Intramedullary mature teratomas require a nuanced approach that balances complete resection and neurological preservation. Early diagnosis and individualized surgical planning are crucial for optimizing outcomes. Despite the challenges, effective management is achievable, and ongoing research is essential to refining treatment strategies for this rare tumor.
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