Metastatic supratentorial ependymoma: A case presentation and systematic review of the literature

Abstract

This study presents a rare case of metastatic supratentorial ependymoma with extra-axial dissemination and includes a systematic review of 23 published cases. Our patient was a 27-year-old male initially diagnosed with a CNS WHO Grade II tumor, which later demonstrated ZFTA translocation and progressed to CNS WHO Grade III disease with multiple recurrences and metastases to the bone and liver, ultimately resulting in death from tumor progression and septic shock. The systematic review included English-language studies published between 2000 and 2023 and analyzed patient demographics, primary tumor location, and metastatic patterns. Most reported cases involved supratentorial, CNS WHO Grade III ependymomas, with a median age of 16.64 years and no apparent gender predominance. Lymph nodes, lungs, and the integumentary system were the most frequent metastatic sites, supporting both lymphatic and hematogenous routes of dissemination, while no association was identified between WHO Grade, primary tumor location, and metastatic distribution. Surgical resection with adjuvant radiotherapy remains the standard treatment for primary tumors, whereas chemotherapy is most commonly used for metastatic disease; however, the rarity and complexity of extra-neural metastatic ependymoma highlight the importance of molecular profiling and larger datasets to improve management strategies