Preoperative Intracranial Dissemination of Spinal Myxopapillary Ependymoma Attributed to Tumor Hemorrhage

Abstract

Background: Spinal myxopapillary ependymoma (SME), generally considered a benign entity, can exhibit brain and whole-spine metastases as well as local recurrence after surgery. However, the presence of preoperative retrograde intracranial dissemination at the time of diagnosis is very rare.

Case description: We report a case of SME in a 22-year-old man who presented with acute exacerbation of chronic back pain shooting down both thighs and weakness in both legs. Magnetic resonance imaging of the brain and whole spine showed an enhancing mass occupying the majority of the spinal canal at the L1-L2 level and multiple foci dissemination, including in the right pons, lateral midbrain, and occipital lobe, and at the C7, Th6, L4, and S2 levels of the spinal canal at the time of diagnosis. On gross total removal of the dominant tumor located at the L1-L2 level, severe intradural arachnoiditis and syrinx filled with xanthochromic cerebrospinal fluid was noted, indicating the presence of previous tumor hemorrhage. Histopathologic analysis of the tumor supported SME diagnosis, and <1% of cells showed Ki-67 expression. We speculated that distant retrograde dissemination could have been attributed to metastatic spread through cerebrospinal fluid caused by tumor hemorrhage, which may explain distant dissemination despite low expression of Ki-67.

Conclusions: Screening of the whole brain and spine at the time of diagnosis is imperative when tumor is detected at any level of the neuraxis. The present case of SME with a preoperative intracranial lesion is the fifth case documented in the medical literature.

Keywords: Gross total removal; Preoperative intracranial dissemination; Spinal myxopapillary ependymoma; Tumor hemorrhage.