Myxopapillary ependymoma with pleuropulmonary metastases and high plasma glial fibrillary acidic protein levels.

Introduction Myxopapillary ependymoma is a rare glial tumor with almost exclusive localization in the distal spinal cord (intraspinal variant) or the sacrococcygeal subcutaneous tissue (extraspinal variant). The average age at manifestation has been reported to be approximately 36 years. The overall incidence is approximately 0.08 per 100,000 persons per year for males and 0.05 per 100,000 persons per year for females. Myxopapillary ependymoma typically lacks histopathologic signs of malignancy and corresponds to WHO grade 1. In this article, we report a rare case of extraspinal myxopapillary ependymoma with multiple pleuropulmonary metastases detected 24 years after initial diagnosis. The case is of interest for two reasons. First, distant metastases of myxopapillary ependymoma are exceedingly rare. Second, we found high levels of glial fibrillary acidic protein (GFAP) in the blood plasma of our patient, which corroborated the notion that this protein may be a useful circulating biomarker for selected glioma cases.