Fatal holocord recurrence of a pregnancy-related, low-grade spinal ependymoma: case report and review of an unusual clinical phenomenon

Introduction: Pregnancy-related spinal tumors (PRSTs) are unusual tumors that present during pregnancy or within a year after delivery. We describe a fatal holocord recurrence of a spinal ependymoma, which, to the best of our knowledge, is one of the most extensive PRSTs reported thus far.

Case presentation: A 21-year-old primigravida presented at 6 months of gestation with urinary incontinence for 2 months and spastic paraparesis for 1 month. MRI showed a conus intramedullary lesion from T10 to 12. Near-total resection of the lesion was performed. The histopathological diagnosis was that of a cellular ependymoma (WHO grade II). The patient presented 6 months later with progressive quadriparesis and breathing difficulty. MRI demonstrated holocord recurrence of the tumor with edema extending to the pontomedullary junction. The patient succumbed to respiratory failure before decompression of the tumor could be performed.

Discussion: This case highlights an unusual clinical course of a pregnancy-related, low-grade spinal ependymoma. The possible hormonal and genetic mechanisms underlying the aggressive involvement of the entire spinal cord by the recurrent tumor are discussed in the light of a literature review. Future studies may shed light on the possibility of utilizing these mechanisms as therapeutic targets to alter the clinical course of aggressive spinal ependymomas.