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

Abstract

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.

Fig. 1. Plain MRI of the spine showing an intramedullary lesion in the region of the conus.

a Sagittal T1-weighted image showing an isointense lesion extending from T10 to T12, b Sagittal T2-weighted image demonstrating the lesion to be hyperintense. A ventrally exophytic component is noted. c T2-weighted axial sequence at T11 level showing an enlarged cord and the exophytic component. d Screening T2-weighted image of the cervical spine and e whole-spine screening showing no other lesion.

Fig. 2. Histopathological examination demonstrating features of a cellular ependymoma (WHO Grade II).

Paraffin section of the lesion showing a cellular tumor with: a round to oval cells arranged in fibrillary matrix with strong and diffuse GFAP positivity (Inset), b high-power view of the same demonstrating attempted ependymal rosette formation (arrow), and c paranuclear dot positivity for EMA (arrow). [Hematoxylin and eosin: a ×40; b ×400] [Avidin Biotin Complex immunoperoxidase (A-inset) ×400; c ×400].

Fig. 3. Thoracic spine MRI showing a diffusely enlarged spinal cord with a holocord intramedullary lesion.

a T2-weighted sequence demonstrating the lesion to be predominantly hyperintense with areas of mixed intensity in the conus. b Gadolinium enhanced T1-weighted sagittal image showing heterogeneous enhancement in the dorsal spine. c T2-weighted axial sequence at T10–11 level showing an enlarged cord.

Fig. 4. Holocord recurrence of the lesion.

a Sagittal T1-weighted image of the cervical spine demonstrating an isointense lesion expanding the cord; b Sagittal gadolinium enhanced T1-weighted image showing heterogeneous contrast enhancement in the cervical region; c Sagittal T2-weighted image showing the hyperintense intramedullary lesion extending up to C3 (arrow) with edema extending cranially to the pontomedullary junction. d Sagittal T2-weighted image of the whole spine showing a diffuse holocord involvement by the lesion. Diffuse thickening of the cauda equina is noted as well.