Anomalous hemorrhage in myxopapillar ependymoma: illustrative case

Abstract

BACKGROUND

This is the case of a 22-year old male with bilateral leg pain caused by an intradural lesion at the L3 level. On MRI, there were no signs of hemorrhage. Resection was performed and a raspberry-like lesion with attached veins was identified. Histopathological analysis showed a myxopapillary ependymoma.

OBSERVATIONS

Although hemorrhage is rare, myxopapillary ependymomas are highly vascularized and carry the highest risk of intratumoral bleeding among spinal ependymomas. In the literature, most patients with hemorrhage of myxopapillary ependymomas present with neurological deterioration. Rarely are these patients noted to be clinically stable.

LESSONS

Despite no worsening of symptoms or preoperative evidence of hemorrhage, intraoperative findings revealed a hemorrhagic lesion attached to vascular structures and potentially the filum terminale. Hemorrhage in a myxopapillary ependymoma without clinical deterioration is a rare occurrence.

https://thejns.org/doi/10.3171/CASE25791

This report describes an intradural extramedullary lesion. Preoperative MRI showed no evidence of hemorrhage, nor was there any deterioration of preoperative neurological symptoms. However, intraoperative macroscopic and microscopic findings revealed a rare case of hemorrhage within an myxopapillary ependymoma. Hemorrhage in a myxopapillary ependymoma without associated clinical deterioration is an uncommon occurrence.

Illustrative Case

A 22-year-old male with no prior medical history presented to the outpatient clinic with bilateral leg pain persisting for 2 years. The sharp pain was localized to the posterior aspect of both upper legs, radiating to the lateral lower legs and terminating at the ankles. Symptoms initially began in the left leg and, in recent months, also developed in the right leg. The pain was aggravated by movement and alleviated during periods of rest. There were no motor or sensory deficits in the legs and no symptoms of cauda equina syndrome. The patient had only complaints of stiffness of the back without pain.

Neurological examination revealed no motor or sensory deficits. However, lumbar flexion was limited to 20° due to stiffness.

An MRI study of the lumbar spine with contrast conducted in March 2025 revealed a 1.9-cm homogeneously enhancing intradural lesion at the L3 level (Fig. 1). A linear structure extended cranially toward the conus, radiologically suspected for a vascular structure or an enhancing spinal nerve. The differential diagnosis included neurofibroma, schwannoma, and meningioma. There were no signs of hemorrhage (also not on T2-weighted images), and given the short length of the lesion, a myxopapillary ependymoma was considered less likely. MRI of the neuraxis did not reveal any additional lesions.

FIG. 1.

Sagittal T1-weighted (A), sagittal T1-weighted contrast-enhanced (B), and axial T1-weighted contrast-enhanced (C) MR images, revealing a 1.9-cm homogeneously enhancing intradural extramedullary lesion at the L3 level.

Resection was discussed with the patient and subsequently performed in April 2025. The L2–3 level was confirmed using lateral fluoroscopy. After making an incision and bilaterally dissecting the paravertebral muscles, the spinous processes of L2, L3, and L4 were exposed. Following final confirmation of the level with intraoperative fluoroscopy, a laminectomy of L3 was performed. The L3 nerve roots were identified. Throughout the procedure, manipulation caused reactivity in the right leg. A linear dural incision was made under the microscope. Between the cauda equina roots, a reddish, raspberry-like lesion was identified, protruding on the left side (Fig. 2). A clear dissection plane was visible between the lesion and the nerve roots, suggesting the lesion did not originate from the roots (Fig. 2). Cranially, two structures resembling veins were attached to the tumor, with a similar structure noted caudally (Fig. 3). Closer inspection identified one cranial structure as the filum terminale (Fig. 4). Intraoperative neuromonitoring applied to these structures elicited no response in the lower limbs. After confirming this, the attached structures were coagulated and transected, and the lesion was resected and sent for histopathological analysis (Figs. 3 and 4). The dura was closed using Vicryl sutures.

FIG. 2.

Image exposing the reddish-colored tumor on the left side after linear incision of the dura. Cranially, a venous structure appears. The tumor is dissected from the caudal nerve roots.

FIG. 3.

Cranial to the tumor, a venous structure appears that is transected.

FIG. 4.

Cranial to the tumor, a possible filum terminale is identified. It appears to be attached to the tumor. The filum terminale is transsected.

Intraoperatively, the differential diagnosis shifted from neurofibroma or schwannoma to myxopapillary ependymoma with hemorrhage, or possibly a cavernoma.

Postoperatively, the patient recovered well, reporting reduced leg pain and decreased low back stiffness. He was discharged 2 days after surgery. Histopathological evaluation confirmed the diagnosis of a myxopapillary ependymoma, CNS WHO grade 2, MGMT methylation, gain of chromosomes 7, 9, 18, and 19, with extensive hemorrhage and associated fibrinous material and iron deposition. At the 3-month follow-up, the patient reported no complaints and was able to function normally in daily life. MRI of the lumbar spine revealed minimal contrast enhancement in the cauda equina, which may represent postoperative changes or a small residual tumor (Fig. 5). A subsequent MRI study was scheduled for 6 months later.

FIG. 5.

Postoperative sagittal (A) and axial (B) T1-weighted contrast-enhanced MR images of the lumbar spine, demonstrating a small enhancing structure at the level of the cauda equina, which may represent residual tumor tissue or a postoperative change.

Informed Consent

The necessary informed consent was obtained in this study.

Discussion

Myxopapillary ependymoma is a slow-growing, glial-derived tumor,^1–3 accounting for 13%–27% of all spinal ependymomas.³ It most commonly occurs in young adults, with an incidence of less than 0.01 per 100,000 persons.¹ The typical presentation involves a prolonged history of neurological symptoms.^1–3 Although hemorrhage is rare, myxopapillary ependymomas are highly vascularized and carry the highest risk of intratumoral bleeding among spinal ependymomas.³ Hemorrhage may be associated with acute onset of cauda equina syndrome or paresis.^1,3,4

Observations

In this case, the etiology of the hemorrhage was unclear. The hemorrhage was not identified on preoperative MRI, despite the acquisition of both T1- and T2-weighted sequences. This suggests that the hemorrhage may have developed after the imaging was performed. The patient did not develop any neurological deficits attributable to the hemorrhage; therefore, emergency surgical intervention was not indicated.

Most myxopapillary ependymomas are located intradural and extramedullary.³ On MRI, they are typically isointense on T1-weighted images and show homogeneous postcontrast enhancement,³ consistent with our imaging findings. The gold standard treatment is gross-total resection.³ Subtotal resection or capsular violation increases the risk of local recurrence or dissemination throughout the CNS.³

In our case, an intraoperative hemorrhage was observed despite no preoperative symptom worsening or radiological signs of bleeding. This aligns with a case described by Choi et al.,² in which a patient with an intradural extramedullary lesion actually showed signs of hemorrhage on MRI, but did not experience increased neurological symptoms. That lesion was initially suspected to be a schwannoma, as in our case, but histopathological analysis revealed an ependymoma, but not of the myxopapillary subtype.²

Other reported cases by Tonogai et al.,¹ Albadr et al.,³ and Heuer et al.⁴ described patients with myxopapillary ependymomas who presented with neurological deterioration due to hemorrhage. Tonogai et al.¹ noted MRI evidence of bleeding and intraoperative xanthochromic fluid in the subarachnoid space. Their tumor also appeared as a reddish soft mass, similar to our intraoperative findings. Albadr et al.³ similarly reported a reddish lesion confirmed as a myxopapillary ependymoma on pathological analysis.

Lessons

We report the case of a 22-year-old male with long-standing posterior leg pain due to an intradural extramedullary lesion, ultimately diagnosed as a myxopapillary ependymoma. Despite no worsening of symptoms or preoperative evidence of hemorrhage, intraoperative findings revealed a hemorrhagic lesion attached to vascular structures and potentially the filum terminale. Hemorrhage in a myxopapillary ependymoma without clinical deterioration is a rare occurrence.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: all authors. Acquisition of data: all authors. Drafting the article: Caelers, van Nie. Critically revising the article: all authors. Reviewed submitted version of manuscript: van Nie, Smeets. Approved the final version of the manuscript on behalf of all authors: Caelers. Administrative/technical/material support: Caelers, Smeets. Study supervision: Smeets.

Correspondence

Inge J. M. H. Caelers: Zuyderland Medical Centre, Heerlen, The Netherlands. inge.caelers@mumc.nl.