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Thoracic Exophytic Ependymoma With Severe Spinal Cord Compression

History

This is a case of a 50-year-old female with a history of progressive lower extremity weakness and numbness over the past 6 months. Her weakness progressed to the point that she became wheelchair bound when she presented to us. She also reported diffuse numbness in the lower abdomen and both legs, and bowel and bladder dysfunction.

Examination

On examination, the patient was awake and responsive, and oriented to person, place and time. The cranial nerves (II-XII) intact; there was no pronator drift; bilateral upper extremities are 5/5 with sensation intact; bilateral lower extremities are 3/5 with diffuse numbness, decreased sensation to pinprick, light touch and temperature; no sensation below T7; patellar/Achilles reflexes +1; and bilaterally, toes are downgoing.

Pretreatment Imaging

Figures 1A-1D (below) respectively demonstrate T1 isointense, heterogeneously T2 hyperintense intradural extramedullary lesion in the spinal canal extending from T7 through T10 with heterogeneous post-contrast enhancement. The mass measures 7.6cm craniocaudal by 1.4cm anteroposterior by 1.5cm transverse.

thoracic sagittal MRI, T1 isointenseFigure 1A

thoracic sagittal MRI shows heterogeneously T2 hyperintense intradural extramedullary lesion, T7-T10Figure 1B

thoracic sagittal MRI shows heterogeneously T2 hyperintense intradural extramedullary lesion, T7-T10, post-contrast enhancementFigure 1C

T8, thoracic axial MRI shows heterogeneously T2 hyperintense intradural extramedullary lesion, T7-T10, post-contrast enhancementFigure 1D (above): T8

Diagnosis

Thoracic spinal cord ependymoma.

Suggest Treatment

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Selected Treatment

Posterior thoracic laminoplasty with gross total resection. The pathology was consistent with WHO (World Health Organization) grade II ependymoma.

Post-Treatment Imaging

Figures 2A-2C (below). T1 pre- and post-contrast, and T2 weighted MRI images demonstrate gross total resection of the tumor.

T1 pre-contrast thoracic sagittal MRI demonstrates gross tumor resectionFigure 2A

T1 post-contrast thoracic sagittal MRI demonstrates gross tumor resectionFigure 2B

thoracic sagittal T2 weighted MRI demonstrates gross total tumor resectionFigure 2C

Outcome

The patient did very well. Her lower extremity weakness and numbness were significantly improved, as well as her gait and balance. She could walk using a walker on post-operative day 2. At her one-month  post-operative follow-up, she was walking using a cane.

Case Discussion

The size of the tumor on the thoracic MRI is impressive. The differential includes a spinal meningioma or schwannoma. The ependymoma is presumed to be intramedullary with an exophytic mass.

Clearly, surgical intervention is warranted. Further evaluation of the entire neuroaxis is also prudent with MRIs of the cervical, lumbar, and brain to assess for other lesions or drop metastases. The post-operative MRI suggests an excellent gross total resection.

After gross total resection of an intracranial WHO grade II ependymoma, limited field fractionated external beam radiotherapy (LFFEBRT) can be considered. Post-operative LFFEBRT is recommended for WHO grade II ependymoma when subtotal resection is noted on the post-operative MRI and for grade III anaplastic ependymoma regardless of the extent of resection. Radiation for the spine cord after a gross total resection is more controversial.

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