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Myxopapillary ependymoma of the conus, case 2

 

Clinical history
This 15-year-old boy complained of two prior episodes of nocturnal back pain lasting for several days, both of which resolved completely. The patient now complains of weakness in both legs, gait impairment, a burning sensation in the soles of both feet, and back pain.

Neurological examination revealed paraparesis with loss of deep tendon reflexes, Lasègue sign bilaterally, loss of pain and temperature sensation in the territory of the L4, L5 and S1 roots bilaterally.

McCormick degree of disability III and urinary disturbances were present.

Pre-operative MRI (March 1990)
Images 1 - 2: Sagittal T1WI. Hypointense, ill-defined partially cystic mass anteriorly located at the level of T11, T12, and L1. Note also the presence of a second lesion at the bottom of the dural sac.
Images 3 - 5: Sagittal Gd T1WI. After gadolinium injection, the masses markedly enhance. The cystic intratumoral component is better seen on image 4.

Surgery performed immediately after the MR examination confirms the presence of two sites of tumor infiltration.

Histology
Myxopapillary ependymoma grade II.

Post-operative clinical course
The patient recovered partially to a McCormick stage II and three months later to McCormick I. Radiation therapy was delivered and the patient did well for two years. Several postoperative MRI's were normal.

In March 1992, a routine follow-up MRI discovered metastatic nodules.

Follow-up MRI (June 1994)
Images 6 - 8: Sagittal T1WI centered on the thoracolumbar region, the site of previous surgery. Postoperative changes are seen and at the superior edge of image 8, observe the presence of an isointense lesion: extra- versus intramedullary metastatic lesion?
Images 9 - 13: Sagittal Gd T1WI. Intense contrast uptake of a nodular lesion. The inferior limits of the tumor are clearly defined (T10): the extra-medullary location may now be ascertained.
Images 14 - 16: Sagittal Gd T1WI are obtained at the cervico-thoracic level: additional extramedullary metastatic lesions are found.
Images 17 - 18: Sagittal Gd T1WI of the brain show an enhancing metastatic lesion in the hypothalamic region.

The patient underwent surgery to remove the larger mass at the level of T9-T10, with subsequent chemotherapy.

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- Tumor classification

 

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Fig. 1

Myxopapillary ependymoma of the conus, case 2, Fig. 1
Myxopapillary ependymoma of the conus, case 2, Fig. 2
Myxopapillary ependymoma of the conus, case 2, Fig. 3
Myxopapillary ependymoma of the conus, case 2, Fig. 4
Myxopapillary ependymoma of the conus, case 2, Fig. 5
Myxopapillary ependymoma of the conus, case 2, Fig. 6
Myxopapillary ependymoma of the conus, case 2, Fig. 7
Myxopapillary ependymoma of the conus, case 2, Fig. 8
Myxopapillary ependymoma of the conus, case 2, Fig. 9
Myxopapillary ependymoma of the conus, case 2, Fig. 10
Myxopapillary ependymoma of the conus, case 2, Fig. 11
Myxopapillary ependymoma of the conus, case 2, Fig. 12
Myxopapillary ependymoma of the conus, case 2, Fig. 13
Myxopapillary ependymoma of the conus, case 2, Fig. 14
Myxopapillary ependymoma of the conus, case 2, Fig. 15
Myxopapillary ependymoma of the conus, case 2, Fig. 16
Myxopapillary ependymoma of the conus, case 2, Fig. 17
Myxopapillary ependymoma of the conus, case 2, Fig. 18