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Lumbar spine ependymoma (WHO grade 2)

Case contributed by James Sheldon
Diagnosis certain

Presentation

New onset right sided parasthesia in lumbosacral distribution. Reduced anal tone.

Patient Data

Age: 25 years
Gender: Male

An expansile intramedullary mass arises from the spinal cord and extends from the T11 to L4/5 vertebral levels. It demonstrates isointense T1 signal and hyperintense T2 cord signal compared with normal cord.

Avid enhancement throughout the mass, with only small regions of non/less prominent enhancement. Scalloping of the posterior lumbar vertebral bodies, particularly L2-L4, with the tumor extruding through and widening the lumbar neural exit foramen bilaterally from T12-L3 (most prominent at L3).

The lesion has maximal dimensions of 34mm (trans) x 16mm (AP) at the L2 vertebral level.

No evidence of hemorrhage into or surrounding the tumor. Enhancing serpentine vessels along the lower thoracic spinal cord suggest venous hypertension.

There is straightening of the normal lumbar lordosis.

Bone marrow signal is normal at all levels.

Bilateral extrarenal pelvis and hydroureteronephrosis.

Conclusion:

Enhancing, well-circumscribed intramedullary lower thoracic/lumbar spinal cord mass is most likely a myxopapillary ependymoma, although the extensive intramedullary component is unusual. An ependymoma is another albeit less likely possibility. Given the inferior extension, an astrocytoma is thought unlikely (unless within a low lying conus - note no Chiari), as are schwannomas (which should enhance vividly).

Case Discussion

Biopsy result

The sections show a moderately cellular tumor, forming perivascular pseudorosettes. The tumor cells form cuffs around blood vessels. The tumor cells have mildly enlarged round to ovoid nuclei and small nucleoli. There is no myxoid material in the background. Mitoses are inconspicuous. There is no microvascular proliferation or necrosis. The tumor cells are diffusely GFAP positive. There is focal EMA positivity with perinuclear dot-like pattern. The Ki-67 index is up to about 3%. The features are those of ependymoma.

DIAGNOSIS: Lumbar spinal tumor: Ependymoma (WHO Grade II).

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