Ependymoma grade II, case 8

Clinical history
45-year-old male with a five year history of slowly progressive motor and sensory deficits of the right hand arm. Later on, motor deficits developed in the left leg.

Neurological examination
- motor deficit of the right arm, predominatly in the right hand.
- refexes are present and brisk in the legs and diminished in the arms.
- hypoesthesia of both arms more marked on the right
- gait impairement due to left leg paresis
- urinary disturbances
- McCormick degree of disability grade II

First surgery (September 1990)
On the basis of an initial MRI, not available here, this patient first underwent surgery. Pre-operative diagnosis suggested an astrocytoma. At surgery, no definite cleavage plane could be found. The tumor was partially cystic and hemorrhagic. Biopsy performed during surgery was also suggestive of the diagnosis of astrocytoma and finally, only partial tumor removal was accomplished. Immediately after surgey the patient's neurological condition worsened but returned to the pre-surgical status after three months.
Final histology and special stains led to the diagnosis of low grade ependymoma.
For this reason, the therapeutic strategy chosen was to observe the patient carefully and to reoperate only for new symptoms. Indeed, 18 months later, the motor deficit worsened and MRI was performed in advance of re-intervention

Post-operative MRI (January 1991)
Image 1: Sagittal T1WI. Please describe this post-operative image: how extensive is this tumor? Do you see any findings allowing you to confirm the diagnosis of ependymoma rather than astrocytoma? What kind of cystic component do you see (see -->)?
Images 3 - 4: Sagittal Gd T1WI.

Second surgery (May 1992)
The tumor extending from C4 to T1 was totally resected.

Follow-up MRI (October 1993)
Images 5 - 6: Sagittal T2WI. How would you characterize the surgical result?
Images 7 - 11: Sagittal T1WI. 3D acquisition. Very thin slices permit optimal evaluation of the post-operative changes.
Image 12: Reformated curvilinear coronal T1WI.

DIAGNOSIS:

Ependymoma grade II

Pre-operative MRI
Shows post-operative changes with extensive C2-T1 laminectomy. A well-delineated tumor is seen, extending from C4 to T1. The tumor enhances intensely and the typical cap sign is seen at the caudal end of the tumor, suggesting the diagnosis of ependymoma, rather than astrocytoma. Note the associated bulbar cyst.

Surgery
Total removal of the tumor was achieved.

Post-operative clinical course
Unfortunately, the patient's condition worsened significantly to McCormick IV. The patient was quadriplegic immediately after surgery and regained only partial use of both arms. The patient recovered from urinary disturbances, but remained paraplegic.

Post-operative MRI
Shows total removal of the tumor, with cord atrophy at the site of tumor resection. Note the adhesions between the posterior surface of the spinal cord and the dural sac, and the disappearance of the bulbar cyst.

Comment
- This case illustrates that even on pathological examination, differentiation between astrocytoma and ependymoma may be difficult on the basis of biopsy examined during surgery.
- The final clinical outcome closely correlates with the pre-operative neurological condition and this case illustrates the need for early diagnosis and surgical treatment.
- Ependymoma versus astrocytoma

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