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Astrocytoma, case 6

 

Clinical history
This 16-year-old girl complained of a two week history of vertigo and progressive weakness of both legs, with rapid progression over the three days prior to evaluation.
Notably, the parents recalled that 10 days after smallpox vaccination, their then one and one half-year old child rapidly developed left arm monoplegia, which was considered to be a severe complication of a late vaccination. For many years the patient has had torticollis and a left deviation of the head. Plain X-rays documented a progressive scoliosis.

Initial neurological examination revealed spastic paraparesis with exaggerated deep tendon reflexes and an abnormal bilateral extensor plantar response. Degree of disability McCormick grade 2 and urinary disturbances were present.

Pre-operative MRI (April 1990)
Images 1 - 5: Sagittal Gd T1WI. Massive spinal cord enlargement is seen, caused by a mainly polycystic tumor with several enhancing ill-defined solid components. Note the pathological enlargement of the spinal canal with scalloping of the vertebral bodies.

Surgery (May 1990) 
Video 1: astrocytoma
The tumor could only be partially resected.

Stage 1: Infiltrating tumor.
Stage 2: Biopsy.
Stage 3: Opening of the intratumoral cyst.
Stage 4: Attempt to dissect the tumor- no cleavage plane identified.
Stage 5: Debulking with CUSA (Cavitron Ultrasonic Surgical Aspirator).

Histology
Low grade astrocytoma.

Post-operative MRI (June 1990)
Images 6 - 10: Sagittal T1WI. Extensive laminectomy was performed from C3 to C7. The cord is still markedly enlarged. The multiple cystic components are still visible. Note post-operative hemorrhagic changes at the caudal extent of the tumor (see -->).
Images 11 - 14: Sagittal Gd T1WI. Contrast enhancement is seen within the wall of the various cysts.
Image 15: Coronal Gd T1WI. Further demonstrates the anatomic relation of this complex tumor.

Post-operative clinical course
At first the patient's condition worsened to McCormick grade 3, but after three months the patient recovered partially, regained strength in both legs and could again walk.

Second follow-up MRI (June 1994)
Image 16: Sagittal T1WI.
Image 17: Sagittal PD image.
Images 18 - 20: Sagittal T2WI.
Image 21: Sagittal Gd T1WI.
Images 22 - 24: Axial Gd T1WI.
This follow-up MR shows slowly progressive tumor infiltration with the same characteristics as described earlier. Note on the T2WI areas of decreased signal at the inferior part of the tumor, corresponding to hemosiderin deposits at the site of previous hemorrhage (see image 20).

Follow-up
In 1995, the MRI images were similar to those shown in 1991: multiple cysts were seen mixed with ill defined solid components enhancing heterogeneously after contrast injection.
In 1997, spontaneous evolution is seen with dramatic collapse of the cystic elements.

Control MRI in 1997
Image 25: Sagittal T1WI
Image 26: Sagittal T2WI
Image 27: Sagittal Gd T1WI
Image 28: Axial Gd T1WI

The cervical spinal cord has still an irregular shape and the remaining tumor infiltration demonstrates ill-defined heterogeneous enhancement after contrast injection. The global size of the cervical cord has significantly decreased.
On T2WI, multifocal low signal intensity areas corresponding to hemosiderin deposits persist.

Clinical evolution: neurological examination remained completely unchanged.
A new annual follow-up MRI was again obtained in 1998.
Image 29: Sagittal T1WI
Image 30: Sagittal T2WI
Image 31: Sagittal Gd T1WI
Image 32: Axial Gd T1WI

The MRI images are similar to those obtained in 1997. The cystic components remain very small.

This uncommonly long follow-up illustrates the natural clinical evolution of low grade astrocytomas and favors the recommendation to refrain from giving radiotherapy with potential deleterious effects to the cord.

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Fig. 1

Astrocytoma, case 6, Fig. 1
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