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Astrocytoma, case 3

 

Clinical history
This 30-year-old patient works as a gardener. His medical history goes back 15 years, when communicating hydrocephalus was discovered and shunted. This hydrocephalus was attributed to arachnoiditis. For about 10 years the patient complained of slowly progressive loss of strength and increasing numbness in the right arm. These sensory motor deficits slowly progressed year after year, and anesthesia and major paresis of the right hand occurred. A few months before presentation new symptoms were reported, manifested by loss of sensation and paresis of the right leg with gait impairment. For two weeks the patient suffered from headaches, vertigo, and profound fatigue. Brain CT demonstrated a cystic lesion in the upper vermis and cervical CT showed multiple cystic lesions in the cervical cord with peripheral enhancement.

Neurological examination
At neurological examination significant right scapular and brachial muscular atrophy was observed, with complete loss of sensation and deep tendon reflexes. A pyramidal syndrome was clearly evident, involving the left arm and both legs. Motor strength was reduced in both the left arm and the legs. Grade of disability: McCormick II. No urinary disturbances were identified.

Pre-operative MRI (February 1989)
Images 1 - 3: Sagittal T1WI. A large cyst in the upper cerebellar vermis is seen as a well-delineated low signal intensity area. In the cervical spinal cord, from C1 to T1, multiple cystic lesions are seen, capped by hypointense areas, suggestive of hemosiderin deposits.
Images 4 - 6: Sagittal Gd T1WI. Multiple patchy areas of contrast uptake are seen at the periphery of the cervical cyst and at the upper part of the vermian cyst.

At this time, the differential diagnosis included hemangioblastoma, cystic astrocytoma, and syringomyelia secondary to arachnoiditis.

Surgery
Multiple cystic lesions were found at surgery, emptied and marsupialized. Multiple biopsies were performed and spinal cord decompression was achieved. The surgeon had the strong impression that he was dealing with a glial tumor with multiple old hemorrhages.

Histology
Dysplastic astrocytoma.
In dysplastic astrocytomas cellularity is low. They are often nodular and contain only few abnormal nuclei, but without mitosis. The name "dysplastic astrocytoma" has been attributed to their similarity with gliosis or with tumors encountered in certain phacomatoses. As in pilocytic astrocytomas, a cleavage plane can be more easily be found at surgery, compared to "ordinary astrocytomas". These tumors correspond to grade I of the WHO classification.

Post-operative clinical course
Immediately after surgery the patient's neurological condition worsened with transient tetraparesis, resolving after a few weeks. Slow but progressive neurological improvement was observed. One year later, the patient presented with McCormick grade of disability II, and moderate spasticity persisted, as well as hypoesthesia starting at the C2 level.

Post-operative MRI (January 1990)
Image 7: Sagittal PD image.
Images 8 - 11: Sagittal GE T2WI.
Compared to the pre-operative study, the spinal cord has decreased in size, but there are extensive and persistent areas of low signal intensity, corresponding to hemosiderin deposits. Persistent small cystic components are shown as high signal intensity areas.

Follow-up MRI (January 1992)
Image 12: Sagittal T1WI of the brain. A persistent cystic lesion is seen in the upper vermis.
Image 13: Sagittal Gd T1WI. The enhancing nodule located at the upper part of the cyst is unchanged. At the level of the posterior medulla oblongata enhancing areas are also seen.
Image 14: Axial Gd T1WI help to identify the exact location of the enhancing lesion within the medulla oblongata.
Image 15: Sagittal PD and 16: Sagittal T2WI images of the spine. These follow-up images show the cervical lesions unchanged.

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Fig. 1

Astrocytoma, case 3, Fig. 1
Astrocytoma, case 3, Fig. 2
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Astrocytoma, case 3, Fig. 11
Astrocytoma, case 3, Fig. 12
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Astrocytoma, case 3, Fig. 14
Astrocytoma, case 3, Fig. 15
Astrocytoma, case 3, Fig. 16