gms | German Medical Science

Objective: Pediatric intramedullary spinal cord astrocytomas are rare, comprising approximately thirty-five percent of intraspinal neoplasms. Low-grade gliomas are the most common spinal cord tumors in children and young adults. Outcome for low-grade astrocytomas is better in children than adults, but not as favourable as that of ependymomas. Depending on the patients’ age of diagnosis, the degree of the preoperative motor deficits, the histological differentiation, the long-term survival and the quality of life for pediatric patients with low-grade gliomas treated by complete resection is often superior than in patients receiving subtotal resection and postoperative radiotherapy. In the majority of spinal astrocytomas progressive neurological deficits due to local recurrence limits survival, whereas spinal seeding seems to be very uncommon.

Methods: Here we present an extremely rare case of a female patient operated and irradiated for a low-grade cervical astrocytoma at the age of 18 months who became symptomatic 20 years after initial surgery with amaurosis and neuropathic radicular pain. The ophthalmologic exam showed a prominent papilledema and following lumbar drainage of approximately 200 ml CSF per day the visual acuity improved. A cranial MRI showed signs of a pseudotumor cerebri. A spinal MRI demonstrated slightly gadolinium enhancing nodules in the cervicothoracic medulla. Leptomeningeal contrast enhancement could be demonstrated. The lumbar MRI showed an strongly gadolinium enhancing intradural tumor of the cauda equina.

Results: Because of the inseparably growth pattern of the infiltrating tumor only an extended biopsy was performed and a glioblastoma was diagnosed. Despite ventriculoperitoneal shunting the visual function rapidly deteriorated to amaurosis. The postoperative MRI now was strongly suggestive of progressive lumbar and cerebellar leptomeningeal tumor dissemination. Palliative 30 Gy radiation therapy was administered to the lumbosacral region and a Temozolomid chemotherapy was started. 2 months after diagnosis the patient died with signs of progressive caudal cranial nerve dysfunction.

Conclusions: Anaplastic progression of spinal astrocytomas even two decades after initial diagnosis, resection and irradiation therapy is rare, but may be associated with CSF metastasis. Once local anaplastic progress is documented, MRI follow-up studies should include the whole spinal canal and the neurocranium to detect and possibly treat leptomeningeal dissemination as early as possible.