gms | German Medical Science

Introduction: A 44-year old woman presented with chronic back pain and a suspected irritation of the L5 radix on the right hand side. However, neurological examination disclosed no radicular syndrome but a strong gait ataxia, a slight weakness of the left leg and a numbness distal to Th8 on the right side (Cooper-Epstein-Grading I). SSEPs underlined a severe lesion with a loss of somatosensory evoked potentials. Magnetic resonance imaging (MRI) of the thoracic and cervical spine showed a small intramedullary enhancing nodule at the level Th9-10 with a long-segment syrinx formation up to the mesencephalon. These findings suggested an ependymoma or astrocytoma as cause for the syringomyelia.

Material and methods: A well-defined and highly vascularized tumor with a grey hue and fatty consistency was encountered. The tumour was excised en bloc. Histopathology confirmed an endothelial hemangioblastoma (WHO grade I). Our patient showed no other features of VHL which is associated in up to 20% of the cases with hemangioblastoma.

Results: The outcome was good without any new neurological deficites. MRI (day 2 post-op) confirmed the tumorresection. The syrinx was somewhat smaller. Hemangioblastomas are low-grade tumours, highly vascularized, often associated with von Hippel-Lindau’s syndrome (VHL). In the majority of the cases they occur in the posterior fossa. Rarely they may be attached to spinal nerve roots, or at the origin of the filum terminale (about 2% of all spinal cord tumours).

Conclusion: A sporadic hemangioblastoma arising from the medulla should be considered as a rare cause of lower back pain with or without neurological symptoms.Total excision of the tumor offers cure. Then, there is no need for any additional treatment of the syrinx.