Spinal CSF drop metastasis of an unsual malignant glioneuronal tumour of the brain

Cerebral malignant glioneuronal tumours (MGNT) are rare and therefore hardly described in literature. Histopathologically they are characterized by mixture of glial- and neuronal-type differentiated cells. MGNT show high polymorphism but are generally similar to malignant glioma (WHO classification Grade III and IV), so they may be underdiagnosed. Despite this similarity they demonstrate a special entity of tumours with a typical pathology and differing clinical characteristics. Here, we report an unusual case of a malignant glioneuronal tumour with spinal metastasis.

Case report: A 52-year old man presented with acute onset of fronto-temporal headache. On examination he had no further neurological deficit. Initial noncontrast CT-tomography revealed an right temporal lobe abnormality nearby the basal ganglia, interpreted as an atypical haemorrhage. The headache ceased and further cerebral imaging (CT, MRI) showed a regredient bleeding without further pathology. Additional cerebral angiogram was normal. Two month after initial presentation he was admitted again because of recurrence of a strong headache. MR- and CT-tomography showed a new cerebral haemorrhage localized in place of the initial bleeding. A neurosurgical temporoparietal craniotomy and evacuation was performed that revealed a tumour. Therapeutically an extensive tumour debulking was carried out within the same surgery. The postoperative course was uneventful and after discharge the patient underwent radiotherapy and chemotherapy. Three month later the patient presented with gait difficulty due to a right accentuated flaccid paraparesis with a sensory level at T.10. Additionally, he reported urenary retention and constipation. Spinal MRI showed one intra- and one extramedullary mass, that were diagnosed as drip metastasis of the initial glioneuronal tumour. Therapeutically, a neurosurgical laminectomy was carried out. Within six weeks the patient experienced a rapid clinical detoriation with paraplegia and progressive loss of consciousness. He expired seven month after initial cerebral haemorrhage.

Discussion: Glioneuronal tumours are a rare and hardly desribed entity of malignancy. MGNT have a marked tendency to metastasize, which is uncommonly seen in other malignant glioma. Although this subtyp of tumour shows a high polymorhism in histopathology and clinical course, our MGNT seems somehow special with a faster fatal progression and a particular cellular composition.