Intramedullary spinal cord tumour associated with neurofibromatosis type 1.

Abstract

We investigated the characteristic features of intramedullary spinal cord tumour associated with neurofibromatosis type 1 (NF-1). We have experienced 44 cases of pathologically confirmed intramedullary spinal cord tumour. Diagnosis of NF-1 was done according to the criteria set by the National Institute of Health Consensus Development Conference. Within the described population NF-1 was diagnosed in two patients. Both the patients were male and histopathologically the tumours were anaplastic astrocytoma and glioblastoma multiforme respectively. 6 cases of NF-1 associated with intramedullary spinal cord tumour based on the above diagnostic criteria have so far been reported, including our 2 cases. Of these 6 cases, 5 were male and in one sex was not described. The tumour was an astrocytoma in all 6 cases. This finding suggested that intramedullary spinal cord tumour associated with NF-1 tends to occur predominantly in males and that histopathologically the tumour is likely to be an astrocytoma. We conclude that the criteria proposed by the National Institute of Health Consensus Development Conference are contributory in making an accurate pre-operative pathological diagnosis of intramedullary spinal cord tumour associated with NF-1.