EPIDURAL INTRASPINAL CHORDOMA - CASE-REPORT IN A CHILD - REVIEW OF THE LITERATURE

The authors report the case of a chordoma developed exclusively in the epidural space at the L1-L2 level in a 6 year old child. The tumor was easily removed in toto. No post-operative radiotherapy was given. The diagnosis of chordoma was based on the histological characteristics of the lesion and the results of immunohistological studies which were both typical of this lesion. This observation is exceptional because of the young age of the patient, the lumbar level of the tumor and, even more, the absence of any bone involvement. The 8 previously published cases of chordoma developed along the craniospinal axis but without bone involvement are reviewed and analysed: all were subdural; 7 were intracranial (6 prepontic, 1 suprasellar): only one was intraspinal. These lesions raise the problem of their diagnosis, of their treatment and of their embryogenesis. They could develop from ectopic notochordal remnants: the <<ecchondrosis physaliphura>>.