Intracranial Chordoma in Children

Chordoma has been generally regarded as a neoplasm of adult life, derived from remnants of notochord. It can occur anywhere along the axial skeleton but is most frequently found at the two poles; in the sacrococcygeal region and intracranially in the clivus. Sacrococcygeal chordomas are the most frequent of these tumors in children and were reviewed in 1933 by Montgomery and Wo lman.1Clival chordomas in children occur infrequently and have received even less attention. This report deals with the presence of a chordoma of the clivus, metastatic to the lungs, in a 21/2-yearold boy who, after review of the literature, would appear to be the youngest recorded case with this disease. In addition, the clinical, radiographic, and pathologic features of intracranial chordoma in children are reviewed and discussed. Report of a Case The patient's illness began when he was 21/2 years old with rapid onset of bilateral