Cranial chordomas in infancy and childhood. A report of two cases and review of the literature

Abstract

Cranial chordomas are uncommon, accounting for less than 1% of all intracranial neoplasms. Although they are presumed to arise from congenital notochordal remnants, it is rare for these tumors to present in childhood. Only 35 cases of cranial chordomas have been reported in children 16 years of age or younger. We report 2 additional cases of pediatric cranial chordomas. One occurred in a 4 month old infant and to our knowledge represents the earliest age of presentation yet reported. The second case documents the value of MR imaging in delineating the extent of the tumor and defining its relationship to adjacent structures.