Maffucciʼs Syndrome Associated with Intracranial Chordoma

Abstract

This report discusses Maffucci's syndrome associated with intracranial chordoma. Of the 170 reported cases of this syndrome, only 27 have been associated with intracranial lesions and none was involved with intracranial chordoma. Chordomas are occasionally difficult to differentiate from chondroma and chondrosarcoma. An immunohistochemical analysis was able to prove, however, that the chordoma of this case significantly differed from chondroma and chondrosarcoma.