Intrasellar chordomas mimicking pituitary adenoma

Object. Whereas chordomas involving the sellar region are uncommon, largely or entirely intrasellar examples are rare. The goal in this study was to p resent examples of these rare tumors as a guide to their proper diagnosis a nd treatment. Methods. The authors report three cases in which the chordomas filled the p ituitary fossa and presented as nonfunctioning pituitary adenomas. All lesi ons exhibited the typical histological patterns and immunophenotype of chor doma. One tumor, studied ultrastructurally and subjected to DNA analysis, w as shown to have a diploid histogram. The authors present a clinicopatholog ical study of these three cases and review the literature on intrasellar ch ordomas. Conclusions. Although these tumors are easily misdiagnosed and therefore ma y not receive optimal treatment, aggressive surgical resection can yield a favorable prognosis in lesions with a limited extent.