Intrasellar Chordomas Mimicking Pituitary Adenoma
Whereas chordomas involving the sellar region are uncommon, largely or entirely intrasellar chordomas are rare. The authors report three cases in which the chordomas filled the pituitary fossa and presented as nonfunctioning pituitary adenomas. All lesions exhibited the typical histological patterns and immunophenotype of chordoma. One tumor, studied ultrastructurally and subjected to DNA analysis, was shown to have a diploid histogram. The authors present a clinicopathological study of these three cases and review the literature on intrasellar chordomas.
Chordomas are rare, slow-growing malignant tumors of the midline, representing approximately 1% of all malignant tumors of the bone and 0.1 to 0.2% of intracranial neoplasms. They are thought to arise from cell rests, particularly of the proximal and distal extremes of the notochord. Approximately 50% develop in the sacrococcygeal region, 35% in the sphenooccipital region, and 15% in vertebrae. Most chordomas involving the sellar region are parasellar or suprasellar, and largely or entirely intrasellar lesions are very rare. In terms of their characteristics demonstrated on neuroimaging studies and their clinical presentation, intrasellar chordomas may mimic pituitary adenomas.
In this report we describe the clinical and operative features of three examples of intrasellar chordomas mimicking nonfunctioning pituitary adenoma. All lesions were subjected to immunocytochemistry and one to electron microscopy and DNA image analysis.
Whereas chordomas involving the sellar region are uncommon, largely or entirely intrasellar chordomas are rare. The authors report three cases in which the chordomas filled the pituitary fossa and presented as nonfunctioning pituitary adenomas. All lesions exhibited the typical histological patterns and immunophenotype of chordoma. One tumor, studied ultrastructurally and subjected to DNA analysis, was shown to have a diploid histogram. The authors present a clinicopathological study of these three cases and review the literature on intrasellar chordomas.
Chordomas are rare, slow-growing malignant tumors of the midline, representing approximately 1% of all malignant tumors of the bone and 0.1 to 0.2% of intracranial neoplasms. They are thought to arise from cell rests, particularly of the proximal and distal extremes of the notochord. Approximately 50% develop in the sacrococcygeal region, 35% in the sphenooccipital region, and 15% in vertebrae. Most chordomas involving the sellar region are parasellar or suprasellar, and largely or entirely intrasellar lesions are very rare. In terms of their characteristics demonstrated on neuroimaging studies and their clinical presentation, intrasellar chordomas may mimic pituitary adenomas.
In this report we describe the clinical and operative features of three examples of intrasellar chordomas mimicking nonfunctioning pituitary adenoma. All lesions were subjected to immunocytochemistry and one to electron microscopy and DNA image analysis.