Intrasellar Chordoma Mimicking Pituitary Macroadenoma with Hyperprolactinemia and Hypopituitarism: Clinical Images with a Surgical Video

A purely intrasellar chordoma is rare among skull base chordomas and is recognized as originating from ectopic embryological notochord located in the sella turcica. In view of its rarity and nonspecific symptoms, clinicians may misdiagnose intrasellar chordoma as pituitary adenoma based on preoperative radiographic images. In this report, we present an intrasellar chordoma that clinically mimicked pituitary macroadenoma with hyperprolactinemia and hy-popituitarism and was successfully resected by endoscopic endonasal trans-sphenoidal surgery. This case demonstrated radiographic features that chordoma should be suspected in sellar lesions. The enlarged sellar with thinned remodeled bone without clival destruction was firstly reminiscent of pituitary adenoma, whereas the very high signal on T2-weighted images and heterogeneous enhancement characteristically suggested chordoma. This rare diagnosis must be considered in the preoperative evaluation of sellar lesions because it can affect how the neurosurgeon prepares for surgery and the sur-gical goals.

Automated summary

Purely intrasellar chordoma is a rare condition that originates from an ectopic embryological notochord in the sella turcica. Due to its rarity and nonspecific symptoms, intrasellar chordoma may be misdiagnosed as pituitary adenoma based on preoperative radiographic images. This report presents a case of intrasellar chordoma mimicking pituitary macroadenoma, which was successfully resected by endoscopic endonasal trans-sphenoidal surgery. The radiographic features, such as enlarged sellar with thinned remodelled bone and high signal on T2-weighted images with heterogeneous enhancement, are characteristic of chordoma and should raise suspicion in sellar lesions.