Intrasellar chordoma masquerading as a pituitary neuroendocrine tumor: Illustrative case.

Autor: Sato D; Department of Neurosurgery, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan., Hasegawa H; Department of Neurosurgery, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan., Kimura S; Department of Endocrinology and Nephrology, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan., Sato J; Department of Endocrinology and Nephrology, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan., Shinya Y; Department of Neurosurgery, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan., Umekawa M; Department of Neurosurgery, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan., Yasunaga Y; Department of Pathology, Graduate School of Medicine, University of Tokyo, Bunkyo-ku, Tokyo, Japan., Makita N; Department of Endocrinology and Nephrology, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan., Saito N; Department of Neurosurgery, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan.
Jazyk: angličtina
Zdroj: Surgical neurology international [Surg Neurol Int] 2024 May 10; Vol. 15, pp. 159. Date of Electronic Publication: 2024 May 10 (Print Publication: 2024).
DOI: 10.25259/SNI_116_2024
Abstrakt: Background: Chordomas are rare, locally aggressive neoplasms recognized as derivatives of the notochord vestiges. These tumors typically involve the midline axial skeleton, and intracranial chordomas exhibit proclivity for the spheno-occipital region. However, purely intrasellar occurrences are extremely rare. We report a case of intrasellar chordoma, which masqueraded as a pituitary neuroendocrine tumor.
Case Description: An 87-year-old female presented with an acutely altered mental state after a few-week course of headaches and decreased left vision. Adrenal insufficiency was evident, and magnetic resonance imaging revealed an intrasellar lesion with heterogeneous contrast enhancement and marked T2 hyperintensity. Central adrenal insufficiency due to an intrasellar lesion was suspected. Cortisol replacement was initiated, and transsphenoidal surgery was performed. Anterosuperior displacement of the normal pituitary gland and the absence of the bony dorsum sellae were notable during the procedure. Histological examination led to a diagnosis of conventional chordoma, and upfront adjuvant stereotactic radiosurgery was executed. She has been free from tumor progression for 12 months.
Conclusion: This case and literature review suggested that the pathognomonic features of intrasellar chordoma were heterogeneous contrast enhancement, marked T2 hyperintensity, osteolytic destruction of the dorsum sellae, and anterosuperior displacement of the pituitary gland. Clinical outcomes seemed slightly worse than those of all skull base chordomas, which were the rationale for upfront radiosurgery in our case. Neurosurgeons should include intrasellar chordomas in the differential diagnosis of intrasellar lesions, carefully dissect them from the adjacent critical anatomical structures, and consider upfront radiosurgery to achieve optimal patient outcomes.
Competing Interests: There are no conflicts of interest.
(Copyright: © 2024 Surgical Neurology International.)
Databáze: MEDLINE