Background: Chordoma that occurs primarily in the sella turcica is rare and presents unique treatment challenges.
Objective: The purpose of this study was to determine common features, treatment approaches, and survival characteristics of intrasellar chordoma.
Methods: Institutional databases, the SEER (Surveillance Epidemiology and End Results) database, and PubMed/EMBASE were queried for chordoma with a primarily intrasellar component. The SEER database was also queried for adult skull base chordoma. Patient-level data were extracted where available. Kaplan-Meier survival analyses were conducted.
Results: Among 80 cases, the mean age at presentation was 55.6 (standard deviation, 15.9), with a female predominance (1.16:1.00). Patients experienced symptoms for a mean duration of 19.0 months, including cranial nerve deficits, hypopituitarism, and hyperprolactinemia. Among patients receiving treatment, 77.5% underwent surgery. In addition, less than half of the patients (n = 34, 47.3%) received adjuvant radiation therapy. The 5-year overall survival (OS) of intrasellar chordoma was 60.0% (standard error [SE], 6.9). Patients aged 40 years and younger had a 5-year OS of 80.8% (SE, 12.2), compared with patients older than 40 years, who had an OS of 55.4% (SE, 7.7) (Mantel-Cox, P = 0.044). Males experienced a lower 5-year OS (44.0; SE, 9.7) than did females (76.8; SE, 8.5), (Mantel-Cox, P = 0.003). Median OS was greater in patients with skull base chordoma than in patients with intrasellar chordoma (Mantel-Cox, P = 0.046).
Conclusions: Intrasellar chordoma presents frequently with visual disturbances and hyperprolactinemia and has a slightly higher incidence in females. Young age predicts a better prognosis. Intrasellar chordoma has a lower overall survival than has skull base chordoma.
Keywords: Chordoma; Intrasellar; Radiation therapy; Transsphenoidal surgery.
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