Presentation, treatment, and long-term outcome of intrasellar chordoma: a pooled analysis of institutional, SEER, and published data.

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Presentation, treatment, and long-term outcome of intrasellar chordoma: a pooled analysis of institutional, SEER, and published data.

World Neurosurg. 2017 Oct 20;:

Authors: Ahmed AK, Dawood HY, Arnaout OM, Laws ER, Smith TR

Abstract
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 Surveillance, Epidemiology, and End Results (SEER) 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 (SD: 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. Additionally, less than half of the patients (34, 47.3%) received adjuvant radiation therapy. The 5-year overall survival (OS) of intrasellar chordoma was 60.0% (SE: 6.9). Patients aged 40 and younger had a 5-year OS of 80.8% (SE: 12.2), compared to patients older than 40, who had a 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 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 skull base chordoma.

PMID: 29061463 [PubMed - as supplied by publisher]

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