Introduction: Chordoma is a locally aggressive tumor arising from notochord remnants. Little is known about the characteristics and long-term prognosis of intrasellar chordoma. The purpose of this study was to determine common features, treatment approaches and survival characteristics of this rare clinical entity.
Methods: Institutional databases at BWH/MGH were searched for intrasellar chordoma. The National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database November 2015 submission was queried for ‘chordoma’ (ICD-O-3 9370/3) occurring primarily in the ‘pituitary gland’ (Site C75.1). Using PubMed/NCBI, a comprehensive literature review of reported cases of intrasellar chordoma was conducted. From all sources, patient-level data was extracted where available. SEER was also queried for skull base chordoma. Kaplan-Meier statistical analyses were conducted with a primary endpoint of death. A p-value of less than 0.05 was considered significant.
Results: A total of 89 cases were evaluated. Of these, 8 were a chondroid variant. The mean age at presentation was 55 (SD ± 15.8), with a slight female predilection (1.1:1). Patients experienced symptoms for a mean duration of 19.3 months, including neurological (93.9 percent) and endocrine (58.5 percent) disturbances. Among patients receiving treatment, 88.6 percent underwent surgery, 52.4 percent with a transsphenoidal/endonasal approach and 47.6 percent with a craniotomy. Additionally, 45.5 percent of patients received adjuvant external beam radiation. The median overall survival (OS) rate was 90.0 months for intrasellar chordoma, compared to 154.0 months for non-intrasellar skull base chordoma (Mantel-Cox, p=0.036). Patients aged 0-49 had a higher median OS than patients 50 or older (182.0 v. 63.0 months, p = 0.013). Adjuvant radiation did not result in a higher median OS (p = 0.48).
Conclusions: Unlike intracranial chordoma, overall survival in intrasellar chordoma is not improved with adjuvant radiation. Young age predicts a better prognosis. Intrasellar chordoma has a lower overall survival than non-intrasellar skull base chordoma.
Patient Care: This research will inform neurosurgeons and neurosurgery trainees on the presentation and management of intrasellar chordoma.
Learning Objectives: By the conclusion of this session, participants should be able to:
1) Describe the presentation of intrasellar chordoma.
2) Identify the prognostic indicators of overall survival of intrasellar chordoma.