Introduction: Chordoid Gliomas (CG) are rare low-grade neoplasms which frequently arise within the third ventricle. Their location and tight adherence to the hypothalamus makes gross total resection (GTR) difficult and aggressive resection is reported to have an increased risk of post-operative hypothalamic dysfunction. We performed a systematic review to determine surgical complications and prognostic factors that are associated with recurrence due to the void of large retrospective studies for CG.
Methods: Two reviewers (LA, WC) performed a comprehensive search on 3/9/2015 utilizing the terms “chordoid” AND “glioma” on the following databases: MEDLINE (Pubmed/OVID), Embase, Scopus, and Web of Science. The search was refined by limiting to manuscripts published in English. Studies that did not stratify each patient individually or included orbital-based lesions were excluded. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed.
Results: 81 patients were accrued from 54 studies which met the study criteria. There were 33 males (41%) and 48 females (59%) with a median age at diagnosis of 48 years (range 5-72). Extent of resection (EOR) was reported in 52 patients which was inclusive of: biopsy (11%), subtotal resection (STR) (45%), and GTR (44%). GTR was associated with an improved progression-free survival (PFS) (p=0.028). The most common surgical approaches reported included: trans-lamina terminalis (n=17), transcallosal (n=7), and transcortical (n=5). A trans-laminar terminalis approach demonstrated a strong trend towards decreased overall rates of postoperative morbidity (p=0.051). Postoperative complications occurred in 31 patients with the most common being new onset diabetes insipidus (40% of complications). Post-operative morbidity was not associated with age, tumor size, or extent of resection.
Conclusions: GTR should be the primary goal for the treatment of CG due to improved rates of tumor control without an increased risk of postoperative complications. Surgical approach appears to be a stronger predictor of complication rates than EOR.
Patient Care: There is a lack of clinical data for the treatment and outcomes of chordoid gliomas due to the rarity of the tumor. We pooled together all published case reports and small case series in order to provide clinicians a better picture of this poorly understood neoplasm. Our finding of EOR's lack of impact on postoperative morbidity is novel for this tumor since most reports suggest that a greater resection results in a worse postoperative outcome.
Learning Objectives: 1) Determine the utility of EOR in PFS for chordoid glioma
2) Describe the role of surgical approach in postoperative morbidity
3) Determine the role of EOR in postoperative morbidity