Symptomatic Progression, Recurrence, and Long-Term Follow-Up of Patients With Intracranial Epidermoid Cysts

Neurosurgery. 2024 Dec 19. doi: 10.1227/neu.0000000000003309. Online ahead of print.

Abstract

Background and objectives: Intracranial epidermoid cysts are rare, slow-growing but highly recurrent tumors with incompletely understood symptoms, progression, complications, and outcomes. The aim of the study was to characterize the symptomatology, surgical management, and long-term outcomes of these tumors.

Methods: This single-center retrospective analysis identified patients with pathologically confirmed intracranial epidermoid cysts from 1989 to 2023. Demographic and clinical variables were collected at diagnosis, before and after each resection, and at the latest follow-up. Differences across time points were evaluated using analysis of variance with post hoc Tukey's honestly significant difference tests and Pearson χ2 test with posthoc Bonferroni-corrected z-tests for independent proportions (significance: P ≤ .05). Kaplan-Meier, Cox regression, and multivariate logistic regression analyses were used to investigate predictors for recurrence-free survival and 30-day readmission following first surgery, respectively.

Results: Of 146 patients in the final cohort, the average age at diagnosis was 40.3 years, and 52.1% of patients were female. The mean maximum tumor dimension at presentation was 4.3 cm. Most presenting symptoms were significantly reduced at the latest follow-up, including headaches (presentation: 40.4%/latest follow-up: 8.8%, P < .001), vertigo/nausea/vomiting (33.1%/3.7%, P < .001), ataxia/incoordination (22.8%/4.4%, P < .001), and seizures (11.8%/2.9%, P < .005). Similarly, significant improvements were observed in cranial nerve (CN) II deficits (11.0%/3.7%, P = .020) and CN V (11.8%/3.7%, P = .012) and CN VIII excitatory symptoms (11.8%/2.9%, P = .005). Following the first resection, 12.5% of patients were readmitted within 30 days; lumbar drain placement was associated with higher odds of readmission (adjusted odds ratio [95% CI] = 8.26 [1.29-88.19]). Evidence of tumor remnant on the immediate postoperative MRI was associated with a shorter time to recurrence following the first surgery (P = .023).

Conclusion: The clinical progression of intracranial epidermoid cysts is marked by a spectrum of troublesome presenting symptoms and a notable tendency for recurrence, particularly following subtotal resection. Nevertheless, symptomatic improvement is the rule even if reoperations are needed, and mortality is exceedingly rare.