Surgical management and prognostic factors for Endolymphatic Sac Tumor: a single-institute experience with a systematic review.

Objective: To evaluate the clinical features, surgical outcomes, and predictors of progression-free survival (PFS) in patients with Endolymphatic Sac Tumors (ELSTs).

Methods: This retrospective study analyzed 15 cases from Beijing Tiantan Hospital and 237 from literature (1988-2023), focusing on patients with pathologically confirmed intracranial or skull ELSTs who had comprehensive treatment and follow-up records. Univariate and multivariate Cox regression analyses were employed to identify factors influencing PFS.

Results: Cases from our institute comprised 10 males and 5 females with an average age of 39.1 years. Among these, 86.7% underwent gross total resection (GTR). During the follow-up period, two patients (13.3%) were lost to follow-up. After a mean follow-up of 74.9 months, one patient experienced recurrence and another died from unrelated causes. A review of literature identified 237 additional patients, including 134 females (56.5%), with an average age of 39.8 years; 22.8% of these patients had von Hippel-Lindau disease. The GTR rate was 69.2%. After a mean follow up of 53.2 months, 33 recurrences occurred, and the median PFS was 48 months. Additionally, eight patients died during the follow-up period, none of the deaths were attributed to ELSTs. Multivariate analysis identified GTR (HR 0.279, 95% CI 0.086-0.903, p = 0.033) as a significant protective factor against recurrence among the pooled cases.

Conclusions: GTR is crucial for improving PFS in ELST patients, emphasizing the need for advanced surgical techniques and long-term follow-up due to potential recurrences.