Abstract Background: Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic malignancy, and evidence guiding systemic therapy remains limited. To address this unmet need, we conducted a multi center study to characterize real world treatment patterns and clinical outcomes, with the goal of informing evidence-based therapeutic recommendations for PACC. Methods: Clinical and treatment data were collected from 156 patients with PACC across eight pancreatic centers in China. Analyses focused on systemic therapy regimens and survival outcomes. Additional stratified analyses were performed among patients who underwent surgical resection to assess the therapeutic value of FOLFIRINOX in neoadjuvant and adjuvant settings. Results: A total of 156 patients were identified. The median age at diagnosis was 65.3 years, and 48.6% of patients underwent surgical resection. Most patients presented with abdominal pain or pancreatitis (69%). Laboratory parameters showed no correlation with tumor size, metastatic status, or survival. The median overall survival (OS) was 18.9 months from diagnosis, with significant differences based on metastatic status (15.6 vs. 31.8 months). Both surgical resection and FOLFIRINOX were associated with improved OS. Among surgically treated patients, neoadjuvant or adjuvant FOLFIRINOX conferred a significant OS advantage compared with gemcitabine- or capecitabine-based regimens (p = 0.001). In a multivariable Cox regression model, both surgical resection and FOLFIRINOX remained independent predictors of superior OS. Conclusions: This multi center study provides the largest real-world evidence to date supporting FOLFIRINOX as the preferred systemic therapy for PACC. Ongoing incorporation of international cohorts and expanded stratified analyses will further refine treatment recommendations. The overarching objective of this project is to establish standardized, evidence-based guidelines for the management of PACC. Citation Format: Guoliang Qiao. FOLFIRINOX represents the preferred therapeutic option for patients with pancreatic acinar cell carcinoma: A multi-center study abstract. In: Proceedings of the American Association for Cancer Research Annual Meeting 2026; Part 1 (Regular Abstracts); 2026 Apr 17-22; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2026;86(7 Suppl):Abstract nr 1236.
Guoliang Qiao (Fri,) studied this question.