JOURNAL OF RARE DISEASES 2025;4(4):437-445

doi:10.12376/j.issn.2097-0501.2025.04.006

Research Progress in the Diagnosis and Treatment of Pancreatic Acinar Cell Carcinoma

Wenfei LI 1 ; Yuan XIE 2 ; Liyang MO 3 ; Junjie DANG 2 ; Qi WANG 3 ; Yang YANG 3 ; Qiuying SUN 3 ; Zhenping WEN 2 ; Sai GE 4 ; Xiaotian ZHANG 4

Affiliations

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Keywords

pancreatic acinar cell carcinoma; diagnosis; treatment; targeted therapy; immunotherapy

Country

China

Language

Chinese

Abstract

Pancreatic acinar cell carcinoma (PACC) is a rare exocrine tumor of the pancreas with distinct clinical and pathological features. In recent years, advancements in molecular biology techniques have led to a deeper understanding of the molecular mechanisms underlying PACC. Progress in imaging, endoscopic, and molecular diagnostic technologies has improved the early detection rate of PACC. The primary treatment modalities for PACC include surgical resection, chemotherapy, targeted therapy and immunotherapy; however, the therapeutic efficacy still requires further improvement. This article reviews the current research status of PACC, covering its epidemiology, pathological characteristics, molecular alterations, diagnostic methods, and treatment strategies, and discusses the controversies and future directions in PACC research.