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Pancreatic Acinar Cell Carcinoma in a Patient with Familial Adenomatous Polyposis Who Achieved Long-Term Survival after Repeated Resection of Liver Metastases: A Case Report

Year: 2026

Type: article

Abstract: INTRODUCTION: Pancreatic acinar cell carcinoma (PACC) is a rare exocrine pancreatic neoplasm that differs from pancreatic ductal adenocarcinoma (PDAC) in its histogenetic origin and oncological behavior. Although recurrence is common after curative-intent resection, the optimal management of recurrent PACC remains undefined. Furthermore, although a certain number of pancreatic tumors have been reported in association with familial adenomatous polyposis (FAP), the relationship with PACC in particular remains poorly understood. The present case may help address this issue. CASE PRESENTATION: ) variant was identified, leading to a definitive diagnosis of FAP. CONCLUSIONS: We experienced a case in which an aggressive surgical approach-repeated hepatectomy for multiple recurrent liver metastases from PACC-achieved long-term recurrence-free survival. This supports active surgical resection as a viable strategy for PACC liver metastases. Furthermore, this rare case of PACC arising in a patient with FAP also raises the hypothesis that the APC/β-catenin pathway may be biologically involved in PACC tumorigenesis; however, a single case cannot establish an epidemiological association between FAP and PACC incidence, and further investigation is warranted.

Source: Surgical Case Reports

DOI: 10.70352/scrj.cr.26-0605