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.
- 1Department of Gastroenterological Surgery, Saitama Cancer Center, Ina-machi, Saitama, Japan.
- 2Department of Pathology, Saitama Cancer Center, Ina-machi, Saitama, Japan.
Abstract
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. At age 22, the patient underwent distal pancreatectomy for a pancreatic tumor, which was diagnosed as PACC. A local recurrence occurred in the fourth postoperative year and achieved complete response with chemotherapy. From the tenth postoperative year onward, the patient developed repeated liver metastases, for which a total of 5 hepatectomies were performed; the patient has remained recurrence-free for 72 months since the last surgery. During the clinical course, a pathogenic adenomatous polyposis coli (APC) variant was identified, leading to a definitive diagnosis of FAP. 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.
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BibTeX
@article{takahashi2026pancreatic,
title = {{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.}},
author = {Ayano Takahashi and Toshiro Ogura and Naganori Yamada and Koichiro Kumano and Ibuki Fujinuma and Hiroaki Kanda and Amane Takahashi},
journal = {Surgical case reports},
year = {2026},
month = oct,
doi = {10.70352/scrj.cr.26-0605},
url = {https://europepmc.org/article/MED/42953875},
note = {CC BY},
}
RIS
TY - JOUR
TI - 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.
AU - Ayano Takahashi
AU - Toshiro Ogura
AU - Naganori Yamada
AU - Koichiro Kumano
AU - Ibuki Fujinuma
AU - Hiroaki Kanda
AU - Amane Takahashi
PY - 2026
DA - 2026/10/28
JO - Surgical case reports
DO - 10.70352/scrj.cr.26-0605
UR - https://europepmc.org/article/MED/42953875
AB - 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. At age 22, the patient underwent distal pancreatectomy for a pancreatic tumor, which was diagnosed as PACC. A local recurrence occurred in the fourth postoperative year and achieved complete response with chemotherapy. From the tenth postoperative year onward, the patient developed repeated liver metastases, for which a total of 5 hepatectomies were performed; the patient has remained recurrence-free for 72 months since the last surgery. During the clinical course, a pathogenic adenomatous polyposis coli (APC) variant was identified, leading to a definitive diagnosis of FAP. 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.
SN - 2198-7793
ER -