Purpose
Pancreatic acinar cell carcinoma (ACC) is a rare cancer of the exocrine pancreas. Because of its rare incidence, the efficacy of chemotherapy in this patient population has been largely unknown. Therefore, we retrospectively analyzed the outcomes of patients with advanced pancreatic ACC who received chemotherapy.
Materials and Methods
Between January 1997 and March 2015, 15 patients with unresectable or metastatic pancreatic ACC who received systemic chemotherapy were identified in Asan Medical Center, Korea.
Results
The median age was 58 years. Eleven and four patients had recurrent/metastatic and locally advanced unresectable disease. The median overall survival in all patients was 20.9 months (95% confidence interval [CI], 15.7 to 26.1). As first-line therapy, intravenous 5-fluorouracil were administered in four patients (27%), gemcitabine in five (33%), gemcitabine plus capecitabine in two (13%), oxaliplatin plus 5-fluorouracil/leucovorin (FOLFOX) in two (13%), and concurrent chemoradiotherapy followed by capecitabine maintenance therapy in two (13%). The objective response rate (ORR) to chemotherapy alone was 23% and the median progression-free survival (PFS) was 5.6 months (95% CI, 2.8 to 8.4). After progression, second- line chemotherapy was administered in eight patients, while four patients received FOLFOX and the other four patients received gemcitabine. The ORR was 38%, and patients administered FOLFOX had significantly better PFS than those administered gemcitabine (median, 6.5 months vs. 1.4 months; p=0.007). The ratio of time to tumor progression (TTP) during first-line chemotherapy to TTP at second-line chemotherapy was significantly higher in patients administered FOLFOX (4.07; range, 0.87 to 8.30) than in those administered gemcitabine (0.12; range, 0.08 to 0.25; p=0.029).
Conclusion
Our results suggest that oxaliplatin-containing regimens may have improved activity against pancreatic ACC.
Citations
Citations to this article as recorded by
Precision oncology promise in the management of pancreatic acinar cell carcinoma Suriya Baskar, Rishi R. Patel, Matthew T. Gao, Brandon E. Rose, Sawyer Bawek, Deepak Vadehra, Timothy J. Brown, Nicholas J. Hornstein, Udhayvir S. Grewal European Journal of Cancer.2026; 234: 116223. CrossRef
Exceptional Response to Durvalumab and Tremelimumab in Pancreatic Acinar Cell Carcinoma With Ultramutated Phenotype Associated With a DNA Polymerase-ε Mutation Benjamin Koh, Subotheni Thavaneswaran, Frank Lin, John Grady, Meghana Maddula, Koroush S. Haghighi, Maya Kansara, Min Li Huang, Mandy L. Ballinger, John Simes, David M. Thomas, David Goldstein JCO Precision Oncology.2026;[Epub] CrossRef
Pancreatic Acinar Cell Carcinoma: A Rare Pancreatic Malignancy with Distinct Biology and Emerging Therapeutic Opportunities Noor Farhoud, Raed Moh’d Taiseer Al-Rajabi, Joaquina Celebre Baranda, Haoran Li, Wei Zhang, Ravi Kumar Paluri, Ashish Manne, Prasad Dandawate, Weijing Sun, Anup Kasi Cancers.2026; 18(14): 2315. CrossRef
Survival Outcomes and Genetic Characteristics of Resected Pancreatic Acinar Cell Carcinoma Alex B. Blair, Shannon N. Radomski, Joanne Chou, Mengyuan Liu, Thomas Clark Howell, Wungki Park, Eileen M. O’Reilly, Lei Zheng, Vinod P. Balachandran, Alice C. Wei, T. Peter Kingham, Michael I. D’Angelica, Jeffrey Drebin, Sabino Zani, Dan G. Blazer, Richa Annals of Surgical Oncology.2025; 32(3): 1869. CrossRef
Clinical Outcomes and Molecular Profiling of Pancreatic Acinar Cell Carcinoma: A Retrospective Study Cody Eslinger, Bobak Seddighzadeh, Claire Yee, Zaid Elsabbagh, Rish Pai, Chris Hartley, Jason Starr, Tanios Bekaii-Saab, Thorvardur R. Halfdanarson, Mohamad Bassam Sonbol JCO Precision Oncology.2025;[Epub] CrossRef
Pediatric pancreatic acinar cell carcinoma with a non-canonical BRAF-KMT2C fusion and a classic SND1-BRAF fusion: a case report and literature review Yaqin Wang, Jiasi Zhang, Dimin Nie, Ai Zhang, Qun Hu, Aiguo Liu BMC Pediatrics.2025;[Epub] CrossRef
The Growth Modulation Index (GMI) as an Efficacy Outcome in Cancer Clinical Trials: A Scoping Review with Suggested Reporting Guidelines Kilian Trin, Cynthia Dalleau, Simone Mathoulin-Pelissier, Christophe Le Tourneau, Derek Dinart, Carine Bellera Current Oncology Reports.2025; 27(5): 516. CrossRef
Pancreatic Acinar Cell Carcinoma: Demographics, Treatment, and Survival Outcomes, A Retrospective Population-Based Study Abdul Qahar Khan Yasinzai, Asif Iqbal, Diego Olavarria-Bernal, Kalyani Ballur, Agha Wali, Shalini Ballur, Bisma Tareen, Marjan Khan, Hritvik Jain, Israr Khan, Nooran Fadhil, Amir Humza Sohail, Asad Ullah Journal of Gastrointestinal Cancer.2025;[Epub] CrossRef
Pancreatic panniculitis associated with presumed advanced pancreatic tumor: A case report and literature review Dunya Yang, Shouyue Chen, Xiaoyu Zhang, Xiangwu Lin, Shaoqin Lin, Qunxiang Chen, Zongjing Zhang, Jie Li, Xi Chen Pathology - Research and Practice.2025; 275: 156214. CrossRef
Comprehensive review of pancreatic acinar cell carcinoma: epidemiology, diagnosis, molecular features and treatment Kenji Ikezawa, Makiko Urabe, Yugo Kai, Ryoji Takada, Hirofumi Akita, Shigenori Nagata, Kazuyoshi Ohkawa Japanese Journal of Clinical Oncology.2024; 54(3): 271. CrossRef
Pathological complete response with FOLFIRINOX therapy for recurrence of pancreatic acinar cell carcinoma Katsuhito Teramatsu, Nao Fujimori, Masatoshi Murakami, Sho Yasumori, Kazuhide Matsumoto, Kohei Nakata, Masafumi Nakamura, Yutaka Koga, Yoshinao Oda, Yoshihiro Ogawa Clinical Journal of Gastroenterology.2024; 17(4): 776. CrossRef
Complete pathological response to pembrolizumab in pretreated pancreatic acinar cell carcinoma Valeria Merz, Francesca Maines, Stefano Marcucci, Chiara Sartori, Michela Frisinghelli, Chiara Trentin, Dzenete Kadrija, Francesco Giuseppe Carbone, Andrea Michielan, Armando Gabbrielli, Davide Melisi, Mattia Barbareschi, Alberto Brolese, Orazio Caffo Journal of Cancer Research and Clinical Oncology.2024;[Epub] CrossRef
A narrative review on rare types of pancreatic cancer: should they be treated as pancreatic ductal adenocarcinomas? Victor Hugo Fonseca de Jesus, Mauro Daniel Spina Donadio, Ângelo Borsarelli Carvalho de Brito, Arthur Conelian Gentilli Therapeutic Advances in Medical Oncology.2024;[Epub] CrossRef
A case of unresectable ectopic acinar cell carcinoma developed in the portal vein in complete response to FOLFIRINOX therapy Shinnosuke Nakayama, Akihisa Fukuda, Tadayuki Kou, Manabu Muto, Hiroshi Seno Clinical Journal of Gastroenterology.2023; 16(4): 610. CrossRef
New treatment insights into pancreatic acinar cell carcinoma: case report and literature review Fangrui Zhao, Dashuai Yang, Tangpeng Xu, Jiahui He, Jin Guo, Xiangpan Li Frontiers in Oncology.2023;[Epub] CrossRef
A resected case of acinar cell carcinoma of the pancreas with liver metastasis following chemotherapy using modified FOLFIRINOX Shuhei Yamada, Haruka Motegi, Yoshiki Kurihara, Tomonori Shimbo, Isao Kikuchi, Toshiki Wakabayashi, Tsutomu Sato Surgical Case Reports.2023;[Epub] CrossRef
A Simple Overview of Pancreatic Cancer Treatment for Clinical Oncologists Ingrid Garajová, Marianna Peroni, Fabio Gelsomino, Francesco Leonardi Current Oncology.2023; 30(11): 9587. CrossRef
Progressive nodule-like lesions on bilateral lower limbs FA Yang, KS Cheng, JW Chou Journal of Postgraduate Medicine.2023; 69(1): 50. CrossRef
Mixed pancreatic acinar cell-ductal adenocarcinoma: Complexities in diagnosis and treatment Abdullah Nasser, Catherine L. Forse, Cynthia Walsh, Terence Moyana, Rakesh Goel Current Problems in Cancer: Case Reports.2022; 5: 100144. CrossRef
Optimizing Chemotherapy of Pancreatic Acinar Cell Carcinoma: Our Experiences and Pooled Analysis of Literature Jian-Ying Xu, Wen-Long Guan, Shi-Xun Lu, Xiao-Li Wei, Wen-Jie Shi, Chao Ren, Yu-Hong Li, Sheng-Ping Li, Miao-Zhen Qiu, Feng-Hua Wang Clinical Medicine Insights: Oncology.2022;[Epub] CrossRef
Successful conversion surgery after FOLFIRINOX therapy in a patient with advanced pancreatic acinar cell carcinoma with a solitary peritoneal dissemination: A case report Sunao Uemura, Hiromichi Maeda, Nobuhisa Tanioka, Sachi Yamaguchi, Masaya Munekage, Hiroyuki Kitagawa, Tsutomu Namikawa, Shota Yamamoto, Takuhiro Kohsaki, Mitsuko Iguchi, Kazushige Uchida, Kazuhiro Hanazaki Cancer Reports.2022;[Epub] CrossRef
Prolonged response on olaparib maintenance in metastatic pancreatic acinar cell carcinoma associated with a germline BRCA 2 mutation, revealed by severe panniculitis Margaux Lelong, Jean‐Luc Raoul, Yann Touchefeu, Jean‐Marie Berthelot, Paul Arnolfo, Tamara Matysiak‐Budnik, Hélène Senellart Clinical Case Reports.2022;[Epub] CrossRef
Efficacy of chemotherapy combined with toripalimab in PD-L1–positive and high tumor mutation burden pancreatic acinar cell carcinoma: case report Huanji Xu, Xin Wang, Sheng Zhou, Qiancheng Hu, Dan Cao Tumori Journal.2021; 107(6): NP24. CrossRef
Metastatic Acinar Cell Carcinoma of the Pancreas Elena Busch, Wiebke Werft, Nina Bougatf, Thilo Hackert, Dirk Jäger, Christoph Springfeld, Anne Katrin Berger Pancreas.2021; 50(3): 300. CrossRef
Pancreatic acinar cell carcinoma: A multi-center series on clinical characteristics and treatment outcomes Vishwajith Sridharan, Mari Mino-Kenudson, James M. Cleary, Osama E. Rahma, Kimberly Perez, Jeffrey W. Clark, Thomas E. Clancy, Douglas A. Rubinson, Lipika Goyal, Fateh Bazerbachi, Kavel H. Visrodia, Motaz Qadan, Aparna Parikh, Cristina R. Ferrone, Brenna Pancreatology.2021; 21(6): 1119. CrossRef
Down‐regulation of metabolic pathways could offset the poor prognosis conferred by co‐existent diabetes mellitus in pancreatic (head) adenocarcinoma Nilesh Gardi, Madhura Ketkar, Ross A. McKinnon, Stephen J. Pandol, Shilpee Dutt, Savio G. Barreto ANZ Journal of Surgery.2021; 91(11): 2466. CrossRef
Pathological complete response in a patient with metastatic pancreatic acinar cell carcinoma who received a chemotherapy regimen containing cisplatin and irinotecan Hiromitsu Maehira, Hiroya Iida, Haruki Mori, Nobuhito Nitta, Aya Tokuda, Katsushi Takebayashi, Sachiko Kaida, Toru Miyake, Akiko Matsubara, Masaji Tani Clinical Journal of Gastroenterology.2021; 14(6): 1772. CrossRef
Survival Outcome and Prognostic Factors for Pancreatic Acinar Cell Carcinoma: Retrospective Analysis from the German Cancer Registry Group Ekaterina Petrova, Joachim Wellner, Anne K. Nording, Rüdiger Braun, Kim C. Honselmann, Louisa Bolm, Richard Hummel, Monika Klinkhammer-Schalke, Sylke Ruth Zeissig, Kees Kleihues van Tol, Sylvia Timme-Bronsert, Peter Bronsert, Sergey Zemskov, Tobias Keck, Cancers.2021; 13(23): 6121. CrossRef
The Unusual Suspects of the Pancreas—Understanding Pancreatic Acinar Cell Carcinomas and Adenomas Andreas Minh Luu, Tim Fahlbusch, Johanna Munding, Waldemar Uhl, Chris Braumann Journal of Gastrointestinal Cancer.2020; 51(1): 172. CrossRef
Long-term survival of two patients with recurrent pancreatic acinar cell carcinoma treated with radiofrequency ablation: A case report Mariacristina Di Marco, Riccardo Carloni, Stefania De Lorenzo, Elisa Grassi, Andrea Palloni, Francesca Formica, Stefano Brocchi, Daria Maria Filippini, Rita Golfieri, Giovanni Brandi World Journal of Clinical Cases.2020; 8(7): 1241. CrossRef
Robot-assisted combined pancreatectomy/hepatectomy for metastatic pancreatic acinar cell carcinoma: case report and review of the literature Anthony Michael Villano, Dany Barrak, Anish Jain, Erin Meslar, Pejman Radkani, Walid Chalhoub, Nadim Haddad, Emily Winslow, Thomas Fishbein, Jason Hawksworth Clinical Journal of Gastroenterology.2020; 13(5): 973. CrossRef
Patients With Acinar Cell Carcinoma of the Pancreas After 2005 Yuan Zong, Changsong Qi, Zhi Peng, Lin Shen, Jun Zhou Pancreas.2020; 49(6): 781. CrossRef
One size does not fit all for pancreatic cancers: A review on rare histologies and therapeutic approaches Monica Niger, Michele Prisciandaro, Maria Antista, Melissa Anna Teresa Monica, Laura Cattaneo, Natalie Prinzi, Sara Manglaviti, Federico Nichetti, Marta Brambilla, Martina Torchio, Francesca Corti, Sara Pusceddu, Jorgelina Coppa, Vincenzo Mazzaferro, Fili World Journal of Gastrointestinal Oncology.2020; 12(8): 833. CrossRef
Successful BRAF/MEK inhibition in a patient with BRAFV600E-mutated extrapancreatic acinar cell carcinoma Elena Busch, Simon Kreutzfeldt, Abbas Agaimy, Gunhild Mechtersheimer, Peter Horak, Benedikt Brors, Barbara Hutter, Martina Fröhlich, Sebastian Uhrig, Philipp Mayer, Evelin Schröck, Albrecht Stenzinger, Hanno Glimm, Dirk Jäger, Christoph Springfeld, Stefan Molecular Case Studies.2020; 6(4): a005553. CrossRef
Pancreatic panniculitis and elevated serum lipase in metastasized acinar cell carcinoma of the pancreas: A case report and review of literature Rainer Christoph Miksch, Tobias S Schiergens, Maximilian Weniger, Matthias Ilmer, Philipp M Kazmierczak, Markus O Guba, Martin K Angele, Jens Werner, Jan G D'Haese World Journal of Clinical Cases.2020; 8(21): 5304. CrossRef
The contemporary trend in worsening prognosis of pancreatic acinar cell carcinoma: A population-based study Nie Duorui, Bin Shi, Tao Zhang, Chuyao Chen, Chongkai Fang, Zhijun Yue, Peng Wu, Zhiming Wu, Xuewu Huang, Meng Li, Girijesh Kumar Patel PLOS ONE.2020; 15(12): e0243164. CrossRef
Pancreatic acinar cell carcinoma diagnosed by BCL10 staining: A case report Kei YAMANE, Takayuki ANAZAWA, Toshihiko MASUI, Kazuyuki NAGAI, Seiichiro TADA, Kenta INOGUCHI, Kenzo NAKANO, Yuichiro UCHIDA, Akitada YOGO, Kyouichi TAKAORI, Shinji UEMOTO Suizo.2019; 34(1): 46. CrossRef
Prognostic Factors of Acinar Cell Carcinomas Axel Egal, Jérôme Cros, Magali Svrcek, Laurence Chiche, Geneviève Belleannee, Flora Poizat, Lionel Jouffret, Frédérique Maire, Louis de Mestier, Pascal Hammel Pancreas.2019; 48(10): 1393. CrossRef
EGFR amplification induces sensitivity to antiEGFR therapy in pancreatic acinar cell carcinoma Corentin Richard, Julie Niogret, Romain Boidot, Francois Ghiringhelli World Journal of Gastrointestinal Oncology.2018; 10(4): 103. CrossRef
Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors Oronzo Brunetti, Giuseppe Aprile, Paolo Marchetti, Enrico Vasile, Andrea Casadei Gardini, Mario Scartozzi, Sandro Barni, Sara Delfanti, Fernando De Vita, Francesco Di Costanzo, Michele Milella, Chiara Alessandra Cella, Rossana Berardi, Ivana Cataldo, Aldo Pancreas.2018; 47(6): 759. CrossRef
Pancreatic acinar cell carcinoma—case report and literature review Zhang Xing-mao, Zhang Hong-juan, Li Qing, He Qiang BMC Cancer.2018;[Epub] CrossRef