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 W Casey; Carlos Fernandez-Del Castillo; David Patrick Ryan; Keith D Lillemoe; Andrew L Warshaw; Kumar Krishnan; Yasmin G Hernandez-Barco

doi:10.1016/j.pan.2021.05.011

Pancreatic acinar cell carcinoma: A multi-center series on clinical characteristics and treatment outcomes

Pancreatology. 2021 May 15:S1424-3903(21)00162-9. doi: 10.1016/j.pan.2021.05.011. Online ahead of print.

Authors

Vishwajith Sridharan¹, Mari Mino-Kenudson², James M Cleary³, Osama E Rahma³, Kimberly Perez³, Jeffrey W Clark⁴, Thomas E Clancy³, Douglas A Rubinson³, Lipika Goyal⁴, Fateh Bazerbachi¹, Kavel H Visrodia¹, Motaz Qadan⁵, Aparna Parikh⁴, Cristina R Ferrone⁵, Brenna W Casey¹, Carlos Fernandez-Del Castillo⁵, David Patrick Ryan⁴, Keith D Lillemoe⁵, Andrew L Warshaw⁵, Kumar Krishnan¹, Yasmin G Hernandez-Barco⁶

Affiliations

¹ Division of Gastroenterology, Massachusetts General Hospital, Harvard School of Medicine, Boston, MA, USA.
² Division of Pathology, Massachusetts General Hospital, Harvard School of Medicine, Boston, MA, USA.
³ Dana Farber Cancer Institute and Brigham Women's Hospital, Harvard School of Medicine, Boston, MA, USA.
⁴ Division of Medical Oncology, Massachusetts General Hospital, Harvard School of Medicine, Boston, MA, USA.
⁵ Division of Surgical Oncology, Massachusetts General Hospital, Harvard School of Medicine, Boston, MA, USA.
⁶ Division of Gastroenterology, Massachusetts General Hospital, Harvard School of Medicine, Boston, MA, USA. Electronic address: yhernandez-barco@mgh.harvard.edu.

PMID: 34023183
DOI: 10.1016/j.pan.2021.05.011

Abstract

Background: Acinar cell carcinoma (ACC) is a very rare tumor of the exocrine pancreas, representing less than 1% of all pancreatic malignancies. The majority of data regarding ACC are limited to small case series.

Methods: This is a retrospective study conducted at a large healthcare system from 1996 to 2019. Patients with pathologically confirmed ACC were included, and demographic data, tumor characteristics, and treatment outcomes were abstracted by chart review. Survival curves were obtained by using the Kaplan-Meier method and compared using the log-rank test.

Results: Sixty-six patients with ACC were identified. The median patient age at diagnosis was 64, and 42% presented with metastatic disease. The majority presented with abdominal pain or pancreatitis (69%), and laboratory parameters did not correlate with tumor size, metastatic disease, or survival. Several somatic abnormalities were noted in tumors (BRCA2, TP53, and mismatch-repair genes). In patients with localized disease that underwent resection, the median time to develop metastatic lesions was 13 months. The median overall survival (OS) was 24.7 months from diagnosis, with a survival difference based on metastatic disease at diagnosis (median 15 vs 38 mos). Surgery was associated with improved survival in non-metastatic cases (p = 0.006) but not metastatic cases (p = 0.22), and chemotherapy showed OS benefit in metastatic disease (p < 0.01). Patients with metastatic ACC treated after 2010 utilized more platinum-based agents, and there was a OS benefit to FOLFOX or FOLFIRINOX chemotherapy compared to gemcitabine or capecitabine-based regimens (p = 0.006).

Conclusion: Pancreatic ACC patients often present with advanced disease. Surgery was associated with survival benefit among patients presenting with localized disease. The use of FOLFOX or FOLFIRINOX chemotherapy regimens was associated with improved OS in metastatic patients. These data add to our knowledge in this rare malignancy, and improves understanding about the genomic underpinnings, prognosis and treatment for acinar cancers.

Keywords: Acinar cell carcinoma; Chemotherapy outcomes; Institutional series.