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Acinar cell carcinoma of the pancreas: a rare disease with different diagnostic and therapeutic implications than ductal adenocarcinoma

  • Original Article – Clinical Oncology
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Abstract

Purpose

Acinar cell carcinoma (ACC) of the pancreas is a very rare cancer, constituting 1 % of all malignant non-endocrine pancreatic tumors. Only very limited data exist to guide treatment in patients with advanced ACC.

Methods

Between 2000 and 2015, 15 patients with ACC were diagnosed and/or treated at our high-volume comprehensive cancer center. Medical records and correlating serum levels of the potential serum tumor markers CA 19-9, CEA and lipase were analyzed retrospectively.

Results

A substantial antitumor activity was observed for treatment regimens containing 5-FU and oxaliplatin with partial responses or prolonged disease stabilizations (>12 months) observed in 6 out of 7 patients (86 %). Activity was also observed for single-agent 5-FU and its oral prodrugs. Serum lipase levels were elevated in 7 of 12 patients with advanced disease (58 %), whereas CEA and CA 19-9 seemed to be of minor importance for ACC (elevated pre-treatment levels in 4/12 and 3/12 cases, respectively). In selected patients, repeated serum lipase measurements were available and accurately predicted response to chemotherapy and relapse after surgery.

Conclusions

5-FU- and oxaliplatin-containing regimens are active in advanced ACC. Lipase kinetics may be a useful novel tool to monitor the course of disease as well as treatment effects in ACC.

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Abbreviations

ACC:

Acinar cell carcinoma

CA 19-9:

Carbohydrate antigen 19-9

CEA:

Carcinoembryonic antigen

ECOG:

Eastern Cooperative Oncology Group (ECOG) score

OS:

Overall survival

PDAC:

Pancreatic ductal adenocarcinoma

References

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Acknowledgments

This work is part of the doctoral thesis of Johannes Philipp Burger.

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Authors and Affiliations

Authors

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Correspondence to Stefan Boeck.

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Conflict of interest

Stefan Boeck has received honoraria for scientific presentations from Celgene and Roche, research funding from Celgene, Clovis Oncology and Roche, travel grants from Celgene and Roche, and acted as consultant for Celgene and Baxalta. Michael Haas has received honoraria for scientific presentations from Celgene, research funding (for his institution) from Boehringer Ingelheim and Roche, and travel grants from Boehringer Ingelheim and Celgene. Volker Heinemann has received honoraria for scientific presentations from Baxalta, Celgene and Roche, research funding from Celgene and Roche, and acted as consultant for Baxalta, Celgene and Roche. Thomas Kirchner has received honoraria for scientific presentations from Merck KGaA, and AstraZeneca, research funding from Merck and Roche, and acted as consultant for Amgen, AstraZeneca, Merck, MSD, Pfizer and Roche. All other authors declare no conflict of interest.

Ethical standard

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. For this retrospective study, formal consent was not required (Approval Number 134-15, Ethics Committee Ludwig-Maximilians-University Munich).

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Kruger, S., Haas, M., Burger, P.J. et al. Acinar cell carcinoma of the pancreas: a rare disease with different diagnostic and therapeutic implications than ductal adenocarcinoma. J Cancer Res Clin Oncol 142, 2585–2591 (2016). https://doi.org/10.1007/s00432-016-2264-7

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  • DOI: https://doi.org/10.1007/s00432-016-2264-7

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